10th World Cleft Lip, Palate & Craniofacial Congress

Diagnosis, Prevention, Genetics & Management of Cleft & Craniofacial Deformities

Current Techniques, Research & Future Directions

Transactions of 10th World Cleft Lip, Palate & Craniofacial Congress of International Cleft Lip and Palate Foundation (ICPF)

Pre-Conference Live Surgical Workshop October 24 & 25, 2016 Balaji Dental & Craniofacial Hospital, Chennai, India

Main Conference October 26-28, 2016 Hyatt Regency, Chennai, India

Edited By : Prof. S.M. Balaji, MDS, PhD., DSc.,

1 10th World Cleft Lip, Palate & Craniofacial Congress

10th World Cleft Lip, Palate & Craniofacial Congress

Congress Secretariat:

Balaji Dental & Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai, India Ph: 044-4294 7222 E-mail : [email protected]

2 10th World Cleft Lip, Palate & Craniofacial Congress

Foreword

Dear Colleagues,

It is my pleasure and honor to bring out the official record of the scientific proceedings and transactions of the 10th World Cleft Lip and Palate Conference held in Chennai, India. The official programs of this meeting, held under the aegis of International Cleft Lip and Palate Foundation includes diverse, multidisciplinary proceedings in the management of cleft lip and palate. This record and documentation of the happenings of the meeting would serve as a ready source of reference for all orations, invited talks and free scientific presentations. Most of the research work and experience shared in conferences are often not published in open domains and remains untraceable. It is the proceedings that officially document such valuable knowledge that serves a ready reference for future generations. The treatment options in the domain of surgery, occlusion rehabilitation, speech, and genetics have undergone rapid modification in the last few years. Such modifications results in large series of research manuscripts across the globe. The deeper understanding of the etiopathogeneisis, utilization of biomimetics and tissue engineering are creating new avenues of research. With expanding cleft networks and voluntary missions, even in remotest area and in limited resources settings, newer cost effective protocols are being evolved. These protocols are being presented and discussed in this meeting. I believe that this compilation have reflected the true nature of the International Cleft Lip and Palate Foundation. I am sure that this book will serve as a worthwhile Withreminder best regardsof all the scientific progress achieved through this meeting. Prof. S.M. Balaji Congress President 10th World Cleft Lip, Palate & Craniofacial Congress

3 10th World Cleft Lip, Palate & Craniofacial Congress CONTENTS

Prof. Kenneth Salyer, USA...... ICPF Presidential Oration - Global Craniofacial Care: 45 Year Personal Perspective 013 Prof. Anthony Markus, UK...... Prof. David Precious Memorial Oration - David Precious - A Celebration 015

Prof. M. S. AnthonyS. Ramakrishnan Wolfe, MemorialUSA Oration - Pediatric Plastic Surgery becomes Craniofacial Surgery, t hanks to Paul Tessier ...... 016

Prof. Dr.Dr. C. GE R. Ghali, Sundararajan USA...... Memorial Oration - EXIT (Ex Utero Intrapartum Treatment) Procedure: A Modality f or the Airway Management of the Select Cleft and Craniofacial Patient Population 017

Prof. Dr.Dr. Kawai Adrian Memorial Sugar, UK...... Oration - Orthognathic Surgery and Alveolar Bone Grafting in Cleft Lip and Palate Patients 019

Prof. Dr.Dr. J. NR G. KannappanKrishnaswamy, Memorial India...... Oration - Management of patients with cleft lip and palate - The Orthodontist’s Perspective 021 Prof. Dr. S.M. Balaji, India...... Prof. Dr. U. S. Nayak Memorial Oration - Lip to Cranium 022 Sai Shamini, India ANAMAYA IndoPacific Cleft Prevention Program: Shifting the attention to prevention of cleft lip and palate 026 Karthika Panneerselvam, India...... 028 Review of studies on a role of folic acid in etiology and prevention of cleft lip and palate

Viviana Armentano, Argentina...... 030 The role of medical geneticist in prevention of cleft lip and palate -

Nagato Natsume, ...... 032 Preventive research on cleft lip and palate in Japan -

Nagato Natsume, Japan...... 033 International Collaboration for Cleft Lip and/or palate from Japan

KangminAhn, S. Korea...... 035 PTCH1 gene mutation in nevoid basal cell carcinoma syndromewith diverse clinical manifestations

Kaori Matsumura, Japan ...... 036 Microarray analysis of global gene expression during palate development

Ashwin Dalal, India ...... Genetic Testing for Diagnosis of Craniofacial defects: Methods and Indications 038 Shankar VH, India ...... 039 Medical Genetics in Craniofacial Clinical Practice: Indian Scenario

Takeshi"Cleft lip Mitsuyasu& palate treatment , Japan...... team in Kyushu University Hospital, Fukuoka Japan -Prenatal counseling and visiting on birth day - 040 Sunil Kumar Rout, India...... Cleft palate repair – Not just closing the gap - 041 Aysegul M. Tuzuner, Turkey...... 043 Cleft Lip and Palate Care, Our Clinical perspectives

Jesus Andrés Duque, Colombia...... 047 Deleare Approach For Cleft Lip And Palate Our Experience

Piranit Nik Kantaputra, Thailand ...... 048 Orofacial Clefting and Dental Anomalies -

4 10th World Cleft Lip, Palate & Craniofacial Congress

Siddaramappa Jagdish Patil, India...... 050 Genetic studies in orofacial clefts

Girisha KM, India...... 051 Approach to a case with orofacial cleft: Clinical genetics perspective

MirekEarly response Tolar, USA...... of human mesenchymal stem cells to mechanical force and supplyof oxygen – research 052 and clinical perspectives

Kazuto Hoshi, Japan ...... 054 Three-dimensional tissue-engineered cartilage using biodegradable polymers

Thomas"Clinical outcomeEngstrand, and Sweden...... bone regenerative effects from using calcium phosphate-basedimplants in 056 cranial and facial repair

Fares Kablan, Israel ...... 058 The Buccal Fat Pad Free Graft; A new free fat tissue graft in the oral cavity

Manlio Galie, Italy...... 070 Treatment of Congenital Malformations of the malformation surgery MauriceGuidelines Mommaerts, for patient specific Belgium...... jaw linedefinition with titanium implants in aesthetic, deformation and 071

Adi Rachmiel, Israel ...... 082 Management of obstructive sleep apnea in syndromic Patients

Jesus Andrés Duque, Colombia...... 084 Maxillary External Distraction Osteogenesis In Cleft Craneofacial Anomalies

Manlio Galie, Italy ...... 085 Management of TMJ Ankylosis and Micrognathia in Children

Ayanga GN, Mongolia...... 086 Cleft care in Mongolia

Abhay Datarkar, India...... 090 Effectiveness and Patients Satisfaction of One Stage Correction of Secondary Cleft deformity- Indian Scenario

Jalal Uddin Mahmood, Bangladesh...... 092 India Cleft Lip and Palate Management in a Rural Camp in Bangladesh

John Westine, USA ...... 099 International Foundation for children with cranio-facial disorders

Manlio Galie, Italy ...... 101 Celebrating the Past, Looking to the Future: New Educational Projects of the EACMFS

Parakrama Wijekoon, Sri Lanka ...... 102 Management of cleft lip and palate ; are we delivering the best? deformities OPThree Kharbanda, decades of India...... excellence in Cleft Orthodontics for complex Secondary cleft 103

Arif Yezdani, India ...... 104 Cleft Care Orthodontics

Orest Topolnitsky, Russia ...... 106 Specifics . of Orthognathic surgery patients with cleft lip and palate

Krishna Nayak US, India...... 109 Distraction of Cleft

Adi Rachmiel, Israel...... 110 Management of Maxillary cleft deficiency - Long term results -

Jong Woo Choi, Korea ...... 112 Current Issues in Cleft related orthognathic surgery - 5 10th World Cleft Lip, Palate & Craniofacial Congress

Lian Ma, China...... 114 The Key Points for the Good Final Result of Cleft Lip and Palate Patient

Alexander Hemprich, Germany ...... 115 "Diagnosis, treatment and outcome of patients with submucous cleft palate - a retrospective data analysis -

Adil Mamedov, Russia ...... 117 Surgical Treatment Of Children With Cleft Lip And Palate In Newborn Period

S. Anthony Wolfe, USA ...... 118 Re-thinking Bilateral Clefts

Domenico Scopelliti, Italy...... 119 Operation Smile Comprehensive Care approach: scientific and humanitarian vision -

Masaharu Mitsugi, Japan...... 125 Bone grafting using Auto-Tooth Bone

Sadique Hussain, India ...... 126 Secondary Alveolar Grafting with Tibial Graft – A simple technique -

Mzubanzi Mabongo, South Africa...... 127 Alveolar Cleft Grafts: Our experience

Krishna Shama Rao, India...... 135 Perspectives in the management of Complex Craniofacial Anomalies

Jong Woo Choi, Korea ...... 136 "One-piece frontoorbital advancement with distraction but without a bandeau for craniosynostosis -

SikkiLocalized Singh, “ Vertical South Relapse”Africa ...... Or Failure Of Eruption Of The Dentition In Bone Grafted Area Following 137 Orthodontic Correction In Young Patients With Cleft Lip Palate: Case Studies -

Martín Romero Maroto, Spain ...... 145 Transversal Problems In Cleft Patients-

Amish Mehta, India ...... 146 Craniofacial Deformities and Orthodontics: Perils and Plights: Are we Crying Wolf?-

Amin Samadian, USA...... 147 Social media in education towards cleft prevention

Hector Villegas, Mexico...... 148 Cleft prevention from patient doctor perspective -

Héctor Montoya...... 149 Orthodontist’s role in the treatment of patients with nonsyndromic cleft lip and palate. Importance of prevention

Marie M. Tolarova, U.S.A...... 151 Prevention as Solution of Global Health Issues Related to Cleft Lip and Palate -

Annamalai Thangavelu, India...... 168 Hemi Facial Hypertrophy – A Case Report -

Josep Rubio Palau, Spain...... 175 3D Planning In Craniofacial Surgery -

G.Distraction E. Ghali, OsteogenesisU.S.A...... versus Traditional Orthognathic Surgery in the Management of Craniofacial 187 Deformities -

R Ramesh, India...... 188 Challenges in airway management in children with craniofacial abnormalities-Case Capsules

Vijay Mathur, India...... 189 Evidence based chair-side preventive dentistry for cleft patients

CJ Venkatakrishnan, India ...... 190 A simple and novel splint to prevent reattachment of reconstructed auricle in microtia patients

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Koichi Satoh, Japan...... 191 Images and Measurements During Speech Using a 320-Row Area Detector CT

BalakrishnanAnatomical and D, Functional India...... basis of the production of the Spoken sound, in 193 Health and in Children with Velo Pharyngeal Dysfunction.

Vedhasorubini K, India...... 195 Perceptual and Instrumental Assessment of Speech parameters in Cleft lip and palate

Usha A Dalvi, India...... 197 Management Strategies in Restoration of Speech and Language in Children-

Thamizharasan S, India...... 199 Compensatory errors of Speech – Assessment and Management-

Ambethkar Suprent, India...... 201 Feeding Assessment - Prosthetic appliances for feeding and for speech correction

Julie Sandra, India ...... 202 Stressed Parents and Anxious child

Domenico Scopeliti, Italy...... 204 The SecondaryRhynoplasty in CleftLip and Palate Patients

Jin-Young Choi, Korea...... 211 Secondary correction of cleft lip nasal deformity

NicolaiComputer- Adolphs, assisted Germany...... craniofacial surgery – experiences of the interdisciplinary 202 Berlin Centre for Craniofacial Surgery

LahiruEvaluation Prabodha, of the Prenatal Sri Lanka...... and postnatal growth and associated congenital anomalies of cleft palate 214 children in Sri Lanka

David Tauro, India...... 227 Aesthetic Camouflage for Correction of Cleft Deformities Surgical Options and Outcomes

Yogesh Bharadwaj, India...... 228 Management of secondary zygomatico-orbital deformities

Olga Zangieva, Russia ...... 229 Presurgical orthodontic treatment of children with a bilateral cleft lip and palate

Mithun Naik, India...... 237 Modified 3D NAM appliance for the correction of shifted pre maxilla

Lam Chin Yan, Malaysia...... 242 Median Cleft Lip with Congenital Midline Upper Lip Sinus: A rare case report

Mahesh Kumar Mugria, Pakistan...... 243 Bilateral Cleft Lip Management By Bilateral Millard Procedure; Outcome Of 50 cases-

Aswath G., India...... 244 Palatal Ruguoscopy in Patients with Cleft Palate – A Pilot Study -

SatishA comparative Prathigudupu, evaluation India...... of complications of single and multiple stage 245 procedures in unilateral cleft lip, alveolus and palate patients -

Anubhav Jannu, India ...... 254 D-Cleft: an innovative method for feeding neonates born with Cleft palate

Arvind Sivakumar, India...... 258 Influence of NAM & GPP on UCLP patients in mixed dentition: A 3D Model analysis -

Sauvik Singha, India...... 259 Feeding considerations of cleft lip and cleft palate patients -

Satadru Roy, India...... 260 Comparison of Early Onset Nasoalveolar Molding With PatientsWho Presented for MoldingUpto1Year of Age 7 10th World Cleft Lip, Palate & Craniofacial Congress

Shailaja. R, India...... 262 Nasoalveolar Molding – The First Step Towards Rehabilitation

NavdeepThermographic Bhusri, Evaluation India...... of the Effect of Pre-surgical Nasoalveolar Molding on Lip Tension in 263 Unilateral Cleft Lip and Palate Infants Pre and Post Cheilorhinoplasty-

Bharathi K, India...... 278 Combo Flap Interposition In Recurrent Tmj Ankylosis -

Manay Roshini Srinivas, India...... 285 Modification of Ryle's Nasogastric tube -

UmangObservational Kothari, prospective India...... study to correlate incidence of cleft palate 289 fistula with anatomical variation of cleft palate -

Bala Jagannath Gupta .B, India...... 293 External for cleft nasal deformities- a case series -

UroofFollicular-Unit Rahmatullah, Hair Transplantation India...... to correct Cleft Lip Mustache Alopecia using a new Atraumatic 302 Technique for Hair Implantation -

MariaColombian Del Pilar,Air Force Colombia...... Cleft Lip And Palate (Happy ) Program. 10 Years 310 Changinglifes From The Colombian Skies-

Roselinda Ab Rahman, Malaysia...... 311 USM-Indonesia Cleft Charity Project

Murod M. Jafarov, Uzbekistan...... 312 International cooperation for improving in-country mission for children with cleft lip & palate in Uzbekistan-

Koichi Ueda, Japan...... 314 3D Computer assisted elastic models for cleft lip -

S.Rengasamy Iniyan, India ...... 316 Zygomatic Hypoplasia in Cleft Lip and Palate Patients– A Pilot study

Nirmal Tony, India...... 317 Unilateral Cleft Lip Correction - Millard Vs Delaire

Abdul Latif, Indonesia ...... 318 "Unoperated Clefts Teach Us A Lesson A Cephalometric Study

Anand Sekhar, India...... 319 A Multidisciplinary Approach in the Management of Patients with Cleft Lip and/ or Palate.

Karavanov D, Ukraine...... 320 The tongue flap for closure of oronasal fistulas.

Yasmin Mary Jose, India...... 322 Alveolar Bone Graftingin Unilateral And Bilateral Cleft- How We Do It

Kavitha, India...... 323 Controversies In Alveolar Cleft Reconstruction In Cleft lip and Palate Management

Eitan Bar Droma, Israel...... 330 Facial Reconstruction after Pre Maxillary Resection in a Bilateral Cleft Lip and Palate Patient- Case Report

Upasak Mukherjee, India...... 336 Surgical And Orthodontic Correction Of A Case Of UCLAP

MurodAge aspects M. Jafarov, in application Uzbekistan...... of preoperative orthodontic treatment using the device NAM 340 for children with cleft lip and palate in the Republic of Uzbekistan-

SungWoonLong-term effect On, S.Korea...... of maxillary protraction using facemask and miniplate anchorage in cleft lip and palate patients: 344 a long-term follow-up study - 8 10th World Cleft Lip, Palate & Craniofacial Congress

Arul Prakash.P, India...... 345 Compliance of pregnant women of low socio economic group with peri-conceptional folate therapy

Sreejee Gopalakrishnan, India...... 346 Cleft Lip And Palate: Prenatal Diagnosis, Prevention And Treatment-

SrinivasanPrevalence OfNN, Superstitious India...... Beliefs As An Predisposing Factor For Cleft Lip,Palate And Other Birth Defects 362 Among Expectant Mothers And Their Parents-

R.Manonmani Pavithra, India...... 370 Gestational smoking and Alcohol abuse as etiological factors for cleft lip and palate. a growing concern in india-

China-Li Zengjian, China...... 374 Association of DHFR and 10q25.3 with Non-syndrome Cleft Lip with or without Cleft Palate in Northern

Wu Nan, China ...... Association study of candidate genes ABCA4 and MAFB with NSCL/P in Northeast Han Population 375 Deepak C, India ...... 377 heterogygous mutation of Arhgap29 gene in Indians-

Jiji Pamila Rossi, India...... 386 genetics in clefting-

Adebola,Team Organization Nigeria...... and Standard of Cleft Care in Nigeria: Charting a way forward for provision of 387 comprehensive cleft care-

Ramasamy.M, India ...... 389 "Naso alveolar moulding : after surgery???-

Kanmani Preethi, India...... 392 Distractor - A Magic Wand For Cleft Patients-

NatalieLongitudinal Loomans, and cross-sectional Belgium ...... model analysis of transverse maxillary growth in 399 cleft patients: comparison of two treatment strategies-

K.Sriraman, India...... 401 Cleft Maxillary Deficiency Addressed With Anterior Maxillary Distraction And Dental Implants – A Case Report-

Shambhavi"Effectiveness Singh, of intravenous India ...... haemocoagulase on haemorrhage control in bi-maxillary orthognathic surgery 402 in cleft lip and palate patients: A prospective, randomised, controlled, double-blind study-

JagadishPatil, India...... 404 Utility of Cortico-Cancellous iliac crest bone graft in management of Alveolar Cleft-

Yadav"Efficacy Abhilasha, of alveolar India bone ...... grating with autologus PRP and without PRP in cleft alveolus 413 patients- A Comparative Study-

Somuri Anand Vijay, India...... 414 An Evaluation of autogenous bone grafting with PRF in alveolar cleft patients

Nalini Sailaja, India...... 422 Donor Site Morbidity Associated With Anterior Iliac Crest Harvest For Alveolar Cleft Bone Grafting-

Vivek G, India...... 423 Management Of Premaxilla In Bilateral Cleft Lip And Palate-

ShankarPadmasree, India...... 424 Prosthodontic Aspects Of Cleft Lip And Palate : A Review -

Manikandan Somasundaram, India...... 425 Oral Health - Related Quality Of Life & Dental Caries Status In Children With Orofacial Cleft. An Indian Outlook -

V.K. Balakiran, India...... 436 Dental Caries In Patients With Cleft Lip And Palate - Predisposing Factors And The Role Of Prevention

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MS Saravana Kumar, India...... 445 What we see commonly is not normal always- Tongue Tie a Midline Defect

Arun V, India...... 446 "Know it early or late" - psychological effect of prenatal diagnosis of cleft lip and palate - A systematic review

Harsh Pipalia, India...... 456 Computer assisted Morphometrics in complex cranial vault reconstructive surgery

Sarika K, India...... 458 New treatment modality for maxillary hypoplasia in cleft patients - Review

FarzaliyevThe importance Ismayil, of using Azerbaijan 3D cephalometry ...... in planning reconstructive surgery with 465 craniofacial deformities

Mahendra Maharjan, Nepal...... 467 Rare Craniofacial Clefts - Our Experience

R.Murugan, India...... 468 A journey of surgery in crouzon syndrome-3 to 30 years

V. Suresh, India...... 469 Checklist Manifesto for Craniosynostosis correction

Kavitha Devi, India...... 476 Selective osteotomy during growth for apertognathia in a Crouzon’s child - A case report

Sam Raj G, India...... 477 Surgical Management of Late Reporting Pruzansky Grade III Hemifacial Microsomia

Yunus Amin, India...... 484 Transmogrification - An Ongoing Case Of Hemifacial Hypertrophy

Sathish Vashista, India...... 486 Efficacy and success of structured collagen as an Interpositional graft in the treatment Temperomandibular joint

Ravi Kumar M P, India...... 488 Post Ankylotic Deformity Correction- Report of 2 Cases with Different Surgical approach

R.K.Sathishkumar, India...... 489 Current approaches in the orthodontic management of cleft lip and palate

HimijaEvaluation Karia, of variation India...... in dimensions of sella turcica in patients with cleft lip and palate as compared to 497 Class I controls: A pilot study

Umarevathi Gopalakrishnan, India...... 500 Screw Design For Cleft Cases

SangeethaEvaluation ofDuraisamy, skeletal and India...... dental changes followed by maxillary expansion 508 using NITI palatal expander in patients with unilateral cleft lip and palate

Harsh Pipalia, India...... 510 Computer Assisted Morphometrics In Complex Cranial Vault Reconstructive Surgery

YoshihideMaxillary distraction Mori, India...... osteogenesis for cleft lip and palate patients -Influence on stability and speech of 517 maxillary anterior segmental distraction osteogenesis (MASDO) with rigid external distraction (RED) system

Manikandhan R, India...... 518 Morphometric distraction for mandible inasymmetry corrections

J A Nathan, India...... 519 Nasoalveolarmoulding - Contemporary approach for cleft infants

A.PRASZANTH, India...... Prevention Of Orofacial Cleft In The Developing World 520

Nizam Abdulla, India...... 521 Dentofacial Characteristics Of Repaired Cleft Lip And Palate Patients In Malay-Sia 10 10th World Cleft Lip, Palate & Craniofacial Congress

J.Abhishek, India...... 523 Niti Palatal Expander - When & How???

A.M.SHAKEEL, India...... 524 Myofunctional Dental Preparation Before Bone Grafting

Ami MAENO, Japan...... 525 Clinicostatistical investigation on cleftlip and/or palate patients in the Kyushu University Hospital

Amirthaa Varshini, India...... 527 Hemi-facial hypertrophy with trismus-abstract

Ankush Sune, India...... 528 Furlows Palatoplasty

Anuj Chauhan, India...... 529 My acquaintance with cleft patients in one year of residency

M. Arun, India...... 530 Modified Le Fort I osteotomy along with secondary Alveolar grafting for a Cleft facial deformity case

Ashish Prasad, India...... 531 Orthodontic management of excessive incisor display of an adult bilateral cleft lip and palate patient

Bhuvaneswari .M, India...... 532 Modified presurgical orthopaedics - NAM (Nasoalveolar Moulding)

Byoung Moo Seo, Korea...... 533 Application of Fisher method for cleft lip patients

Catherine S. Chandran, India...... 534 Saethre - Chotzen Syndrome: A rare congenital disease in a young South Indian Male

Nambinayaki.E.M, India...... 535 TECHNIQUES IN CLEFT OSTEOTOMY

Evan .A. Clement, India...... 536 Alveolar graft before and after maxillary expansion in the cleft lip and palate patient

Geetha Sridharan, India...... 537 Management Whistle Deformity in bilateral Cleft lip

Hideto Imura, Mongolia...... 538 Project of Gene banking for Cleft Lip and/or palate patients in Mongolia

JahamgeerGive Comfort Badusha, For Your India...... Implants ! - A Comprehensive Case Report On Dental Implant Rehabilitation On 539 Illiac Grafted Maxilla

Erdenetsogt Jargaldavaa, Mongolia...... 540 Velopharyngeal function following Mongolian method for primary palatoplasty

Joji Alex, India...... 542 A systematic review on Rigid external distractors in Cleft patients:

Adam KB, Malaysia ...... 543 Distraction Osteogenesis: Treatment Option For Facial Cleft Repair

Young-Wook Park, Korea...... 545 Long-term Follow-up of Early Cleft Maxillary Distraction

M. LAVANYAA, India...... 547 Correction Of Nasal Septal Deviation In Cleft Lip Patients - A Comparison Of Two Techniques

Manoj Kumar Musunuri, India...... 549 Tessier’S Classification Of Clefts And Surgical Treatment Plan For Each type

Manu Prasy, India...... 550 Orthomorphic Correction of mandibular Asymmetry with Reverse sagittal split Osteotomy- A Case Report

Mara Pilmane, ...... 551 Characterization of common tissue events in cleft lip palate affected tissue in children 11 10th World Cleft Lip, Palate & Craniofacial Congress

Mary Sheloni Missier, India...... 553 EFFECT OF NASO ALVEOLAR MOULDING ON DENTO ALVEOLAR ARCH

Nataliya Tetrueva, Russia...... 554 Rare cases of congenital anomalies of the nose

S.Nirmal Raj, India...... Bilateral Cleft Lip Repair With Delaire & Bob Mulliken Techniques 556 Nithya Jagdish, India...... Dermatoglyphics - a diagnostic tool 557 Rajesh Murugan, India...... Morphologic variability in nonsyndromic operated patients affected by cleft lip & palate : Cephalometric study 558 dentition stage V.K.Facial Gopinath, profile and Malaysia...... maxillary arch dimensions in unilateral cleft lip andpalate children in the mixed

559 Reetesh Navani, India...... Alveolar bone grafting 560 Remmiya Mary Varghese, India...... Benefits And Limitations Of Nasoalveolar Moulding Appliance (Nam) : A Scientific Review 561 S.I.P. Sheik Arifa, India...... Dental And Skeletal Asymmentries For Orthognathic Surgery 563 Sivaiah Sarnu, India...... VERMILION FREE BORDER RECONSTRUCTION A NOVEL TECHNIQUE 564 Stephan Rajkumar, India ...... OBSTRUCTIVE SLEEP APNOEA IN THE CLEFT POPULATION 565 S.V.HAYMENTH, India...... PRENATAL DETECTION OF CRANIOFACIAL ANOMALIES 566 Swetha Sridharan, India...... The GOSLON Yardstick in Patients With Unilateral Cleft Lip and Palate- A SYSTEMATIC REVIEW 567 Toko Hayakawa, Japan...... The trial telepractice for cleft palate speech between Mongolia and Japan 569 Veerasankar S, India...... Naso – Alveolar Moulding 570 Venkateswaran Ananthanarayanan, India ...... Prenatal Diagnostic Markers Of Cleft Lip And Palate 572 Yukiko Morita, Japan...... A case of extremely low birth weight infant with cleft lip and palate (2nd report) 573 Gauri Gupta, India...... Iliac bone graft and osseointegrated implants 574 Narendra Reddy Chittamuru, India...... Role of 3D Printed skull models in complex craniofacial surgeries 575 Manay Roshini Srinivas, India...... 577 Geometric Compensations

Priya.B, India...... 578 Evaluation Of Bone Volume In Secondary Alveolar Bone Grafting In Cleft Lip & Palate Patients

Misato Katayama. Japan...... 580 A case of a rib and costochondral complexgraft

Sruthi S, India...... 582 Debulked Simplified Expansion & Alignment Appliance For Cleft Lip & Palate Patients

12 10th World Cleft Lip, Palate & Craniofacial Congress

ICPF Presidential Oration

The ICPF Presidential Oration is the highest honor bestowed upon by the ICPF congresses & are delivered by legendary, stellar & most distinguished surgeons of our times in recognition of their exemplary expertise, outstanding accomplishments & exceptional abilities to lead, inspire, and collaborate with broad impact.

Global Craniofacial Care: 45 Year Personal Perspective

Kenneth E. Salyer Founding Chairman and Director, World Craniofacial Foundation, , , USA

Introduction

As a Pioneer in America and internationally in infant craniofacial surgery an early awareness of the need to provide care for all patients became a personal mantra. Globally the majority of children with craniofacial deformities living in developing countries do not have access to good treatment. There is a lack of knowledge, leadership, infrastructure, and support of craniofacial teams in the developing world. This is Primarily due to lack of funding where primary care is inadequate. The World Craniofacial Foundation (WorldCF) is a 501c3 (USA) tax exempt foundation dedicated to providing access, treatment, and education through the development and encouragement of multidisciplinary teams. This isPlan an update on these activities. ●●

Establish centers for craniofacial and cleft care by providing support and ●● teaching in developing countries. Provide expansion of knowledge in craniofacial care and related specialties database.through teaching, educational programs, scholarships, fellowships, visiting professorships, research studies, and a worldwide patient information ●●

Support and facilitate recognized leading craniofacial centers to increase their care of indigent patients. 13 10th World Cleft Lip, Palate & Craniofacial Congress ●●

Create, facilitate, and provide financial support, as seed money for the development of sustainable integrated craniofacial teams within existing ●● hospitals, universities, private hospitals, and other medical centers. Motivate, stimulate, and provide opportunities for careers in craniofacial ●● and cleft surgery. Provide and facilitate public awareness as well as medical education about Resultsreconstructive plastic surgery, emphasizing craniofacial surgery.

Successful programs, scholarships, visiting professorships, and update of developing multi-disciplinary centers will be presented. Failures, problems, and concerns will be reviewed. The World Craniofacial Foundation has a 25 year history of programs which will be presented as time allows. The goal is expansion of existing centers, creation of new centers, teaching conferences, negotiations with governments, and partnering with other foundations to provide education of ongoing, multidisciplinary, integrated care as the model. Recommendations for the future will be presented.

14 10th World Cleft Lip, Palate & Craniofacial Congress

Prof. David S. Precious Memorial Oration

Prof. David S. Precious, DDS, MSc FRCDC, FRCS, CM, Dean Emeritus, Professor of Oral & Maxillofacial Surgery, Dalhousie University, Halifax, Nova Scotia, Canada, Former Chair, ICPF, was an internationally- recognized leader in the field. He was widely renowned for his humanitarian outreach missions having donated his time, money and skills to advance and support surgical care for children with cleft lip & palate.

David Precious - A Celebration

Anthony Markus Consultant, Maxillofacial Surgeon, Head of Department Maxillofacial Surgeon, Poole Hospital and Royal Bournemouth Hospital, Director, Dorset Centre for Cleft Lip and Palate, UK

This oration is a celebration of David Precious’ life and contribution to surgery, maxillofacial surgery and the management of cleft in Canada and internationally. It will focus on the training that we both received with Professor Delaire in and his influence on our practice of managing clefts. It will also consider his many achievements and his guidance in establishing ICPF

15 10th World Cleft Lip, Palate & Craniofacial Congress

Prof. M.S. Ramakrishnan Memorial Oration

Prof M.S. Ramakrishnan, M.S., F.A.C.S, Pediatric Surgeon, Former Professor of Pediatric Surgery, Madras Medical College, Institute of Child Health, Chennai, India, was the first surgeon to hold a professorial chair in South India in Pediatric Surgery. Child’s Trust Hospital is a standing monument of his dream with all sub-specialties of pediatrics and teaching program.

Pediatric Plastic Surgery becomes Craniofacial Surgery, thanks to Paul Tessier

S. Anthony Wolfe Chief, Division of Plastic Surgery, Miami Children’s Hospital Miami Florida 33155, USA

For many years, maxillofacial surgeons toiled on the jaws, and neurosurgeons on the skull and its contents. The area between—the orbital cavities---remained a surgical no man’s land until the contributions of one man- ---Paul Tessier, who single handedly created a new addition to pediatric plastic surgery---craniofacial surgery. One of the things made possible by Dr. Tessier’s new principles was movement of the dystopic orbital cavity, upwards, downwards, together, apart. Correction of obital dystopias will be discussed in detail .

16 10th World Cleft Lip, Palate & Craniofacial Congress

Prof. C.R. Sundararajan Memorial Oration

Prof. Cheyyur Ramaswamy Sundararajan, MS, Mch, is a doyen in Plastic Surgery in India. He was an outstanding surgeon and a legendary teacher & guide for over four decades. He headed the Plastic Surgery Dept. at Madras Medical College, Chennai, India and started the M.Ch Plastic Surgery course here. For his contributions to the field of Plastic Surgery he was awarded the “Dr. BC Roy Award" in 1989, the highest medical honor in the country. EXIT (Ex Utero Intrapartum Treatment) Procedure: A Modality for the Airway Management of the Select Cleft and Craniofacial Patient Population

G.E. Ghali Chancellor & Dean, LSU Health Sciences Center, Gamble Professor & Chairman Oral & Maxillofacial Surgery, Shreveport, Louisiana USA

Background & Purpose

With further refinement of prenatal ultrasound (US) techniques providers of the cleft and craniofacial population are routinely approached to aid in the delivery and care of patients with severe retrognathia, mandibular dysgenesis, or an otherwise compromised airway. Without planned intervention in a controlled team setting many of these neonates are unlikely to survive the prenatalnatural birth imaging. process. Therefore, it is critical to plan and prepare for situations where a compromised airway is likely to be encountered as predicted by

The EXIT procedure ensures a controlled environment on uteroplacental bypass (enabling maternal-fetal gas exchange at the placental interface) while tracheal compression or occlusion is addressed. The purpose of this presentation is to prepare the participant to recognize a compromised airway, coordinate a team approach for addressing the airway, and provide a detailed treatment/surgical algorithm to competently perform the EXIT procedure. Case examples, each with a unique presentation and outcome, will be discussed. A standardized approach for the evaluation of fetal imaging and17 10th World Cleft Lip, Palate & Craniofacial Congress treatment of a compromised airway will be reviewed. The EXIT procedure requires a team approach with coordination and clearly defined roles. Important considerations from the perspective of all team members (obstetrician, neonatologist, anesthesiologist, and craniofacial surgeon) will be discussed. A detailed surgical airway algorithm will be presented with special attention to theMain instruments Objectives needed of Presentation: and the surgical approach taken.

01. Each delegate will be able to recognize a compromised airway as seen in fetal imaging studies in need of a possible EXIT procedure. 02. Each delegate will be able to describe the airway algorithm and the necessary clinical and surgical steps necessary to ensure a safe smooth delivery. 03. Each delegate will be able to describe the EXIT procedure and its various components and the roles of the team members (obstetrician, neonatologist, anesthesiologist, and craniofacial surgeon). when the need presents. 04. Each delegate will be better prepared to perform this life-saving intervention

18 10th World Cleft Lip, Palate & Craniofacial Congress

Prof. Kawai Memorial Oration

Prof. Tsuyoshi Kawai, Professor Emeritus, Aichi-Gakuin University, President, Japanese Society of Oral and Maxillofacial Surgeons, was senior advisor and responsible for many activities of the Japanese Cleft Palate Foundation since it was founded. He established the Cleft Lip and Palate center at Aichi-Gakuin University Hospital and spared no effort to improve the care for cleft lip palate patients.

Orthognathic Surgery and Alveolar Bone Grafting in Cleft Lip and Palate Patients

Adrian Sugar Consultant Cleft and Maxillofacial Surgeon, Morriston Hospital Swansea, UK, Clinical Director, The Welsh Centre for Cleft Lip and Palate, Consultant/Senior Lecturer in Cleft and Maxillofacial Surgery, Cleft Unit and Maxillofacial Unit, Morriston Hospital, ABM University Health Board and Swansea University, Morriston, Swansea SA6 6NL, Wales, UK

The literature is full of studies describing major relapse rates in orthognathic surgery on patients with cleft lip and palate and many other serious complications are well known. This oration describes our experience over 30 years in the Welsh Centre for Cleft Lip and Palate (CLP). For the first half of that period, all children with an alveolar defect were assessed in a multidisciplinary clinic at 8 years of age and operated on subsequently mostly between the age of 8 – 10 years. In our first ABG study, this produced good outcomes in 94% of cases but the worst results were in those operated on late and in BCLP patients (sometimes the same). In the second half of this period, all children with an alveolar defect were assessed at 7 years of age. In our second ABG study, good outcomes were achieved in 97% of cases. In our third ABG study, only 10% of these children needed orthodontic preparation for ABG and at least 70% achieved eruption of the permanent canine tooth on the cleft side, 60% spontaneously. During this period, those patients with repaired cleft lip and palate who needed a maxillary advancement have always had a successful previous ABG and therefore Le Fort 1 oeteotomies are carried out in one piece, with pre-op. orthodontics, radical mobilisation, internal fixation with 4 mini-plates and anterior iliac crest block bone grafting. In our first study of these patients, the maxillary position was more stable that those having this surgery who were non-19 10th World Cleft Lip, Palate & Craniofacial Congress cleft. In a second study comparing this cleft cohort and a cohort of cleft patients who underwent internal distraction osteogenesis to advance the maxilla, the osteotomy group were more stable. We also report in this presentation the results of 2 further studies on very large maxillary advancements and the impact of this surgery on speech. Only a small number of CLP patients have significant naso-maxillary deficiency but these do often benefit from a Le Fort II osteotomy. The results of such procedures are described.

20 10th World Cleft Lip, Palate & Craniofacial Congress

Prof. J.G. Kannappan Memorial Oration

Prof J.G.Kannappan was a former Professor and Principal of the Government Tamil Nadu Dental College and Hospital, Professor Emeritus of the TN Dr MGR. Medical University, Chennai, India. He had been teaching dental and medical students in a career spanning for more than half a century. In 1980s he has been known for his unique classification of dental appliances for the cleft palate patients in India.

Management of patients with cleft lip and palate – The Orthodontist’s Perspective

NR Krishnaswamy Vice Principal, Professor and Head, Dept. of Orthodontics Ragas Dental College and Hospitals, Chennai, India

Cleft lip and palate is by far the most commonly occurring anomaly. It is reported to occur in about 1 in 800 children. The cause is multi factorial and birththe treatment till late adulthood. requires a multi disciplinary approach. The orthodontist plays a pivotal role as a team member and is probably the only specialist involved from

The role of orthodontists include neonatal orthopedics including fabrication of feeding plates and NAM, growth modification in the mixed dentition stage to offset the skeletal imbalance, expansion prior to or after alveolar bone grafting and definitive orthodontic therapy including pre/post presentation.orthodontics in patients requiring orthognathic surgical intervention. The role of orthodontics and the challenges in each of these stages is highlighted in this

21 10th World Cleft Lip, Palate & Craniofacial Congress

Prof. U.S. Nayak Memorial Oration

Prof. Dr. U.S. Nayak, MS., MCh., FRACS (Melbourne), Plastic Surgeon, Prof. & Head of Plastic Surgery, Madras Medical College, Chennai, India, was one of the greatest surgeons, whose word was considered ultimate in Plastic Surgery. He was one of the first from India to be trained under Sir Benjamin Rank, Professor of Plastic Surgery, University of Melbourne. A respected teacher and a surgeon par excellence, he has done monumental work and inspired and trained a large number of surgeons. Lip to Cranium

Balaji SM Director & Consultant, Balaji Dental & Craniofacial Hospital, Teynampet, Chennai, India

The Rehabilitation of Cleft surgery and Palate is an art and Science. It is a science in the domain that the governing principles of the surgery, the design of the flaps, healing principles, and speech corrections are based on basic scientific tenants of anatomy, physiology, surgery while it is considered as an art as it requires and demands the insight of esthetics, patient and caretaker’s psychology. The training of cleft surgery for a maxillofacial surgery requires a certain degree of training and insight in to Plastic Surgery while a for a Plastic surgeon, training and insights in to the concept of occlusion, basal cranial bone refinementIn my becomes earlier part a necessity of professional to provide training, better solutionI had begun for a cleftmy training patient. in

Cleft surgery in its basic format – The repair of cleft lip. Through my earlier experience with Millard’s Technique and nearly all of its modification, I began to understand the functional component – The importance of oral musculature, its orientation, direction of cleft. Once I started to use the acquired knowledge in subsequent cases, I was made aware of the importance of muscle handling, and direction of attachment. Further refinement was gained by understanding the unique morphology of Indian lips which had significant ethnic and cultural distinct difference from standard western procedure. The Classic Indian Nose morphology played a significant role in dictating the design of cleft lip repair incision design. The minor modification of adding one single extension of the classic C arm in to the nose helped to have a more predictable pout with minimum scar and good healing. With more understanding and experience with cleft lip, slow and gradual training22 in cleft palate commences. With training, one offers more services 10th World Cleft Lip, Palate & Craniofacial Congress

than to merely fill the gap with bone or bone like material. In my earlier days of professional practice, I used bone grafts from various sources – iliac crest, costochondral bone grafts, bone shavings from long bone and some time from jaw bones, all depending on the age of the patients. With the advances osteogenesis.in technology and deeper understanding of cleft genesis, I now use tissue engineering products and bone generating technology such as distraction

The correction of cleft palate was a challenge – to understand the mechanism of cleft palate formation was necessitated. Introduction of dynamic field reassignment theory of cleft palate embryogenesis in early 2000, created new insights in to the clarity for cleft palate treatment. The sequential correction with age, early intervention and growth guidance with orthodontics and timing of second palatal repair helped to avoid additional future complication. Guided eruption and pre-surgical, intra-surgical and post-surgical orthodontics helped to harness the full potential of growth centers and growth spurts. At this juncture, I learned the importance of timing of the primary and second surgery of palatal correction. The basic problem in cleft palate is the tissue deficiency and its quality. In practical surgical application, it turns out to be a void approach since irrespective of cleft severity, majority of existing methods relies on transposition of deficient and hypoplastic tissues – and I learnt a method to beat this factor nearly 10 years back. The timing of skeletal maturation, consideration of plasticity of bone are vital parameter With practice and training, I learnt the average Indian’s timing of these procedures. The speech management for cleft palate, especially with diverse language culture was a challenging. Understanding the science and mechanism, including reading of the velopharyngeal function through various modalities helps to understand the speech mechanism. Speech assessment is very difficult because apart from nasality and some qualitative analysis, its evaluation is highly subjective. Moreover, speech evaluation in various Indian language groups escapes comparison. Once a mastery of this is achieved, it could be effectively translated in to clinical practice. Call it “intitution” or “training” or “Algorithm” based learning – it is useful for effectively formulating a customized treatment schedule for the patient. The use of rhBMP-2, a novel bone formation stimulating protein was a game changer. It provided an opportunity for self generation of bone in autologous fashion. The newer, large scale experiences as publications in International literatures helped to refine the technique for Indian patients. Numerous patients have been sparred of additional bone graft procuring23 10th World Cleft Lip, Palate & Craniofacial Congress surgeries – that causes scar and has potential for morbidity. The rate of bone formation is relatively faster, much predictable than the normal, conventional technique. With a relative mastery of the cleft lip and palate, challenge comes with revision of “blotched surgeries”. The complication of thick scars, effect of hypoplastic bone, non-coordinated growth of jaw bones and teeth, improper basal cranial bone growth pose significant challenge. Adding woe to this is the resultant orthodontic mal-alignment. Despite some clever achievements in cleft surgery evidence in favor of modern current approach is rather limited and, to some degree, it is based on criticism of old methods. Advocates of early and radical cleft procedures argue that inhibitory effects of surgical trauma and scars are noted after many and more operations, including conservative methods and so-called sparing procedures. What is more, some of them believe that even if, as a result of earlier treatment, the percentage of patients with midface hypoplasia is greater, treatmentthere is still as possibility far as possible. for orthognathic operations. In other words, in order to gain interim success, they are inclined to shift the responsibility and burden of

The value of such treatment ought to be verified by outcomes following growth completion. Unfortunately, in India evaluations based on large cleft cohorts and long-term observations are rather sparse indeed, and due to the lack of precise measurements, record keeping and publication of findings are seldomly done. Hence, continuous disputes and controversies are rarely based on unquestionable scientific proofs, and it definitely is not Evidence Based Medicine. In this situation, developing one’s own Evidence is necessary. Correction of all these soft and hard tissue defects would require a deeper understanding how these defects were formed in the first place. Once that is understood, correction of the same based on established scientific foundation needs to be established. To achieve this, imaging studies, especially 3D and CT scans are vital. The dimensional and morphological deviations are easily visualized. Growth prediction softwares provide vital help in this regard. But the values used in these are not based on Indian growth pattern but rather on Western parameters. Understanding the growth pattern of Indian and Western population, soft tissue profiles and basal bone difference would help to effectively translate the growth prediction. Staged reconstruction of the 24midface might be required in severe cases. 10th World Cleft Lip, Palate & Craniofacial Congress

With sufficient expertise gained, natural progression of referral cases brought in the need to learn more of upper third of correction and rare Tessier’s cleft correction. Understanding the ocular, malar and nasal bone morphology helped me to treat these complex cases. For this, I needed to learn the fine art of Distraction Osteogenesis, usage and harnessing the various vectors as well as managing the complicated effect of vectors. Synchronizing the bone growth vectors, occlusion and bone expansion with use of Distraction Osteogenesis is by itself an art- equivalent to rope walking. A mistake in the bone expansion and consolidation will yield disastrous results – from failure of bone formation to distorted growth, coupled with deranged occlusion. With experience and deeper understanding of the fundamental philosophies of treatment, an effective, customized treatment guideline could be drawn. With refining of the learning curve, I faced another important cleft rehabilitation requirement – Cleft rhinoplasty. As the development of nose is highly linked with the growth of the midface and nose, most of the cleft patient faces problems with the nose, once the final face is established. Cleft rhinoplasty is a sub-specialty by itself. Given the complex nature of Indian nose and its substantial difference with Western patients, rehabilitation posed a difficult challenges. It requires patience to understand, implement and master the art. Although an ideal management of clefts is unrealistic, the use of relatively harmless and sparing methods enables achievement of satisfactory speech, appearance and midface development. As the patients appearance depends on numerous factors, such as primary or delayed nose repair, alveolar bone grafts or secondary lip and nose corrections no single or multi-precise measurements or parameters are suggested But largely, subjective evaluation of the outcomes are used in daily clinical practice. In my experience, the outcomes were regarded as satisfactory. Despite reasonably good outcomes, the prospect for essential improvement in complete cleft repair seems to be rather vague and determined mostly by tissue deficiency. Judging from literature and my own observations, no more should be expected from various palate or vomer flap transpositions, because there is a deficit of reconstructive material. As always I am in the look for newer experience with advanced materials or technology to improve the existing technology and knowledge.

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ANAMAYA IndoPacific Cleft Prevention Program: Shifting the attention to prevention of cleft lip and palate

Sai Shamini1,2, Shreekala Kandapalanivel2, Elavenil Panneerselvam2,3, Karthika Panneerselvam2,4, Krishna Kumar Raja. V.B2,3, Manikandhan Ramanathan2,5, Marie M. Tolarova6, 7 1Madha Dental College & Hospital, Chennai, INDIA, 2ANAMAYA Indo-Pacific Cleft Prevention Program, Chennai, INDIA, 3SRM Dental College, Chennai, INDIA, 4 Karpaga Vinayaga Institute of Dental Sciences, 5Meenakshiammal Dental College, Chennai, INDIA,6Cleft Prevention International Foundation, USA 7University of the Pacific, San Francisco, California, USA

Introduction

Incidence of cleft lip and palate anomalies rising worldwide. It is being tackled by multidisciplinary treatment or, at least, corrective surgeries. A course of this treatment and rehabilitation is a long and challenging journey- physically, mentally and psychologically - for both the patient and the family. Also, treatment and non-medical expenses related to care for an individual affected with a cleft represent a large amount of money being spent. Regardless of improvement of surgical treatment and help by numerous NGOs, this approachMethods is not resolving this health issue satisfactorily.

A change in approach to meet this challenge is the need of the hour. There is an urgent need to redirect our focus to prevention. Etiopathogenesis of orofacial clefts, which is complex, multifactorial, complicated by ethnic and geographical factors, needs to be better understood. Research points towards professionals.potential benefits of folic acid, which may substantially reduce the baby’s risk for a facial cleft. This knowledge needs to be reinforced even among health Results

population.We are presenting and discussing ANAMAYA’s experience in awareness building among health professionals and different categories of general 26 10th World Cleft Lip, Palate & Craniofacial Congress Discussion

We strongly believe that bridging this knowledge gap is an important step to prevention – thus, focusing on the march ahead towards bringing a cheer on the faces of every newborn baby and mother. ANAMAYA has been conceived and led by Dr. Shreekala in association with Dr. Marie Tolarova, a world pioneer in the field of cleft lip prevention. It has been committed to prevention in the India since 2010. The main focus of this NGO is in creating awareness to prevent cleft lip and palate andConclusion other birth defects.

We are driven with a mission to create a “Cleft Free India”, which our name ANAMAYA denotes in Sanskrit.

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Review of studies on a role of folic acid in etiology and prevention of cleft lip and palate

Karthika Panneerselvam1,2, Shreekala Kandapalanivel2, Elavenil Panneerselvam2,3, Sai Shamini2,4, Manikandhan Ramanathan5, Krishna Kumar Raja V.B3, Marie M. Tolarova6,7 1Karpaga Vinayaga Institute of Dental Sciences, Madurantagam, INDIA, 2ANAMAYA Indo-Pacific Cleft Prevention Program, Chennai, INDIA, 3SRM Dental College, Ramapuram Campus, Chennai, INDIA, 4Madha Dental College & Hospital, Chennai, INDIA, 5Meenakshiammal Dental College, Chennai, INDIA, 6Cleft Prevention International Foundation, USA, 7University of the Pacific, San Francisco, California, USA

Objective

Folic acid (FA) is critically important for cellular proliferation and functions. Association of FA deficiency and non-syndromic cleft lip and palate (NSCLP) has been suggested by several studies. The objective of this presentation is to review: (1) studies suggesting that FA deficiency can result in NSCLP; (2) studies on allelic variants in folate pathway genes; (3) literature describingMethods various prevention methods.

An extensive search of electronic database was performed. The search words included FA metabolism, prevention, folate-related genes, prevention programs, and India. Studies on influence of FA supplements during pregnancy andResults FA food fortification were also included.

The search produced over 40 articles that were reviewed. Association of deficiency of FA during pregnancy and NCLP has been suggested. Various studies have been done on folate pathway genes and NSCLP. Some of the folate pathway genes that were studied include MTHFR, MTHFD, RFC1, MTR, BHMT and TS. Studies done in India have shown association of TGFB3 rs2300607 (IVSI+ 5321) gene variant and MTHFD1 1958G>A polymorphism with NSCLP. A study on supplementation of a high dose of FA periconceptionally showed a significant reduction of NSCLP recurrences. Another study on intake of recommended dose of FA periconceptionally showed decrease of first occurrences of NSCLP. In other studies, fortification of staple food with FA reduced birth prevalence of neural28 tube defects. 10th World Cleft Lip, Palate & Craniofacial Congress Discussion

The vast majority of studies suggest that polymorphisms of folate-related genes are associated with NSCLP. However, there is not a complete agreement. This could be not only due to a sample selection bias, but also because differences existConclusion between different ethnicities and locations.

At this time, more awareness about etiology of NSCLP and prevention by periconceptional FA consumption needs to be implemented among health care providers and in general population.

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The role of medical geneticist in prevention of cleft lip and palate

Viviana Armentano Head Department of Medical Genetics. University of Mendoza. Boulogne Sur Mer 683. CP 5500 Mendoza Argentina.

INTRODUCTION

A medical geneticist needs to be actively involved in a multidisciplinary management of a patient affected with orofacial cleft since his or her birth. A medical geneticist determines a correct diagnosis (isolated orofacial cleft or orofacial cleft as a part of a syndrome), calculates recurrence risk, and formulatesMETHOD a prevention approach.

With the help of a medical geneticist, patient’s family gathers data to determine, if this child is the first or if there are other family members already affected. By taking medical and genetic history, the medical geneticist is finding about lifestyle and diet of the parents of the patient (nutrition, work exposures to toxins, environmental factors such as cigarette smoke, alcohol, drugs, etc.). It is important to thoroughlythe mother, inquire feelings about of the shame health and of guilt. patient’s The mother at the time of conception and pregnancy. Child’s birth defect may have a negative emotional impact on medical geneticist should support the family from the start stimulating the mother-childRESULTS bonding and breastfeeding.

All the data will help to determine the risk of recurrence in this family and toDISCUSSION design a personalized preventive approach, if another child is planned.

The geneticist-physician should be prepared to explain probable causes that originate the clefts, the symptoms and data leading to correct diagnosis, treatment, prognosis, and prevention. At an educational level, the medical geneticist has a commitment to educate the public about risk factors for cleft lip and30 palate and how to avoid at least some of them in our environment. 10th World Cleft Lip, Palate & Craniofacial Congress CONCLUSION

The medical geneticist takes care of a child affected with cleft throughout the lifespan - starting at the time before pregnancy, during periconceptional period, and ending in adulthood. The role of medical geneticist is not limited to the individuals affected with a cleft, but expands to the patient’s family. Finally, the medical geneticist has to go beyond family limits to perform in a dynamic social role participating in prevention programs in the society.

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Preventive research on cleft lip and palate in Japan

Nagato NATSUME Professor, Aichi-Gakuin University, Executive Director, Japanese Cleft Palate Foundation, Executive Director, Japanese Medical and Dental Network, Secretary Treasurer, International Cleft Lip and Palate Foundation, Japan

We should concern not only treatment of cleft lip and palate but also prevention. I will present the new prevention methods resulted from our study and those related to the Hanoi Declaration, the declaration aiming at prevention of the abortion due to a prenatal diagnosis of fetus with cleft lip and palate, from my over-30-year-experience in the etiology, animal testing and genetic research. The Hanoi Declaration declared in Hanoi, Vietnam in 2013 is under being evolved and I will additionally present the work targeting at establishment of the guideline which protects the human right of fetus with cleft lip and palate and gynecologists and will be out in 2018 in Germany.

32 10th World Cleft Lip, Palate & Craniofacial Congress

International Collaboration for Cleft Lip and/ or palate from Japan

Nagato NATSUME Professor, Aichi-Gakuin University, Executive Director, Japanese Cleft Palate Foundation, Executive Director, Japanese Medical and Dental Network, Secretary Treasurer, International Cleft Lip and Palate Foundation, Japan

Japanese Cleft Palate Foundation (JCPF), which was established in 1992 for aiming at caring of the patients and their families in Japan, has pursed actively to perform medical and dental assistances to twenty countries in the world in cooperation with Japanese Medical and Dental Network. Two hundred volunteers, in every year, including oral maxillofacial surgeons, anesthetists, dentists, nurses and speech therapists have joined to the charitable operations and they have accomplished the medical and dental technical transfer with bringing equipment and medicines, some of which are donated by the company. . Some of the mission contributed to shipping donated second hand equipment and building the hospitals, operation ward, training center, the congenital anomalies monitoring center, and speech therapy room as well. The total expenses we have spent for this activities, including the donated materials cost, are estimated to thirty-two million dollars, which shows that JCPF is one of the biggest non-profit organization in the field of cleft lip and/or palate in Japan. Our wide and high quality of our activities were recognized by the Economic and Social Council, United Nations, as one of the members of Consultative Status In this relation, JCPF has accomplished various types of assistances to the developing countries not only in the medical and dental field but also in cultural exchange and making friendship. As a result, JCPF proposed the establishment and invited Honorary Consulate of the Socialist Republic of Vietnam, the Lao People’s Democratic Republic, the Federal Democratic Republic of Ethiopia and Mongolia. Now we are earnestly making the necessary process of establishing Honorary Consulate of the People's Republic of Bangladesh. 33 10th World Cleft Lip, Palate & Craniofacial Congress

In addition, we perform the gene banking named “World Cleft Bank”. Though we started from the medical related field as described, I would like to talk on my experience on our grass-route exchange program of the study concerning analysis of genes causing oral congenital anomalies.

34 10th World Cleft Lip, Palate & Craniofacial Congress

PTCH1 gene mutation in nevoid basal cell carcinoma syndrome with diverse clinical manifestations

Kangmin Ahn Department of oral and maxillofacial surgery, College of medicine, University of Ulsan, Asan medical center, Seoul, South Korea

Background

Gorlin syndrome is an autosomal dominant inherited disease that exhibits high penetrance and variable expressivity. Typical clinical signs are multiple basal cell carcinoma, keratocystic odontogenic tumor(KOT) and rib anomalies with/without cleft lip and palate, and mental retardation. In this presentation, Gorlin syndrome patients with PTCH gene analysis were reviewed. Patients and methods: Seven consecutive patients were referred to evaluate multiple jaw cysts. Male to female ratio was 3:4. All patients mutation.underwent multiple cysts enucleation under general anesthesia. Gene analysis was performed in four patients about patched (PTCH) tumor suppressor gene Results

Multiple jaw cysts were enucleated without segmental jaw resection. Basal cell carcinoma and cleft lip was found in one patient. Patched (PTCH) gene mutation was found in two patients. Multiple KOT recurrences were found inConclusion three patients. Hypertelorism was found in all cases.

PTCH gene mutation was found in Gorlin syndrome. Recurrence of the keratocystic odontogenic tumor was fond in 50% of the patients. Periodic follow-up is mandatory to monitor jaw KOT recurrence.

35 10th World Cleft Lip, Palate & Craniofacial Congress

Microarray analysis of global gene expression during palate development

Kaori Matsumura, Shinsaku Arai, Takeshi Mitsuyasu, Ami Maeno and Seiji Nakamura Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Japan

Introduction

Development of the palate comprises sequential stages of growth, elevation and fusion of the palatal shelves. Failure in these steps may result in cleft palate (CP). Since the palate of CP patient have not contacted, abnormalities of palatal elevation are considered one of the reasons for CP. Furthermore, in some CP cases, only soft palate does not contacted. It is suggested that there is differences of gene expression pattern between hard palate and soft palate during palatal elevation. In this study, we examined gene expression change in hard and soft palate duringMaterials elevation and Methods stage to investigate the pathogenesis of CP.

Timed-pregnant C57BL/6 J mice were used and the age of the embryos was determined by defining the day of appearance of the vaginal plug as E0.5. Total RNA was prepared from a pair of palatal shelves before elevation (E13.5), following elevation and contact (E14.5). Changes in gene expression between the three different stages were analyzed with Whole Mouse Genome Microarray (Agilent Technology). Gene induction or repression was considered significant ifResults the change in gene expression ratio between arrays were 1.5 or greater.

Large upregulated changes at E14.5 were seen in 1022 genes such as Foxn4, Mmp13 in soft palate, Krt20 Fgf21, Wnt11 in hard palate. Large downregulated changes were seen in 1018 genes such as Trim12, Trim67, Rdh19 in soft palate, Msx1, Etv5 in hard palate. These include genes involved in cellDiscussions prolifelation, differentiation, and intracellular signaling.

36 10th World Cleft Lip, Palate & Craniofacial Congress The gene expression pattern was different between hard palate and soft palate during palatal elevation. In soft palate, expression of the gene cluster participated in cell proliferation and cell adhesion were decreased. In contrast, these clustered gene expression were increased in hard palate at the same stage. Moreover, the gene expression involved in morphology of cardiovascular and ear were increased in soft palate during palatal elevation. It is suggested that these clustered genes are the possible candidates genes of cleft palate. Further studies about functional analysis of these genes are needed.

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Genetic Testing for Diagnosis of Craniofacial defects: Methods and Indications

Ashwin Dalal Head, Diagnostics Division, Centre for DNA Fingerprinting and Diagnostics, Hyderabad, Andhra Pradesh, India

Genetic testing refers to analysis of DNA in various forms like chromosome analysis in cytogenetic testing, DNA analysis in molecular genetic testing and protein/biomarker analysis in biochemical genetics testing. Craniofacial defects are known to occur due to various types of chromosomal abnormalities or mutations in the human genome. Chromosomal analysis is done to look for number of chromosomes and structural abnormalities like deletion, duplication, translocation etc. It is indicated in all patients with multiple malformation syndromes. The molecular genetic assays help to diagnose the single gene disorders which lead to craniofacial defects. These assays have been developed based on the type of mutation commonly seen in each disease. In addition the patient needs to be counseled regarding the need and limitations of the test before conducting a molecular genetic test. A number of different techniques are used to identify mutations in genetic diseases. Assays based on PCR and restriction enzymes have been used to detect mutations in past. However improved methods based on sequencing of genes are being used in diseases caused due to point mutations. Some diseases occur due to large deletions or duplications, which cannot be detected by sequencing. Recently newer methods are being increasingly used in diagnostic laboratories for detection of large deletions and duplications. Multiplex Ligation dependent Probe Amplification (MLPA), Real time PCR (RT-PCR), array comparative genomic hybridization etc are some of these techniques. Advances in the field molecular genetics have now made it possible to sequence whole exome or whole genome of an individual. Whole exome sequencing is already being used in clinical practice for diagnosis of rare disorders. Further it is now possible to sequence multiple genes responsible for a phenotype at much lower cost using next generation sequencing technologies. These techniques are going to revolutionize the field of diagnostics using molecular methods in future. Various techniques used in genetic diagnosis of craniofacial defects will be discussed in the presentation. 38 10th World Cleft Lip, Palate & Craniofacial Congress

Medical Genetics in Craniofacial Clinical Practice: Indian Scenario

Shankar VH Consultant Geneticist, Additional Professor of Pediatrics, Department of Pediatrics, SAT Hospital, Government Medical College, Thiruvananthapuram, Kerala, India

Birth defects are one of the important indications for referral to Medical Genetics. Major and minor craniofacial anomalies and variants of facial features are important components of many dysmorphic syndromes. Clinical examination and delineation of phenotype is the first and very important step in the evaluation of a case with craniofacial anomalies. Clinical geneticists and some pediatricians with good clinical expertise in dysmorphology diagnosis have been diagnosing as well as publishing cases with malformation syndromes. With availability of genetic diagnosis in India, more and more cases are diagnosed by DNA based techniques. Cases of varying etiologies confirmed by various genetic techniques will be presented to stress the appropriate use of diagnostic tests. Diagnostic utility of genomic techniques like microarray and next generation sequencing will be discussed using illustrative case scenarios. At the same time simple clinical tools like pedigree drawing, examination of parents and siblings will be documented. It is important to use Human Phenotype Ontology for documentation of phenotypes and syndrome search. The issues in genetic counseling in Indian scenario need to be dealt with by the clinician and includes non-directive counseling. The craniofacial surgeons need to be aware of need and the purpose prenatal diagnosis as well as complexities of counseling in a case with prenatally diagnosed facial clefts in a low risk family. The large Indian population and high levels of consanguinity provides an opportunity to document rare / novel syndromes and every clinician dealing with birth defects needs to look at cases with genetic perspective; so that the families needing genetic counseling and genetic evaluation can be helped. Collaboration with medical geneticists in India can lead fruitful good quality of research in documentation of syndromes, natural history and identification of novel genes in the etiologies of craniofacial syndromes.

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Cleft lip & palate treatment team in Kyushu University Hospital, Fukuoka, Japan -Prenatal counseling and visiting on birth day

Takeshi MITSUYASU, Kaori MATSUMURA, Ami MAENO, Seiji Nakamura Depatment of Oral & Maxillofacial Surgery, Kyushu University Hospital, Fukuoka, Japan

The team approach for managing and correcting cleft lip and palate is important for patients and families. The American Cleft Palate Association “Standards for Approval of Cleft Palate and Craniofacial Teams Commission on Approval of Teams” suggests that teams are comprised of experienced and qualified professionals from medical, surgical, dental, and allied health disciplines working in an interdisciplinary and coordinated system. Our team includes oral surgery, pediatric dentistry, pediatrics, otolaryngology, orthodontics, speech-language pathologist and nurses. Especially after getting the information of baby’s cleft lip and palate (prenatally or after birth), the parents are usually shocked. The parents’ situation is psychosocial emergency, that is disappointment, weakness and hopelessness. Often mothers feel guilty about the baby’s cleft lip and palate. During such a period, parents need to get the accurate information about malformation and its treatment and prognosis. Of course, for the duration parents are in deep anxiety and sorrow. In order diagnosis,to reduce theirthe treatment anxiety, we plan give and the the prenatal prognosis. counselling and visit hospitals on the birthday. At the first communication, we give the information about the

We also support the management of TSUBASA, which is a cleft lip and palate patient’s families’ community. (TSUBASA means wings in Japanese.) We ahold team TSUBASA meeting. meetings twice a year, a lecture meeting and Christmas meeting. Parents can exchange their information each other. And every month we hold

40 10th World Cleft Lip, Palate & Craniofacial Congress

Cleft palate repair – Not just closing the gap

Suni Kumar Rout Senior Consultant, Plastic Surgery, Kalinga Hospital, Bhubaneshwar, Odisha, India

Ours is a high volume cleft centre, busy in cleft service over the period of more than a decade. It is located in the capital city of our state. In the earlier days cleft palate in our center was primarily repaired by either two flap or horse shoe flap technique. Recently soft palates with narrow cleft are being repaired by Furlows double opposing Z - plasty. Over the period of initial 5 years the muscle dissection was limited to division of tensor aponeurosis from the margin of the hard palate and freeing levator veli palatini muscle from the cleft margin. The free margins of levator muscles of both sides were not sutured together, which made a routine thereafter. Since the year 2010 we are dissecting the levator muscle radically, as described by Dr. Brian Sommerlad. The muscles are dissected throughout their entire length and sutured with each other at the cleft margin. To analyze the result of our cleft palate repair we divided the patients into two groups time period wise. Patients operated from 01/01/2005 to 31/12/2009 constitute one group (A) and patients operated from 01/01/2010 to 31/12/2014 constitute the other group (B). Fistula rate Theand resultsVPI score were of summarizedthe two groups below. were compared. Tympanometric analysis of the middle ear function was also performed in a series of cleft palate patients.

Fistula rate – In group ‘A’ total number of patients operated for cleft palate was 756. Out of them 195 patients turned up for follow up. 41 of them observed to have anterior palatal fistula. 2 had complete dehiscence after the primary repair. Hence fistula rate is 21% amongst the follow up patients. It comes down to 5.4% if we consider the total number of patients operated. In group ‘B’ we operated on 480 patients having primary cleft palate. Out of them 138 patients turned up for follow up. 18 of them had fistula in palate which constitute 13% of the follow up patients and 3.75% of total patients. VPI rate – 116 patients of group A operated primarily for cleft palate could be subjected for their speech assessment by our speech pathologist. 29 41% 10th World Cleft Lip, Palate & Craniofacial Congress of them found to have incompetent valving mechanism. In group B, 78 children were assessed and 17% of them had VPI. The speeches were assessed using Pittsburgh weighted speech scale. Tympanogram – We compared the tympanograms of patients operated similarbefore thein both age ofthe 2 groups.years for cleft palate and those underwent repair late (after 6 years of age). The percentage of patients having normal tympanogram was

To conclude we are having a satisfactory outcome in terms of speech, after we made radical dissection of levator veli palatini muscle, a routine during primary repair of cleft palate. The change in surgical technique also did not increase the rate of fistula. Hence repair of cleft palate is not just closing the gap, but achieving good function.

42 10th World Cleft Lip, Palate & Craniofacial Congress

Cleft Lip And Palate Care, Our Clinical Perspectives

Aysegul M. TUZUNER ONCUL Associate Professor, Dept. of Oral and Maxillofacial Surgery, Faculty of Dentistry, Ankara University, Ankara, Turkey

Absract

Any cut in a diamond stays forever because diamonds are forever. Cleft- lip and palate babies are like diamonds. Any reckless surgical touch in a cleft baby stays for a lifetime. When it comes to treatment of a cleft lip and palate baby, many techniques and timing protocols have been described. The goals of primary closure of cleft lip and palate include not only re-establishing normal insertions for all of the nasolabial muscles but also restoring the normal position of all the other soft tissues, including the mucocutaneous elements. The functional closure of the cleft lip, palate and the alveolus is essential. In this lecture, treatments of cleft lip and palate patients with Delaire’s functional cheilorhinoplasty, palatoplasty and Precious’s alveolar bone grafting technique in Ankara University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery will be presented. Moreover late stage secondary cleft lip and palate treatmentIntroduction outcomes due to late referrals will be described.

Cleft lip with/without cleft palate is one of the most frequently occurring craniofacial birth defect. The condition is as old as mankind itself, besides it has been described in different ways by the people of every area. Any cut in a diamond stays forever because diamonds are forever. Cleft-lip and palate babies are like diamonds. Any reckless surgical touch in a cleft baby stays for a lifetime. When it comes to treatment of a cleft lip and palate baby, many techniques and timing protocols have been described. Reports from registry data including European Surveillance of Congenital Anomalies (EUROCAT), the Latin American Collaborative Study of Congenital Malformations (ECLAMC) and international Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) internationally suggest wide variations 43in 10th World Cleft Lip, Palate & Craniofacial Congress the birth prevalence of cleft lip and/or cleft palate (CL/P) across setting (1). Reports of isolated cleft lip with or without cleft palate were 3.4 to 22.9 per 10000 live births while reports of isolated cleft palate only range from 1.3 to 25.3 per 10000 live births (2). A systematic review of CL/P prevalence up to 2012 found that approximately 1 in 700 children world wide born with orofacial clefts (1,3). In a systematic review and meta-analysis of the birth prevalence of orofacial clefts in low and middle-income countries, showed that approximately 1 in every 730 children is born with CL/P (4). In Turkish population, in a study which 21,904 live births were investigated and the incidences of cleft palate alone, cleft lip alone and cleft lip together with cleft palate were found to be 0.77, 0.77, 0.95 in 1,000 births, respectively (5). When the subject is cleft lip and palate many techniques and timing protocols have been described. The goals of primary closure of cleft lip and palate include not only re-establishing normal insertions for all of the nasolabial muscles but also restoring the normal position of all the other soft tissues, including the mucocutaneous elements. The functional closure of the cleft lip, palate and the alveolus is essential. The first printed Turkish Surgical Manuscript by Şerefeddin Sabuncuoğlu in the fifteenth century has several miniature illustrations of different reconstructive surgical procedures. Figure shows a miniature entitled repair of a lip fissure (Picture 1). The founder of our Dentistry Faculty and Oral Maxillofacial surgery department, C. Borcbakan published an analyses of 1000 cases of CLP in Turkey in 1969’s Cleft Palate Journal (6). Maxillofacial surgeons have been involved in the cleft care protocols since Borcbakan’s practice in this field.CLEFT In CARE this lecture PROTHOCOL our treatment protocol in CL/P patients will be presented.

My first presenting with a newborn cleft baby is right after, we had the call from the newborn pediatric unit (Picture 2,6). Feeding instruction, evaluation by the geneticist and hearing tests are done in that unit. After the baby has discharged from the hospital a feeding appliance is done by the Department of Orthodontics in Ankara University, Dentistry Faculty. 7-10 days after the feeding appliance is chanced to a presurgical infant orthodontic appliance for nasoalveolar molding (Picture 3.s). The primary lip repair is done when the baby is 4-6 months old with Delaire’s functional cheilorhinoplasty technique (7,8). Picture 4 and 8 shows the postoperative 1 week after primary surgeries. Picture44 5 and 9 shows the facial appearance of the same patients at the age 10th World Cleft Lip, Palate & Craniofacial Congress

of 3 and 5 respectivelly. In complete CLP patients, depending on the patients socioeconomical situation and the wideness of the cleft two stage or single stage palatoplasty is done. The soft palate is reconstructed first at 6-9 months. The hard palate is closed later in 12-18 months of age. Preoperative, immediate postoperative and 8 months postoperative pictures of case with isolated cleft palate deformity is pictures shown in pictures 10,11,12 respectively. At the age of 2, the patients are referred to the speech pathologist and speech therapy is started if necessary. At 6 years mixed dentition secondary alveolar bone grafting is done with the technique described by David S. Precious (9). Unfortunately sometimes I do not have the referrals at the appropriate time in cases which primary operations were done in other departments or institutions. As a result, late secondary or tertiary bone grafting procedures are also done by using the same technique. Also correction of late adult palate fistulas or failed alveolar bone grafting operations which were done by other departments are also done mostly in our practice.16 years old female patients with bilateral alveolar clefts which was operated two times previously in an other institution is shown in picture 13 a,b and c. Postoperative pictures of the same patient are given in picture 14. Eight weeks after reconstruction of the alveolar cleft defect, orthodontic care with maxillary expansion or facemask protraction devices is done in the department of orthodontics. When the growth is completed, surgical advancement of maxilla is done if necessary (picture 15 a,b). Six months after maxillary advancement dental implant rehabilitation is done (picture 16). The timing and sequencing of the procedures outlined are determined and evaluationsCONCLUSION are scheduled regularly during the entire growth period.

Although there are several controversies about operative techniques and timing of the treatment of a patient with CL/P. Functional anatomical closure of the neuromuscular structure is essential to obtain which is reconstruction of ‘normal’, orofacial muscular balance, lip competency, good nasal breathing, anatomical palatal closure with normal development of speech, hearing and feedingREFERENCES without any fistula. 01. Mossey PA. Little J. Epidemiology of oral clefts: an international perspective. In Wyszynski DF. Ed. Cleft Lip and Palate: From Origin to Treatment. New York: Oxford University Press; 2002:127-158

45 10th World Cleft Lip, Palate & Craniofacial Congress 02. Mossey PA. Little J. Munger RG, Dixon MJ, Shaw WC. Cleft lip and palate. Laneet. 2009;374:1773-1785 03. Mossey PA. Modell B. Epidemiology of oral clefts 2012: an international perspective. Front Oral Biol. 2012;16:1-18 04. Kadir, A, Mossey, PA, Blencowe, H, Moorthie, S, Lawn, JE, Mastroiacovo, P & Modell, B 2016, 'Systematic Review and Meta-analysis of the Birth Prevalence of Orofacial Clefts in Low- and Middle-Income Countries' Cleft Palate-Craniofacial Journal. DOI: 10.1597/15- 221 05. Tunçbilek E, et al, Results of the Turkish Congenital Malformation Survey. Turk J Pediatrics, 1999;41:287-297. 06. Borcbakan C. An analysis of 1000 cases of cleft lip and palate in Turkey. Cleft Palate J. 1969, jul: 6:210-212. 07. Precious DS. Primary bilateral cleft lip/nose repair using the "Delaire" technique. Atlas Oral Maxillofac Surg Clin North Am. 2009 Sep;17(2):137-46. doi: 10.1016/j.cxom.2009.05.006. 08. Precious DS. Primary unilateral cleft lip/nose repair using the "Delaire" technique. Atlas Oral Maxillofac Surg Clin North Am. 2009 Sep;17(2):125-35. doi: 10.1016/j. cxom.2009.05.003. 09. Precious DS. A new reliable method for alveolar bone grafting at about 6 years of age. J Oral Maxillofac Surg. 2009 Oct;67(10):2045-53. doi:10.1016/j.joms.2009.04.102.

46 10th World Cleft Lip, Palate & Craniofacial Congress

Deleare Approach For Cleft Lip And Palate Our Experience

Andrés Duque Craniomaxilofacial Surgery Dept. Roosevelt Institute, Maxillofacial Surgery Dept. Meissen Hospital E.S.E, Cleft Lip and Palate Program Colombian Air Force Reserve, Bogota-Colombia

ABSTRACT

Cleft lip and palate (CLP) is one of the most common congenital facial deformities, in Colombia the overall incidence is 1.28 per 1000 new born7. Historically many techniques have been used but the ideal continues to be controversial, in general primary surgery must be seen as the most important opportunity to the patient for a better or worse quality of life, the knowledge of all growth process, developmental physiology and the expertise of the surgeon can make the difference. Professor Jean Delaire establish several positions to understand the roll of growth and function in the repair of CLP, muscles of the three facial and pharyngeal sphincters need to be reorientaded to their normal position, growth centers need to be respect and all involved structures need to be treated not as separate elements. Deleare protocol at Craniomaxillofacial surgery department at Roosevelt Institute and Cleft lip and palate programs in Meissen Hospital and Colombian Air Force Professional Officers of Reserve (non profit) , established this philosophy since 2006 with excellent results. We present our protocol, experience and statistics using Delaire’s approach for cleft lip and palate patient´s.

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Orofacial Clefting and Dental Anomalies

Nik Kantaputra Center of Excellence in Medical Genetics Research, Department of Orthodontics and Pediatric Dentistry, Faculty of Dentistry, Chiang Mai University, Thailand

Dental anomalies have been known to be associated with many genetic disorders. Hypodontia or missing of teeth can be isolated disorders or associated with many genetic syndromes. Isolated hypodontia has been reported to be caused by mutations in MSX1, PAX9, AXIN2, EDA, EDAR, EDARADD, WNT10A, and GREM2 genes. As being a part of genetic syndromes, hypodontia is associated with orofacial clefts or nail anomalies in MSX1-associated syndromes. MSX1 mutations may cause isolated hypodontia or isolated orofacial clefts. Transcription factor TBX22 is known to have a crucial role in craniofacial development. Mutations in TBX22 causes X-linked semidominant cleft palate and syndrome. Affected individuals may have cleft palate with or without tongue tie. Many of the carriers of the mutated gene have been reported to have only tongue tie. Searching for carriers who have tongue tie may be useful for those who plan to have children, as they have the chance of having children with cleft palate. In addition, the gene for isolated tongue tie.tie has not been reported before in the literature. We have identified for the first time a novel mutation in TBX22 in a child who has nonsyndromic tongue

P63 is a transcription factor which has important roles in the differentiation and development of epithelium cells. Mutations of P63 have been known to be the causes of at least 5 different ectodermal dysplasia syndromes including -Ectodermal dysplasia and Cleft lip/palate Syndrome (EEC), Ankyloblepharon-Ectodermal defects-Cleft lip/palate Syndrome (AEC), Rapp-Hodgkin Syndrome (RHS), Limb-Mammary Syndrome (LMS), and Acro-Dermato-Ungual-Lacrimal-Tooth Syndrome (ADULT). In addition to ectodermal dysplasia syndromes, isolated or non-syndromic split hand-split foot malformation (SHSF) and non-syndromic cleft lip have been described to be caused by mutations in P63. We were the first group in the world who found that Rapp-Hodgkin syndrome was caused by P63 mutation. 48 10th World Cleft Lip, Palate & Craniofacial Congress

Interestingly missing of teeth has recently been described to be associated with colorectal polyps and cancer in a family with AXIN2 mutation. AXIN2 is a transcription factor that has a very important role in Wnt-signaling. As it is an autosomal dominant disorder, missing of teeth then can be used as an “warning feature” for the families at risk of colorectal polyps or cancer. It is believed that knowing that they have missing teeth in the families with colorectal cancer, it might save their lives if they get medical attention early. In conclusion, the lecture will cover the molecular pathogenesis of craniofacial anomalies with emphases on dental anomalies and orofacial clefts.

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Genetic studies in orofacial clefts

S J Patil Consultant Clinical Genetics, Narayana Health City, Bangalore, India

Orofacial clefts (OFC) could be isolated or associated with additional malformations (syndromic). Underlying etiology include chromosomal, single gene/multiple genes, gene-environmental interactions, and teratogens – similar to any other human birth defects. Genetic studies vary depending upon whether the OFC are isolated or syndromic. The phenotype of OFCs can range from minor forms of OFC (eg discontinuous superior orbicularis oris, absent lateral incisors etc), cleft lip with or without cleft palate (CLP), and cleft palate (CPO). CLP has been considered etiologically (genetic basis) different from CPO, although there are some exceptions to this rule. In non-syndromic isolated OFC, the gene identified could be of high penetrance (mutated gene by itself is sufficient to cause phenotype, although with variable expression) or may require additional genes or environmental factors to cause phenotype. Some of the genes contributing to isolated OFC are also known to cause syndromic forms of OFCs. The genes causing non-syndromic OFC include IRF6, MSX1, FOXE1, GLI2, MSX2, SKI, BMP4, TBX22 and WNT3 etc. The genes involved in isolated OFC have shown differences based on the ethnic origin. Most of the genetic testing in the isolated OFCs is done on research basis. In OFC of syndromic origin underlying cause could be single gene mutations (autosomal recessive, autosomal dominant, X-linked), or chromosomal aberration (aneuploidies, gross structural chromosomal rearrangements, copy number variants) - genetic component playing strong role in causing the clinical phenotype. Some of the syndromes of OFCs include chromosomal – 22q11.2 microdeletion, trisomy 13; single gene mutations of Mendelian inheritance - Van der Woude syndrome, EEC syndrome, Kallmann syndrome etc

50 10th World Cleft Lip, Palate & Craniofacial Congress

Approach to a case with orofacial cleft: Clinical genetics perspective

Girisha KM Professor and Head, Department of Medical Genetics, Kasturba Medical College, Manipal University, Manipal, India

Abstract

Orofacial clefts represent anomalies in the development of face and oral cavity manifesting as cleft lip, cleft palate or other rare clefts of face. They can be due to intrinsic defect (malformation) or external pressure (disruption) and can have diverse etiology that includes genetic and non-genetic factors. Chromosomal, Mendelian, multifactorial and environmental etiologies are known to underlie various orofacial clefts. They may be isolated or a part of a syndrome. I will discuss how to approach a case with orofacial cleft taking in to account the development of orofacial structures, modes of inheritance, presence of syndromic features in my presentation with illustrative cases.

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Early response of human mesenchymal stem cells to mechanical force and supply of oxygen – research and clinical perspectives

Mirek Tolar1,2,3, Jess Punch2, Nadim Guirguis2, Veronica Lee1, Ethan Tencati1, Marie Tolarova1,2, Mohamed Fallah2, Robert Boyd2 1Pacific Regenerative Dentistry Laboratory,2 Department of Orthodontics, 3Department of Biomedical Sciences, Dugoni School of Dentistry, University of the Pacific, San Francisco, CA, USA

INTRODUCTION

Quality of microenvironment of mesenchymal stem cells depends, among other factors, on spectrum of dissolved signaling molecules and nutrients, bigquality roles. of substrate for anchorage of cells, oxygen supply and mechanical forces applied to the cells. Clearly, both biochemical and biomechanical factors play MATERIALS AND METHODS

01. In a model experiment in vitro, we applied tension or compression load to an aggregate of human periodontal mesenchymal stem cells. The cells were maintained in atmospheric (20%) oxygen or in a tissue-specific (5%) oxygen concentration.

02. periodontiumDuring orthodontic on the tooth opposite movement, side of thea tooth. force Damage applied to to the a tooth periodontal causes extension of periodontium on one side of a tooth and compression of

cells triggers an innate immune response. We followed time changes in RESULTSconcentration of early-response cytokines in gingival crevicular fluid.

were found. 1. Substantial differences in expression of genes involved in osteogenesis

2. Transient increases in concentrations of interleukin-1beta and interleukin- 1receptor antagonist were documented in real time on tension and 52 compression sides of the moved teeth. 10th World Cleft Lip, Palate & Craniofacial Congress DISCUSSION

These findings may be applied to the art and science of human bone engineering using autologous mesenchymal stem cells. If collected mesenchymal stem cells are expanded in vitro, it is very important to grow them in a tissue-specific low-oxygen atmosphere. The engineered implant should be vascularized within several hours in order to prevent cell damage that would trigger an immune reaction. From this perspective, distraction osteogenesis seems to be the most physiological technique of bone tissue engineering.

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Three-dimensional tissue-engineered cartilage using biodegradable polymers

Kazuto Hoshi The University of Tokyo , Graduate School of Medicine, Tokyo, Japan

Since cartilage has a poor regenerative capacity by itself, its regeneration using a tissue engineering technique may greatly benefit the treatment of various cartilage disorders that cannot be treated by conventional methods. Although autologous chondrocyte transplantation has been already available for clinical use, it is applied only to a filler of focal articular cartilage defect or an injectable material for nasal cosmetic augmentation. In order to broaden its clinical indication to nasal deformity of cleft lip and palate patients, we should make a three-dimensional tissue-engineered cartilage with a greater firmness. Therefore, we developed 1) a proliferation medium for chondrocytes to realize a more than 1000-fold increase in number without using fetal bovine serum, and 2) a scaffold system that effectively preserves chondrocytes in the engineered tissue and provides the adequate 3D shape to the tissue. For the proliferation medium, we examined the optimal combination from 12 putative soluble chondrocyte regulators including FGF-2, IGF-I, insulin, BMP-2, PTH and others. Using the statistical method that is termed “analysis of variance by fractional factorial design”, the effects of the individual factors and the synergy of the combinations were evaluated. As a result, the combination of FGF-2 and insulin with 5% human serum showed a 10-12-fold increase in number within one week and provided an approximate 1000-fold increase around 3 weeks. As the scaffold, we prepared porous scaffolds with various structures and compositions by the sugar-leaching method or fused deposition molding, and transplanted them with chondrocytes in the subcutaneous space of nude mice. The porous PLLA with the combination of the atelocollagen hydrogel revealed both the 3D environment suitable for chondrocytes and sufficient hardness of the whole tissues to realize fair cartilage regeneration. By these techniques, we could make the three-dimensional tissue-engineered cartilage. The safety and the effectiveness of the engineered tissues were confirmed in the in vitro experiments or by the autologous transplantation of the tissue-engineered cartilage54 in beagles. After the acquisition of institutional and governmental 10th World Cleft Lip, Palate & Craniofacial Congress

permission, we began the clinical trial of that tissue-engineered cartilage for treatment of a nasal deformity in patients with a cleft lip and palate. Post- operatively, safety has been confirmed based on the incidence of adverse events including pain, infection or graft failure requiring the removal of tissue- engineered cartilage after transplantation. Effectiveness was also assessed based on exploratory evaluation indicators, such as satisfaction level of patients, activity of daily living, improvement of facial features, less-invasiveness at donor sites and formation of regenerated cartilage. In the presentation, we will show the research and development of the three-dimensional tissue-engineered cartilage and its clinical outcome.

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Clinical outcome and bone regenerative effects from using calcium phosphate-based implants in cranial and facial repair

Thomas Engstrand Plastic Surgeon, Department of Molecular Medicine and Surgery, Plastic Surgery Section, Karolinska University Hospital and Karolinska Institute, Stockholm, Sweden

Background

Autologous bone and alloplastic materials used for cranial and facial repair are associated with resorption, infection and extrusion. Materials with regenerative features may improve outcome, A bone regenerative calcium-phosphate based implant was used in the present chart review. The primary objective was to determine complication rate, as defined as number of explantations due to infection and/or extrusion (N=150). The secondary objectiveMethod was to uncover evidence of bone formation induced by the implant.

One hundred and fifty (N=150) patients with various cranial and defects were treated. The follow-up time was 1-72 months (mean 23 months). In two patients biopsies were taken 9 and 50 months after surgery, respectively, for geneResult expression analyses and histological examinations.

The explantation rate was 5,3 % in the cranial defect cohort where approximately 50 % of treated patients had a history of previously failed autologous bone flaps or conventional implants (PMMA, PEEK, titanium, polyethylene). Early postoperative wound dehiscence and infection occurred in 8 cases. Five patients had local revision due to wound dehiscence that eventually healed without implant removal. A majority of failures occurred within 3 months after surgery whereas two patients had late onset titanium exposure adjacent to the ceramic implants that necessitated partial revision. 56 In one patient, 9 months postoperatively, gene expression analyses revealed 10th World Cleft Lip, Palate & Craniofacial Congress

expression of osteocalcin, type I collagen, osteopontin, calcitonin receptor, and cathepsin K within the reconstructed area. Interestingly, an up-regulation of type I collagen expression was found in the soft tissue covering the implant. In another patient, histological examination revealed vascularized compact bone withinConclusion the reconstructed area 50 months postoperatively.

Weak quality of soft tissue covering the implant, caused by irradiation or scars, exposed titanium, and smoking are identified as major risk factors. We believe that the process of new bone formation as well as new collagen deposition in neighboring soft tissue induced in situ by the ceramic implant may contribute to reducing the number of postoperative complications, especially late-onset weakening of covering soft tissue leading to implant exposure. The same material composition is currently also utilized for advanced facial reconstruction.Disclosure of Interest The outcome in this cohort will be presented in addition.

T Engstrand is inventor of the technique and has direct ownership in OssDsign, the manufacturer of the implant that is the subject of this study. Part of the present study was orally presented at the ISCFS-meeting in Tokyo Sept 2015. The section describing the bone regenerative effects was published as case report in Plastic and Reconstructive Surgery Global Open, August 2015. The cranial implant, that is the subject of this study, is pending FDA approval.

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The use of buccal fat pad free graft in Closure of soft tissue defects and dehiscence in the hard palate. A New Technique

Fares Kablan Department of Oral and maxillofacial surgery, The Baruch Pade Medical Center, Tiberias, Israel. Poria, M.P. The Lower Galilee, Tiberias, Israel. E-mail: [email protected]

ABSTRACT Introduction

The integrity of the palatal mucosa can be lost due to congenital, pathological and iatrogenic conditions. Various surgical techniques have been suggested for the closure of palatal defects. The aim of the current study is to present the free buccal fat pad graft (FBFG) as a novel technique to repair of soft tissuePatients defects and Methods at the palate.

During a two-year period, the free fat tissue graft harvested from the BFP (FBFG) and used to reconstruct five soft tissue defects of the palate in five patients (2 women, 3 men; mean age; 34 years; range, 22–58 years) . In two patients the palatal defect size was 2 to 3 cm, and resulted from resection of pleomorphic adenoma. In two other patients the defect was due to odontogenic lesion, and in the last patient the etiology was an iatrogenic dehiscence during maxillary segmentation surgery. Patients were examined every two weeks in theResults first three months, and thereafter every 3 months.

Five patients were treated with FBFG to reconstruct palatal defects and were followed-up for 6 to 24 months. The healing process of the BFP and the sites.recipient sites were uneventful, with minimal morbidity. At three months after the surgery there was complete epithelialization of the graft at the recipient

58 10th World Cleft Lip, Palate & Craniofacial Congress Conclusions

Harvesting of FBFG is a simple procedure with minor complications, manipulation and handling the graft are easy. The use of the FBFG in reconstruction of small and medium palatal defects is encouraging with excellentKeywords clinical outcomes. soft tissue grafts buccal fat pad, free fat tissue, palatal defects, Oral lesions reconstruction, INTRODUCTION

Pathologic conditions from an odontogenic origion (abscesses, cysts and tumors), residual defects following clefts surgeries, surgical defects after excisions of various cancerous lesions, trauma, and even iatrogenic injuries during segmentation surgeries of the maxilla, can lead to loss of the palatal mucosa integrity. Palatal fistulas and dehiscence may become a major burden on patient quality of life and a surgical challenge for the treating doctor. Various surgical techniques have been suggested for the closure of palatal defects which includes; secondary healing, palatal flaps, tongue flaps, turnover flaps of adjacent muco- periosteum, and pedicled flap from the buccal fat pad(1-9) . The use of the buccal fat pad as a pedicled flap(PBFPF) as a graft for intraoral defects is a common procedure since its first publication by Egyedi in 1977(10), and the outcomes from the use of this flap for closure of intraoral defects have been encouraging. The success of the PBFPF has been attributed to its rich vascular supply, less donor site morbidity, almost constant weight for all individuals, ease to harvest, and the fast epithelialization during 3 to 6 weeks. (11-14) Low complications rate also have been reported regarding the PBFPF and includes; distant dehiscence and necrosis, limitation in mouth opening, mild obliteration of the vestibule, limitation of passing it through dentate areas that can compromise the blood supply, and the limitation of its applications at large defects and in lesions at the anterior regions at the oral cavity.(11). Following years of handling the fat tissue by the author in various asurgeries free graft. it has been noted that the fat tissue from the buccal fat pad that is free from its blood supply, can survive and heal very good, so it can be used as

The concept of transplanting an autogenous fat as free graft is well- documented for cosmetic surgery. It has been used more than 100 years, and59 10th World Cleft Lip, Palate & Craniofacial Congress its clinical behavior, characteristics, and healing are all well known in this area of medicine [15]. The main mechanism of healing of free fat grafts (FFGs) is by fibrosis [16] In 1983, Neder reported the use of BFP as a free graft for oral lesion reconstruction in two patients [17]. Kablan and Laster in 2012 first reported the use of free buccal fat graft (FBFG) with bone augmentation. In their report, they discussed the advantages of the FBFG and the clinical and histologic healing stage, the main healing nature of the FBFGs was fibrosis of the graft [18]. Additional use of the FBFPG was also reported by the author with excellent outcomes (19). The aim of the present report was to introduce additional use of the FBFPG in the treatment small to medium size defects of the palatalPATIENTS mucosa. AND METHODS Methods

During a two-year period, the free fat tissue graft harvested from the BFP (FBFG) and used to reconstruct five soft tissue defects of the palate in five patients (2 women, 3 men; mean age; 34 years; range, 22–58 years) . In two patients(case 1 and5) the palatal defect size was 2 to 3 cm, and resulted from resection of pleomorphic adenoma. In one another patient(case 4), the defect was a fistula from radicular odontogenic cyst that involved tooth 24. In additional patient (case 2) the palatal dehiscence was secondary to incomplete healing from previous resection of ameloblastic fibroma at the anterior maxilla, and in the last patient(case 3) the etiology was an iatrogenic dehiscence during maxillary segmentation surgery (Tab 1). dependsPatients on the wereetiology examined of the original every lesion. two weeks in the first three months, and thereafter every 3 months. The duration for the future follow-up period TECHNIQUE The donor site

The FBFG was harvested from the BFP via the standard approach[Figure 1]. This approach allows access to the BFP through a small horizontal incision in the free mucosa above the second and the third maxillary molars [Figure 1a]. Through blunt dissection, the fat tissue is accessed [Figure 1b-c] and easily mobilized to the oral cavity by progressive blunt dissection [Figure 1d]. The

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desired FBFG is harvested [Figure 1e-f], the BFP is pushed back in its place, and theCASE incision PRESENTATIONS is sutured [Figure 1g]. Case 1 (Fig 2)

A 29 years old female patient was admitted to our department, complaining about a painless swelling on the hard palate. History revealed that the swelling was detected 3 years ago. The lesion was growing slowly.

Figure a to n

Intraoral examination revealed an oval-shape circumscribed lesion, adherent to the underlying structures, covered with slightly erythematous palatal mucosa. The size of the lesion was 2cm in diameter, and extended from the mid-palatal area to the left alveolar ridge with smooth and intact overlying mucosa. The panorex radiograph was uneventful. Clinical diagnosis of pleomorphic adenoma was suspected and confirmed with incisional biopsy. Figures 2a and 2b demonstrate the lesion after biopsy. Excision of the tumor was done under general anesthesia. A wide Local resection including the periosteum with 0.5cm borders was performed with blade (Fig 2c-2e), hemostasis was done by electrosurgery. The bone was not involved. To cover the surgical defect, FBFG was harvested from the left BFP, and its dimensions were 4cm in length and width of 2cm. (Fig 2f). After undermining of the recipient site soft tissue, the FBFG was secured with horizontal and interrupted sutures to the surgical defect.(Fig 2g-h). Prefabricated acrylic stent was used on the upper jaw to support the graft in position for the first 3 days. 61 10th World Cleft Lip, Palate & Craniofacial Congress

Follow-up examinations at 3 days (fig 2i), at 2 weeks (Fig 2j-k), after the surgery showed uneventful recovery. At one month after the surgery the FBFG showed complete epithelialization, and a residual oronasal fistula between the hard and soft tissue palate was found(Fig 2L). The patient was advised to use the acrylic stent to prevent regurgitation of fluid/food into the nose and to prevent speech problems. Two months after the surgery the fistula was healed spontaneously (Fig 2m). The 12 months follow-up recall revealed a good healing of the palate without recurrent of the tumor. The grafted palatal site was as the same color and architecture of the contralateral site.(Fig 2n). The patient will beCase followed-up 2 (Fig 3) for 5 years. side ofA this 35-year tooth. old female patient was referred due to swelling at the hard palate, mobility of the left upper lateral incisor with food retention at the palatal

On examination; the buccal side architecture was healthy (Fig 3a). A painless solid swelling at the palatal region that extended from tooth 11 -13(Fig 3b), this area was smooth except contraction of the soft tissue palatal to tooth 12(fissure like). The computed tomography demonstrated a radiolucent lesion (Fig 3c). Under local anesthesia, the patient underwent resection of the lesion with peripheral osteoctomy (Fig 3d). At this stage the root of tooth 12 was involved, but after meticulous debridement, the tooth was preserved. The contraction site underwent refreshment and primary closure (Fig 3e). The

62 Figure a to n 10th World Cleft Lip, Palate & Craniofacial Congress

followhistological u examination revealed ameloblastic fibroma. Due to the complete resection of the lesion and the peripheral ostectomy the decision was to p the case without additional surgical intervention. Two months after the missed last recall the patient came again to the department complaining about food impaction and high mobility of tooth 12 that necessitated splinting to the adjacent teeth by her dentist. On examination; at the palatal side there was dehiscence of the soft tissue with entire exposure of the root at tooth 12, and accumulation of calculus (Fig 3f). The buccal side was intact (Fig 3g). At second surgery the root of 12 was resected leaving its crown temporary for esthetic reasons (Fig 3h). The soft tissue underwent de-epithelialization and refreshments (Fig 3i). The right BFP was addressed for FBFG (Fig 3j), and the desired FBFG volume was obtained (Fig 3k). The FBFG was easily spread over the recipient site, and meticulous suturing was performed (Fig 3m). At two weeks follow-up the healing of the FBFG at the recipient site was Uneventful (Fig 3n). One year after the FBFG there was satisfied healing of the recipient site. Long term follow upRESULTS is necessary in this lesion.

FBFGs were used in 5 hard palate defects, in 5 patients. The healing process was uneventful. The BFPs healed very well without complications and esthetic disturbances. In 2 cases the FBFG was used to reconstruct a soft tissue defect following resection of pleomorphic adenoma; the healing process went very well in one patient, in the additional patient(case1) a residual oronasal communication was observed after two weeks and was healed spontaneously after three months. In two cases the FBFG was used to cover a residual defect with etiology of odontogenic lesions, with good outcomes. The last case was

treated with FBFG due to iatrogenic injury to the soft tissue of the hard palate during two piece maxillary osteotomy(fig 4a), the dehiscence healed very well by the use of this graft, and full integrity of the palatal soft tissue was observed three months after the injury( fig 4b). 63 10th World Cleft Lip, Palate & Craniofacial Congress

In all the patients, at three months after the treatment with FBFG, the recipient sites healed very well, and were covered with contact epithelium. The patientsDISCUSSION have been followed up 6 to 24 months.

The BFP is located in the masticatory space and consists of central body (corpus) with four extensions: Buccal, pterygoid, superficial, and deep temporal. The total estimated volume is 10 ml and its lower thickness is 6mm. The body and the buccal extension make up more than 50% of the BFP. They are accessible from the oral cavity and are the portions of the BFP that may be used as donor sites for fat tissue grafts. [11-14] The BFP has been used for several reconstruction applications in oral and maxillofacial area more than three decades. The predictable outcomes, the ease to harvest the graft, and the low complication rates make the PBFPG an attractive flap to close small to medium sized lesion in the posterior regions of the oral cavity (11). The nature of healing of the pedicled BFP has been widely reported, and the main mechanism of the healing process is by fibrosis and fast epithelialization of the graft. [11,12,14] Autologous FFGs are widely used in cosmetic surgery and it is well- documented that the major healing process of the FFG is fibrosis of the fat tissue.[15.16] In maxillofacial surgery, the author was the first to investigate and report the clinical and histologic healing process of the FBFG. In that report, immature fibrosis was seen one month and mature fibrosis of the FBFG was seen at 4 months after the surgery. [18] Kablan and Laster discussed in their article the disadvantages and limitations of the pedicled BFP, and showed the advantages of the FBFG, especially its use in the entire oral cavity. [18] In the present paper, an additional clinical application of the FBFG is reported. The FBFG was utilized to reconstruct small to medium size defects in the hard palate. The meticulous, but ease, handling of the graft at the recipient site, with excellent suturing to secure and adapt the FBFG to the recipient bed left the graft stable and enhances its healing and its fast epithelialization.During the follow up period of the patients, the clinical and the histological healing process was the same like the stages that were previously reported by the author(19). During the clinical follow-up period of the cases, epithelialization of the graft began at the second week. The epithelium grew from the borders of the graft at the interface with the recipient site soft tissue, at this stage the 64 10th World Cleft Lip, Palate & Craniofacial Congress

majority of the flap still exist, but by 4 to 6 weeks after the surgery the FBFG was completely epithelialized, and the recipient site was covered with smooth soft tissue. After 3 to 4 months post-surgery the recipient site color was the same as the surrounding tissue. At this stage it was shown histologically by the previous publication of the author, that the FBFG was covered with oral epithelium after the completion of its healing (exactly as the recipient site epithelium), with fibrous healing inside the graft(18). With our rich experience in the use of the FBFG, as a soft tissue graft in the oral cavity, the FBFG has several advantages over the PBFPF that include; 1- less fat volume should be extracted from the BFP to reconstruct the same defect because there is no loss of fat tissue for the pedicle. 2. There is no limiting in mouth opening after the surgery. 3. The vestibular depths dose not affected. 4 There is no fear of dental injury to the graft in full arches patients. 5. The main advantages of the FBFG that there are no anatomical limitation in the use of BFP,CONCLUSIONS and the FBFG can be used in any region at the oral cavity.

In this patient series, the buccal fat pad free graft (FBFG) was proven as a simple procedure that can be performed quickly with minimal morbidity. The donor sites healed very well, without any cosmetic disturbances, making the harvesting of FBFGs a minor and insignificant procedure. The ability of the FBFG to survive its free transfer and the nature of healing that is the same as publishedthe healing in of the the future. pedicled BFP is encouraging the use of BFP as free graft. Other clinical applications of the FBFG have been performed by the author and will be

Aknowledgment to Dr Moti Twezer for his technical support. Financial support and sponsorship - ConflctsNil. of interest

There are no conflcts of interest. and follow up. Table 1: Patients and the treated lesions; location, etiology, defect size,

65 10th World Cleft Lip, Palate & Craniofacial Congress

REFERENCES 01. Gullanc PJ. Palatal island flap for reconstruction of oral defects. Arch Otolaryngol 1977;103:598-9. 02. Diaz FJ,DeanA, Alamillos FJ, Naval L, Fernandez J, Monje F. Tongue flaps for reconstruction of the oral cavity, Head Neck 1994; 16:550. 03. Anavi Y, Gal G, Silfen R, Calderon S. Palatal rotation-advancement flap for delayed repair of oroatral fistule: a retrospective evaluation of 63 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 95(50:527-34. 04. Seckel NG. The palatal island flap on retrospection. Plast Reconstr Surg 1995;96:1262- 1268. 05. Moghe S, Pillai AK, Prabhu S, Nahar S, Kartika UK. Pleomorphic adenoma of the palate. Report of a case. Int J Scientific Study 2014;2(1):54-56. 06. Alkan A, Enal S. Closure of palatal defects following excision of palatal pleomorphic adenoma. J Contemp Dent Pract2008;9(6):99-107. 07. Lehman JA, Curtin P, Hass DG. Closure of anterior palate fistulae. Cleft Palate J. 1978;15:33-38. 08. Murth J. Descriptive study of management of palatal fistula in one hundred and ninety-four cleft individuals. Indian J Plast Surg 2011;44(1):41-46.

66 10th World Cleft Lip, Palate & Craniofacial Congress 09. Erdogan O, Esen E, Ustan Y. Bony palatal necrosis in a diabetic patient secondary to palatal rotational flap. J Diabetec Complications.2005;19:364-7. 10. Egyedi P. Utilization of the buccal fat pad for closure of oro-antral and/or oro-nasal communications. J Maxillofac Surg 1977;5:241-4. 11. Singh J, Prasad K, Lalitha RM, Ranganath K. Buccal pad of fat and its applications in oral and maxillofacial surgery: A review of published literature (February) 2004 to (July) 2009. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110:698-705. 12. Tideman H, Bosanquet A, Scott J. Use of the buccal fat pad as a pedicled graft J Oral Maxillofac Surg 1986;44:435-40. 13 . Loh FC, Loh HS. Use of the buccal fat pad for correction of intraoral defects: Report of cases. J Oral Maxillofac Surg 1991;49:413-6. 20. 14..Martín-Granizo R, Naval L, Costas A, Goizueta C, Rodriguez F, Monje F, et al. Use of buccal fat pad to repair intraoral defects: Review of 30 cases. Br J Oral Maxillofac Surg 1997;35:81-4. 15. Shiffan MA (Ed). Autologous fat transfer. Art, science and clinical practice. Springer- Verlag Berlin Heidelberg; 2010. Part I p. 3-40. 16. Nguyen A, Pasyk KA, Bouvier TN, Hassett CA, Argenta LC. Comparative study of survival of autologous adipose tissue taken and transplanted by diffrent techniques. Plast Reconstr Surg 1990;85:378-86. 17. Neder A. Use of buccal fat pad for graft. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1983;55:349-50. 18. Kablan F, Laster Z. Th use of free fat tissue transfer from the buccal fat pad to obtain and maintain primary closure and to improve sof tissue thickness at bone-augmented sites: Technique presentation and report of case series. Int J Oral Maxillofac Implants 2014;29:e220-31. 21. 19. Kablan F: The use of buccal free fat graft in regenerative treatment of peri-implantitis; A new and predictable technique. Ann Maxillofac Surg2015;5:178-185.

Fig 1 – Harvest of the FBFG. Fig 1a – Small horizontal incision above the second and third maxillary molars. Fig 1b and c – The fat tissue is reached with blunt dissection. Fig 1d – The fat tissue is easily mobilized to the oral cavity. Fig 1e – The desired fat tissue volume is harvedted. 67 10th World Cleft Lip, Palate & Craniofacial Congress

Fig 1f – The FBFG. Fig 1g – suturing of the donor site incision. Fig 2 – Case 1 Fig 2a – Pleomorphic adenoma left palate. Fig 2b – Study model that demonstrates the palatal lesion. palate. Fig 2c – The planed excision of the tumor, the posterior border involves the soft

Fig 2d – Intraoperative view, the surgical palatal defect. Fig 2e – The macroview of the resected specimen (2-3cm). Fig 2f – The FBFG (2-4cm). Fig 2g – Intraoperative view, the FBFG at the recipient site Fig 2h – The FBFG after suturing to the recipient site. Fig 2i – One week follow-up Fig 2j – Extaroral view after one week, uneventful recovery. Fig 2k – Two weeks after the surgery demonstrates signs of healing with granulation tissue at the peripheral regions of the FBFG. Fig 2l - One month follow-up; epithelialization of the FBFG with fat remnants and fistula at the anterior border of the soft palate. Fig 2m – two months follow-up, spontaneous closure of the soft palate fistula and complete epithelialization of the FBFG. Fig 2n – 13 months follow –up. Fig 3-Case 2: Odontogenic lesion Fig 3a – Buccal side clinical view. Fig 3b – Palatal side clinical view; swelling at the anterior lateral area. root. Fig 3c – Cumputed tomography; radiolucent lesions with resorbtion of tooth 12

Fig 3d – Intraoperative view; a solid lesion. Fig 3e – primary closure of the soft tissue contraction. Fig 3f – two months after the first surgery; soft tissue dehiscence and exposure 68of root 12. 10th World Cleft Lip, Palate & Craniofacial Congress

Fig 3g – buccal view at two months. Fig 3h – surgery two, resection of tooth 12 root. Fig 3i – Excision and refreshment of the soft tissue dehiscence . Fig 3j – FBFG donor site. Fig 3k – The FBFG. Fig 3l – suturing and fixation of the FBFG at the recipient site. Fig 3m – FBFG, two weeks follow-up. Fig 3n- 12 months follow-up after the second surgery. Fig 4- A case Fig 4a- Fat remnants at two weeks after the fix of the iatrogenic dehiscence with FBFG Fig 3b- tree months after the surgery, good healing.

69 10th World Cleft Lip, Palate & Craniofacial Congress

Treatment of Congenital Malformations of the Mandible

Manlio Galiè EACMFS Education & Training Officer, Unit of Cranio Maxillo Facial Surgery, Center for Orbital Pathology & Surgery, ( Director and Chief: Prof. Luigi C. Clauser ), St. Anna Hospital and University, FERRARA – Italy

ABSTRACT

years. Distraction Osteogenesis (DO) has been one of the most innovative concepts in craniomaxillofacial syndromology and surgery through the last 25

After a quarter of century of extensive use DO has today specific indications for congenital craniofacial and cleft deformities. Technology has evolved from the first application of external devices to intraoral and hybrid or semiburied techniques. In congenital craniomaxillofacial anomalies distraction is indicated during growth. Mandibular distraction osteogenesis ca n be safely and effectively used to avoid or remove tracheostomy in neonates with severe airway obstruction caused by micrognatia in Pierre Robin Sequence. For selected newborns, mandibular DO will allow for avoidance of a tracheostomy and improved oral feeding. A careful evaluation of the patient’s airway and feeding must be performed and evaluated by a multidisciplinary Team approach. In syndromic micrognathia early mandibular distraction seems to be an appropriate indication. Benefits are functional, morphological, aesthetic with consistent psychological and familiar positive effects. patients.An increased incidence in TMJ complication during distraction osteogenesis in neonates has been recently reported, expecially in syndromic

70 10th World Cleft Lip, Palate & Craniofacial Congress

Guidelines for patient specific jaw line definition with titanium implants in aesthetic, deformation and malformation surgery

Maurice Y. Mommaerts Professor & Head, European Face Centre, Chirurgie Maxillo-Faciale et Plastique de la Face, Brussel, Belgium

Abstract: Context

Aims Asymmetry and unfavourable aesthetics of the jawline

The aim was to provide aesthetic, technical, and operative guidelines for mandibular angle and border augmentation using patient-specific titanium implantsSettings andmade Design by selective laser melting.

MethodsUniversity and Material Hospital – Prospective registry

Twelve patients and 17 implantation sites were documented and prospectively registered. Malformational, deformational, and purely aesthetic indicationsStatistical analysis were encountered. used

ResultsNA

Patient satisfaction was high, probably because the patients had input into the planned dimensions and shape. A serious infection with implant removal occurred in one patient who had six previous surgeries at the same sites. Technical and surgical guidelines were developed including splitting implants into two segments when the mental nerve was at risk, using a three- dimensional (3D) puzzle connection, providing at least two screw holes per71 10th World Cleft Lip, Palate & Craniofacial Congress segment, using scaffolds at the bony contact side, using a “satin” finish at the periosteal side, referring to anatomical structures where possible, making provisions for transbuccal and transoral fixation, using a high vestibular incision, and using a double-layer closure. Aesthetic guidelines are discussed butConclusions could not be upgraded.

Mirroring techniques and 3D print accuracy up to 0.1 mm allow precise planning of jaw angle implants. Patients are pleased when given preoperative renderings for their consideration. Infections can be managed using technical andKey-words operative recommendations and careful patient selection.

Key MessagesMandible, Prostheses and implants, Patient-specific modeling

New technologies necessitate disruptive approaches from the consumer as well. In jaw line contouring with CAD CAM titanium implants, the design warrants knowledge of both production and implantation restrictions. Provided guidelinesIntroduction will help both surgeon and engineer to reduce complications.

Little has been published over the last 25 years on achieving mandibular jawline definition with implants. Whitaker (1991) advocated the use of Teflon/carbon implants (Proplast®, Vitex, Houston, TX, USA) that are subperiosteally placed, lateral to the angle, and without screw fixation. Aiache (1992) described silicone implants available in three sizes that are anatomically carved to the case and placed subperiosteally without screw fixation. In contrast to Whitaker (1991), Aiache lengthened the vertical ramus by having the implant straddle the lower border. Semergides et al. (1995) described a series of 18 patients in whom a submandibular approach was used to place porous ethylene implants (Medpore®, Stryker, Kalamazoo, MI, USA) that also increased posterior height and were transbuccally fixed with two to three screws. The pterygomasseteric sling was reconstructed upon closure, but the final results were not quantified. Yaremchuk (2000) described a series of 11 cases with transorally placed porous ethylene implants (Medpore®), also increasing posterior height and using transbuccal screw fixation. In 2010, Bastides and Zide warned against vertical lengthening, frankly stating that “the masseter cannot be lengthened.” They felt that porous polyethylene or silicone implants72 should not be placed via a facelift incision or Risdon approach, as the 10th World Cleft Lip, Palate & Craniofacial Congress

pterygomasseteric sling will be disrupted, causing the masseter to bulge upon clenching and exposing the lower border of the implant (also demonstrated by Thomas and Yaremchuk, 2009). They too advocated screw fixation. Even with the advantage of screw fixation, asymmetrical results and infectious complications remain issues for many facial surgeons and are reasons not to perform implantation at this visible site. With the advent of additive manufacturing, software is now available that allows precise preoperative design on segmented computerized tomography data using mirroring techniques (3-matic®, Materialise, Heverlee, Belgium; Geomagic Freeform Plus®, 3DSystems, Darmstadt, Germany). This is particularly useful for bilateral aesthetic features like jaw angles and jaw lines. The aim of this study was to present clinically interesting design requirements for jaw line demarcation using titanium implants manufactured by selective laserSubjects melting and (SLM).Methods 1.1. Preoperative planning 1.1.1. Anthropometrical guidelines

Assessing abnormalities and planning corrections are not easy tasks when a patient presents with symmetrical hypoplasia. A proper substrate is missing. Constructs can be made on frontal and profile clinical pictures of a face and compared with ideal proportions and inclinations. Contemporary guidelines exist for men (Mommaerts, 2016) but not for women. For female patients, another approach is more attractive. Women can usually describe which celebrity’s angle or jawline they desire. If the surgeon is lucky, a pure profile or frontal picture of that celebrity is available on the Internet. The patient’s and celebrity’s pictures can be superimposed, and the difference provides an idea of the desired corrections, even down to the millimeter (Fig. 1, Büttner and Mommaerts, 2015). Soft-to-hard tissue ratios

73 10th World Cleft Lip, Palate & Craniofacial Congress have not been published for this sensitive three-dimensional (3D) area. We simply assume that 1 mm of hard tissue augmentation results in 1 mm of soft thetissue patient. augmentation in lateral and vertical directions. This has to be handled with caution, but it provides at least an impression that can be discussed with 2.1.2 Technical design guidelines

Fibrosis and a scarred buccal vestibule from multiple orthognathic, reconstructive, and/or implantation surgeries may prompt a surgeon to access the mandibular border via a submandibular approach. In the present series, the author was not tempted or urged to do so. When choosing the transoral route, maximal interincisal mouth opening, reduced intercommissure width, and reduced lip elasticity may pose a problem when considering voluminous implants. This is often the case in hemifacial microsomia patients for whom orthognathic surgery, ramus reconstruction, and macrostomia correction have been previously undertaken (Mommaerts, 2015). Inserting voluminous implants may also jeopardize the mental nerve, especially when they extend below the mental foramen. For both of these reasons, splitting the implant in two parts may be considered. The posterior and anterior segments can be positioned with a front-to-back and back-to-front action, respectively. The tip of the implant is slipped under the mental foramen after subperiosteal dissection of this area has been undertaken via extension of the buccal incision into the labial vestibule (Fig. 2).

Precise reassembling of the two segments within the wound cavity is best performed with an interlocking design, such as a 3D puzzle connection (Mommaerts et al., 2016). The use of one or two 3D puzzle connections does not make a difference in the author’s experience (Fig. 2). However, an oversized 3D design may hamper insertion of the second segment because more lateral soft tissue stretch is required (Fig. 3). Mirroring a healthy side to a deficient one may prompt the designer to simulate the cranial part accordingly large. This is less important for jaw line definition and may be the cause of wound dehiscence, so it is wise to compromise.74 10th World Cleft Lip, Palate & Craniofacial Congress The implant may straddle the lower border halfway, but the surgeon on the border. should not be forced to strip the pterygomasseteric sling to set the implant tight

Porosities (scaffolding) are useful where there is bony contact (Fig. 4). The friction provides primary stability as with any screw- fixed plate (Cordey et al., 2000). Porosities >500um are osteoinductive. Fukuda et al. (2011) demonstrated osteoinduction as deep as 5 mm in channels with a diameter of 500 um or more within SLM-manufactured titanium implants placed in an non-osseous site, namely the dorsal muscles of Beagle dogs. Scaffolding increases the overall elasticity to more closely approximate that of bone ( et al., 2004) and therefore reduces stress shielding and premature loosening of an implant fixed to the weight-bearing mandible. It also helps in weight reduction. Hence, it makes sense to biofunctionalize the area of bony contacts using scaffolds with a diamond unit cell structure ≥500 um for a few millimeters deep. Sandblasting and acid etching further promote osteoconductivity (de Wild et al., 2013). The lateral surface is micro-shot-peened (250-um Al2O3 broken beads) to obtain a “”satin” finish with a roughness average (Ra) of N7-N10. A highly polished surface may discourage periosteum reattachment, whereas a very rough surface may encourage bacterial growth when contamination with saliva occurs (Mohan et al., 2016). Two screws per implant segment are necessary for proper fixation. Masseter muscle action may otherwise dislocate the posterior segment. In the author’s experience, a single screw in an anterior segment may not prevent rotation. The screw holes are designed with respect to the inferior alveolar nerve using image segmentation software (Mimics Medical 19.0, Mimics Innovation Suite, Materialise, Heverlee, Belgium). Depending the location of the implant and the estimated freedom of access, screw holes are provided in the implant body or in lip extensions (Fig. 4). The extensions are also used to refer to anatomical structures (e.g., molars) for improved initial orientation. When in the jaw angle area, the author recommends providing holes for both the transoral and transbuccal approach, with the latter as a rescue measure (Fig. 5). Countersinking is not required. The author prefers screws with75 10th World Cleft Lip, Palate & Craniofacial Congress

external pentagon-shaped heads (2.3- mm diameter; Surgi-Tec NV, Gent, explantation.Belgium) that facilitate screw removal during placement or eventually upon

extensionAlthough of the implant lateral augmentation, augmentation of the chin initially seems to be a logical

the author prefers a chin osteotomy when chin augmentation is required. With an osseous genioplasty, height reduction can also be obtained. With the advancement of the lower mandibular border, the digastric and geniohyoid muscles are stretched with a positive functional (increase of airway) and esthetic (decrease of mentocervical angle) effect on hyoid position. Jaw line augmentation with titanium implants and chin osteotomy can be done simultaneously (Fig. 6). The choice of titanium over polyetheretherketone (PEEK) is based on the European belief that it is better to prevent long-term complications caused by non-osseointegrated PEEK implants than to handle those complications associated1.2. Operative with osseointegrated technique titanium (FDA philosophy).

The intraoral approach to the jaw angle is made with an incision based on the external oblique line, extending anteriorly, staying 1 cm above the lower recess76 of the vestibule, and avoiding the long buccal nerve. Anterior extension 10th World Cleft Lip, Palate & Craniofacial Congress

continues into the lower lip when the jowl area also needs correction or when voluminous angle implants are placed. A high incision is likely to minimize postoperative saliva spillage into the wound. Watertight closure of the oral mucosal membrane is difficult to guarantee. Enlarging the wound cavity for augmentation inferior and/or posterior to the mandibular border can be performed by dry gauze packing rather than with sharp dissection. Uniform expansion with gauze will lead to periosteum distention and pocket formation rather than perforations. This protects the mandibular branch of the facial nerve in hemifacial microsomia cases, where its course is unpredictable. It also prevents opening the superficial neck to the oral cavity. Intraoperative 3D imaging (e.g., with the BV Pulsera fluoroscopy system, Philips Medical, Eindhoven, the Netherlands) will confirm correct positioning. Infection control is further maintained by copious wound rinsing, rinsing the implant in rifampicin solution, double-layer closure (i.e., horizontal running mattress suture for deep approximation and eversion, superficial running for the epithelial border approximation), application of fibrin glue (Tisseel, Baxter) underResults: the suture line, and 5 days of penicillin perorally.

The guidelines described in section 2 resulted from the author’s experience with this small group. Two male patients insisted on having larger implants than originally designed. Postoperatively, one was pleased with the results, whereas the other admitted to having underestimated the result. No revisions were required to correct design errors. In the end, all patients were satisfied. Complications arose in three patients. One had already undergone several operations (i.e., three orthognathic surgeries and one alloplastic jaw angle implantation with two revision surgeries), each time with an intraoral vestibular incision. The titanium implants were big, and wound dehiscence and infection occurred. The implants were removed, and a smaller set will be inserted via a Risdon approach. A wound dehiscence also occurred in a hemifacial microsomia monthscase, which later. caused no frank infection and was permanently closed during the gluteal fat transplantation (Mommaerts, 2015) and macrostomia correction 3

In one patient, a small implant segment rotated around a single screw fixation, necessitating correction 1 week later using local anesthesia only. No 77 permanent sensory disorders were observed. 10th World Cleft Lip, Palate & Craniofacial Congress

Discussion

Hemifacial microsomia and (revisional) orthognathic surgery patients comprise two common indications for jaw angle and jaw line augmentation. Both are easily planned with mirroring techniques using commercially available image-processing software. Aesthetic augmentation is more challenging. One side has to be designed “de novo,” and the patient’s perception of their ideal jaw definition must be conveyed to the surgeon and designer. A titanium implant is then an all-or-nothing issue. These patients prefer a fee-for-result rather than fee-for-effort policy. It is imperative to discuss the design using renderings or a dynamic PDF obtained from the planning software before manufacturing. All 12 of the patients were satisfied. In Ridwan-Ramana et al.’s (2015) series of 27 porous ethylene implants placed at the mandibular angle in 11 patients, 18.2% were dissatisfied. Communication with the surgeon revealed bythat the the patient. actual percentage might be higher because of the retrospective nature of the study. This underscores the importance of preoperative design approval

Ridwan-Ramana et al. (2015) reported a 27.3% infection rate when using porous polyethelene in the mandibular angle area. As porous ethylene is not osseointegrating (Maas et al., 1990), it may become infected because fibrovascular ingrowth takes 3 months (Ridwan-Ramana et al. (2015). The implant can also migrate when not fixed with long enough screws, which may cause it to be exposed and extruded (Menderes et al., 2004). Aiache (1992) mentioned double closure over silicone implants, and Yaremchuk (2000) mentioned “a generous intraoral mucosal incision 1 cm high in the sulcus at its labial side,” to which the author now adheres. In Whitaker’s (1991) experience, autologous (calvarial bone) onlays and sandwich osteotomies yielded unpredictable results because of resorption and78 symmetry issues, and he discontinued their use. Triaca et al. (2015) used 10th World Cleft Lip, Palate & Craniofacial Congress

an extended chin osteotomy using frequent bone grafts and posterior design corrections with the “chin-wing” technique. Results regarding symmetry, fracture, and infection complications are not yet available at the time of writing. Patient-specific PEEK implants have the advantage of elasticity near the bone (Kim et al., 2009). Medical-grade PEEK (Optima LT, Invibio, West Conshohocken, PA, USA) is as expensive as 3D-printed titanium alloys, but its behaviorReferences when transorally implanted is not yet known. 01. Aiache AE. Mandibular angle implants. Aesthetic Plast Surg 16: 349-354, 1992. 02. Bastidas N, Zide BM. The treachery of mandibular angle augmentation. Annals Plast Surg 64: 4-6, 2014. 03. Büttner M, Mommaerts MY. Contemporary aesthetic management strategies for deficient jaw angles. PMFA News 2 (4), 6-9, 2015. 04. Büttner M, Mommaerts MY. Photoshopping the face: simulated outcomes of orthofacial surgery. PMFA 2 (2), 6-10, 2014. 05. Cordey J, Borgeaud M, Perren SM. Force transfer between the plate and the bone: relative importance of the bending stiffness of the screws friction between plate and bone. Injury 31, Suppl 3: C21-28, 2000. 06. de Wild M, Schumacher R, Mayer K, Schkommodau E, Thoma D, Bredell M, Kruse Gujer A, Grätz KW, Weber FE. Bone regeneration by the osteoconductivity of porous titanium implants manufactured by selective laser melting: a histological and micro computed tomography study in the rabbit. Tissue Eng Part A 19: 2645-2654, 2013. 07. Fukuda A, Takemoto M, Saito T, Fujibayashi S, Neo M, Pattanayak DK, Matsushita T, Sasaki K, Nishida N, Kokubo T, Nakamura T. Osteoinduction of porous Ti Implants with a channel structure fabricated by selective laser melting. Acta Biomater 7: 2327-2336, 2011. 08. Kim MM, Boahene KD, Byrne PJ. Use of customized polyetheretherketone (PEEK) implants in the reconstruction of complex maxillofacial defects. Arch Facial Plast Surg, 11; 53–55, 2009. 09. Lin CY, Kikuchi N, Hollister SJ. A novel method for biomaterial scaffold internal architecture design to match bone elastic properties with desired porosity. J Biomech 37: 623-636, 2004. 10. Maas CS, Merwin GE, Wilson J, Frey MD, Maves MD. Comparison of biomaterials for facial bone augmentation. Arch Otolaryngol Head Neck Surg, 116;. 551–556, 1990 11. Mandibular angle. J Oral Maxillofac Surg. 54:1417–1423, 1996. 79 10th World Cleft Lip, Palate & Craniofacial Congress 12. Menderes A, Baytekin C, Topcu A, Yilmaz M, Barutcu A. Craniofacial reconstruction with high-density porous polyethylene implants. J Craniofac Surg, 15; 719–724, 2004. 13. Mohan K, Cox JA, Dickey RM, Gravina P, Echo A, Izaddoost SA, Nguyen AH. Treatment of infected facial implants. Semin Plast Surg 30: 78-82, 2016. 14. Mommaerts MY, Büttner M, Vercruysse H Jr., Wauters L, Beerens M. Orbital wall reconstruction with two-piece puzzle 3D printed implants: technical note. Craniomaxillofac Trauma Reconstr 9: 55-61, 2016. 15. Mommaerts MY. Hemifacial microsomia: management of the vertical ramus compartment. Plast Aesthet Res 2: 98-106, 2015. 16. Mommaerts MY. The ideal male jaw angle – an internet survey. J Craniomaxillofac Surg 44; 381-391, 2016. 17. Ridwan-Pramana A, Wolff J, Raziei A, Ashton-James CE, Forouzanfar T. Porous polyethylene implants in facial reconstruction: Outcome and complications. J Craniomaxillofac Surg 43: 1330-1334, 2015. 18. Semergidis TG, Migliore SA, Sotereanos GC. Alloplastic augmentation of the 19. Thomas M, Yaremchuk MJ. Masseter muscle reattachment after mandibular angle surgery. Aesthet Surg J 29: 472-476, 2009. 20. Triaca A, Brusco D, Guijarro-Martinez R. Chin wing osteotomy for the correction of hyper-divergent skeletal class III deformity: technical modification. Br J Oral Maxillofac Surg 53; 775-777, 2015. 21. Whitaker LA. Aesthetic augmentation of the posterior mandible. Plast Reconstr Surg 87: 268-75, 1991. 22. Yaremchuk MJ. Mandibular augmentation. Plast Reconstr Surg 106: 697-706, 2000. Acknowledgement none Captions to figures

Figure 1. Photoshop simulation (C) of the jaw angle of Kim Kardashian (B) onto a patient’s original facial profile (A). Figure 2. Rendering of a two-part implant on the lateral mandibular border extending below the mental foramen (Geomagic Freeform Plus ®). osteotomy A. Mandibular shape after two orthognathic surgeries and one chin 80 10th World Cleft Lip, Palate & Craniofacial Congress

B. Implant design in two parts C. Connected double 3D puzzle design D. Anterior part showing the disconnected double 3D puzzle design puzzle design. E. Backside of the 3Dprinted and biofunctionalised assembly, with 3D

Figure 3. Medial part of a left-sided jaw border implant in two parts with a large 3D puzzle design. Each part has two holes for screw fixation. The scaffolding is clearly visible. Figure 4. Left (A) and right (B) single-piece jaw implants designed to extend inferiorly. The extension lips each have two screw holes. Porosities are provided where bone will come in contact. The periphery and lateral surface is “satin” finished to prevent contamination from saliva. Figure 5. Planning of screw hole placement below the mandibular canal: the upper and lower sets are for transoral and transbuccal fixation, respectively. Figure 6. A rescue orthognathic case after bimaxillary surgery and condylar resorption with loss of jaw angle definition and increased anterior facial height. Jaw angle reconstruction was performed together with a chin osteotomy (red arrow) with advancement and height reduction. The extension lips are depicted (white arrows) with reference to the distal side of the last molars. Figure 7. An aesthetic case before (A) and after (B) bilateral jaw angle implantation. Although the implants were rather small (C), the demarcation between the face and neck by surgical definition of the mandibular angle can be well appreciated.

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Management of obstructive sleep apnea in syndromic Patients

Adi Rachmiel Department of Oral and Maxillofacial Surgery, Rambam Medical Center, Haifa, Israel and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel,

Background and objectives

Obstructive sleep apnea in pediatric population is often associated with congenital craniofacial malformations due to hypoplastic mandible and decreased pharyngeal airway. A part of the patients were respiratory distressed and the other part with tracheotomy already. The patients were treated using externalMethods or internal distraction devices for mandibular lengthening.

The patients were treated by mandibular distraction using external distraction devices and internal distraction devices. Comparison between the twoResults methods and the advantages and disadvantages will be presented.

An increase in mandibular body and pharyngeal airway was obtained in all the patients. Using the external devices the average mandibular elongation was 31mm. They are less comfortable to the child, leave 2 visible skin scares, risk of pin loosening that need another procedure for pin replacement or may lead to shorter retention period with greater relapse. The removal of the external devices is simple. Using internal devices the average mandibular elongation was 23 mm. anaesthesia.They are more comfortable to the child with a precise and predictable vector of lengthening. However they need second operation for removal under general

In some cases in very young children with severe micrognathia, it was impossibleConclusions to place internal devices, and external devices were used.

82 Mandibular distraction osteogenesis permit marked increased airway. 10th World Cleft Lip, Palate & Craniofacial Congress

Internal devices are more comfortable to the child with a precise and predictable vector of lengthening. They leave less visible skin scar and should be consider first with the main disadvantage of second operation for removal under general anaesthesia. The external devices permit greater distraction length, however when it was impossible to place internal devices or when there is a need for orgreater shortening distraction the retention length, period. the external devices may be used with the main drawback for child discomfort and risk of pin loosening that need replacement

83 10th World Cleft Lip, Palate & Craniofacial Congress

Maxillary External Distraction Osteogenesis In Cleft Craneofacial Anomalies

Andrés Duque Craniomaxilofacial Surgery Dept. Roosevelt Institute, Maxillofacial Surgery Dept. Meissen Hospital E.S.E, Cleft Lip and Palate Program Colombian Air Force Reserve, Bogota-Colombia

Since its introduction of the maxillary osteotomy has become a widely accepted treatment for correction of midface hupoplasia. As long-term surgical experience grows and improvements are made in technique, equipment and perioperative care, the number of Lefort procedures performed worldwide is. Severe hypoplasia of the maxillary became a challenge and Distraction Osteogenesis is a therapeutic tool especially in the augmentation of the expansioncraniofacial of skeleton the maxilla. at this patient it has and advantage of eliminating bone grafts and alloplastic materials and can be done simultaneously with transversal

We explain different alternatives and evolution of the techniques and innovations of our team in the procurement for better results.

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Management of TMJ Ankylosis and Micrognathia in Children

Manlio Galiè EACMFS Education & Training Officer, Unit of Cranio Maxillo Facial Surgery, Center for Orbital Pathology & Surgery, ( Director and Chief: Prof. Luigi C. Clauser ), St. Anna Hospital and University, FERRARA – Italy,

ABSTRACT

Temporomandibular Joint Ankylosis (TMJA) is a severe disorder described as an intracapsular union of the disc-condyle complex to the temporal articular surface with bony fusion. The causes of ankylosis of the mandible can be classified as congenital, astraumatic, a whole. inflammatory and neoplastic. Idiopathic forms including enlarged coronoid processes have been considered as causes of restricted mouth opening

The management of these disabilities are challenging and rarely based on surgical and rehabilitation protocols. Failure and reankylosis are common. In pediatric age the aim of surgery is to recover morphology and function with normal growth, appropriate speech development, dental occlusion, and normal development of the facial skeleton. The best treatment is yet to be found because no consensus in the literature has been established. Surgical treatment must be tailor-made for each patient and adequate post-operative Rehabilitative Physiotherapy Protocol (RPP) is necessary to achieve success. Mandibular deficiency with ankylosis represents some of the most challenging cases in craniofacial surgery.

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Cleft care in Mongolia

Erdenetsogt J, G, Tserendulam D, Bayasgalan R, Bulgan B, Erdenesaikhan M National Center for Maternal and Child Health of Mongolia, Mongolian Cleft Lip and Palate Association, Mongolia

three Themillion Mongolia people located in central Asia, bordering with China and the Russian federation. It has 1,51 million square kilometres, with a population of around. . In the list of rate of population density Mongolia has2 240th place of 244 world countries. and 1,92 people in one square kilometer The most densely populated2 sovereign nation is Monaco, with a population density of 18,713 people/km Mongolia is cantoned into twenty-one provinces (aimags), and the capital Ulaanbaatar is administrated separately as a capital city with provincial status, in which lives over one million people. The National Center for Maternal and Child Health is located in the capital city, and it is a tertiary hospital which providing nationwide medical service for children. The Cleft team of this center is only and provides nationwide service for cleft patients throughout the Mongolia. 3

The4 national ratio of congenital cleft lip/palate is one for 1314 (2005), and 1072 (2012) live births in the Mongolia. According to these researches the ratio of this abnormality has been increasing in the Mongolia. The Mongolian cleft team has a multidisciplinary service for cleft patients, and consists of plastic/cleft surgeons, orthodontist, cleft-speech pathologist, ENT doctors, and the team coordinator. l5-7 We have a national Cleft Treatment Protoco . Under this protocol all primary cleft lip repair has been done in the patients` age of three months. There is only using the Millard technique and it`s modification for unilateral clefts, and forked flap method for primary bilateral lip repair. Also Tajima procedure is used for primary rhinoplasty together with every case of lip repair. The usage of Nasoalveolar molding is for every case by cleft treatment protocol, but currently can not perform it for every case because of a financial problem for those families who had not travel costs coming to the our center, especially 86far from capital provinces. 10th World Cleft Lip, Palate & Craniofacial Congress

The primary palatoplasty is done by Furlow double opposing Z-plasty, “Mongolian” tenchique, Langenbeck procedure, and the Bardach two long flap technique as well, depending on the classification of cleft palate, in the patients` age of one year. Than speech treatment continues from two year until six year. We conduct an investigation as a Nasopharygoscopy for every child, who underwent palatoplasty at the 3-4 years of patients` age. If the ratio of closure of nasopharyngeal valve is over 0,95, will continue only speech treatment. The secondary palatal procedures such as superior and inferior based pharengeal flap, and Z-plasty in the soft palate for lenthening it are used by recommendation of team speech pathologist, and choosing of above mentioned techniques in depending on severity of VPI, if the ratio is minimal than 0,9. Secondary lip revision and scar revision of the lip are made at the growth.patients` age 5-6 if needed. Also orthodontic treatment begins at this same age for correction of the eruption of permanent teeth, and for control of maxillary

Alveolar bone graft surgery is performed before eruption canine, at the 8-12 years old, and also ear cartilage graft is used for secondary nasal correction at 12-13 years old. Finally, cleft orthognatic surgery is performed at over 16 years old patients, only with recommendation of team orthodontist. The Mongolian Cleft Lip and Palate Association was established in 2007. The activity of the MCLPA is focused on following two main points except daily procedures; 01. Supporting research, and improving knowledge about cleft for medical staffs and public 02. Organizing humanitarian cleft mission in the rural areas, and to expand of participation of team members in international cleft mission The members of Cleft team of Mongolia attend constantly to the scientific conferences and workshop of ICPF and International Association of Oral-. Maxillofacail surgeons with the support of MCLPA, and sharing experiences8-16 with foreign doctors, and introducing the results of our research works Since 1997, cleft mission has being organized by Mongolian cleft team in Mongolian rural areas, in 4 times every year. Since establishing MCLPA, a staffsmissions. expand with a team care, not only cleft surgery, including orthodontic and speech17 treatment, and having workshops for cleft care for local medical 87 10th World Cleft Lip, Palate & Craniofacial Congress

From 2013, the members of our Cleft team as a plastic/cleft surgeon, anaesthesiologist are regularly attend international humanitarian cleft missions with Smile Asia in the Asian countries, Myanmar (2013, 2014), Indonesia (2015),Reference and China (2016). 01. https://en.wikipedia.org/wiki/Mongolia#Administrative_divisions, Oct., 2016. 02. https://en.wikipedia.org/wiki/List_of_countries_and_territories_by_population_density, Oct., 2016. 03. Ariuntuul G, Furukawa H, Uetani M, et al. The prevalence of cleft lip and/or palate in Mongolia. J. Jpn. Cleft Palate Assoc., 31:267-273, 2006. 04. Ayanga G, Davaanyam L, Purevjav N, and Ariuntuul G. “Cleft palate repair in Mongolia: Modified palatoplasty vs conventional technique”. Annals of Maxillofacial surgery, vol2, issue 2, 2012, p-131-35. 05. Health care technology-Surgical treatment of congenital cleft lip, www.estandard.mn, 2011. 06. Health care technology-Surgical treatment of congenital cleft palate, www.estandard.mn, 2011. 07. Health care technology-Orthodontic treatment of congenital cleft lip/palate www. estandard.mn, 2011. 08. Ayanga G. New Technique of Palatoplasty. 6th world cleft lip and palate congress of ICPF conjunction with 14th congress of KCPA, Live Surgery Video Session, June, 2010, Seoul, Korea. 09. Erdenesaikhan M, Ayanga G, Bayasgalan R, et al. Prevalence and types of nonsyndromic CLP in Mongolia. 6th world cleft lip and palate congress of ICPF conjunction with 14th congress of KCPA, June, 2010, Seoul, Korea. 10. Ayanga G, Erdenetsogt J, Tserendulam D, et al. Comparative result of palatoplasty techniques in Mongolia. 6th world cleft lip and palate congress of ICPF conjunction with 14th congress of KCPA, June, 2010, Seoul, Korea. 11. Ayanga G, Bayasgalan R, Tserendulam D, et al. The role of NGO in the care for patients with clefts: Mongolian experience. 3rd workshop of ICPF, June, 2011, SanFrancisco, USA. 12. Ayanga G, Bayasgalan R, Tserendulam D, et al. Fistula rate for new modified “Mongolian” technique for congenital cleft of the palate. 20th Conference of International Association on Oral and Maxillofacial surgeons, November, 2011, Santiago, Chile.

88 10th World Cleft Lip, Palate & Craniofacial Congress 13. Ayanga G, Erdenetsogt J, Bayasgalan R, et al. The closure of postpalatoplasty fistula with local turn-down flap. 7th world cleft lip and palate congress of ICPF, May, 2012, Mahe, Seychelles. 14. Bayasgalan R, Tserendulam D, Ayanga G, et al. Some risk factors of odonthogenic phlegmona among Mongolian children. 21th Conference of International Association on Oral and Maxillofacial surgeons, October, 2013, Barcelona, Spain. 15. Erdenesaikhan M. Association between air pollution oral cleft defects risk in Ulaanbaatar, Mongolia. 22th Conference of International Association on Oral and Maxillofacial surgeons, October, 2015, Melbourne, Australia. 16. Ayanga G, Erdenetsogt J, Tserendulam D, et al. Velopharyngeal function after primary palatoplasty: relevance of age at repair, cleft type, and surgical technique. 21th Conference of International Assosiation on Oral and Maxillofacial surgeons, October, 2015, Melbourne, Australia. 17. Ayanga G, Erdenetsogt J, Tserendulam D, et al. Cleft mission in Mongolian rural areas: 15 years experience. 1st International conference on humanitarian medical missions, Singapore, November, 2014.

89 10th World Cleft Lip, Palate & Craniofacial Congress

Effectiveness and Patients Satisfaction of One Stage Correction of Secondary Cleft deformity- Indian Scenario

Abhay datarkar Professor and Head in Department of Oral & Maxillofacial Surgery, Govt. Dental College & Hospital, Nagpur, Project Director of Centre of Excellence For Craniofacial Surgery, Govt. of Maharashtra, India

Introduction

Secondary cleft deformity is one of the common problems the cleft patients are suffering. The initial (primary) repair of the cleft lip and palate is usually completed during the first year of life. The secondary corrective surgical procedures range is quite wide and varied and requires the interventions till the child achieves the complete growth. It includes procedures to improve the appearance of the lip and nose, to improve the function of speech, to close abnormal communications between the mouth and nose, and even procedures to move and stabilize the upper and lower jaws. In India because of the low socioeconomic strata and the lack of awareness of the secondary corrective surgeryAim the treatment of the patients with cleft deformity is usually deferred.

To evaluate effectiveness and patients satisfaction of One Stage Correction ofMaterial Secondary and Cleftmethods deformity

32 patients of the secondary cleft deformity reporting for the correction of the deformity after 21 years of the age were included in this study. The mean age of the patients ranging from 21to 28 years who underwent secondary anycleft further surgeries treatment. for various reasons. The patients had undergone the primary correction of the cleft lip & palate at the age of one year and have not received

Surgical procedures included the alveolar bone grafting, correction of the jaw deformity, closure of the palatal fistula, rhinoplasty procedure and redo of the lip for improvement of the face aesthetics. Either of the procedures in combination90 were done in all these 32 patients as the single stage procedures. 10th World Cleft Lip, Palate & Craniofacial Congress Observation &Results

Our observation is in India due to low socioeconomic strata and less bothering of the physical appearance in society, patients usually report for secondary correction at the time of marriageable age typically 3-6 months before the marriage. It is very difficult to plan the correction systematically and we need to do the correction in single stage because of time constraint. Combinations of procedure like redo lip, alveolar bone grafting, jaw correction surgery, repair of tissue done simultaneously or in combination in all the patients. Results of the study are acceptable in terms of patient’s satisfaction,Conclusion functional outcome.

The final result of cleft lip and palate treatment not only depends on the extent of the inborn defect, the development of the affected area, individual healing ability, and the therapeutic method used, but also on additional factors such as the patient’s cooperation and the subjective perception of his or her surgeries,deformity. additional We concluded stress, thattime combinationand money. of various secondary surgical procedures done simultaneously, which can help the patients save multiple

91 10th World Cleft Lip, Palate & Craniofacial Congress

Cleft Lip and Palate Management in a Rural Camp Madaripur in Bangladesh

Jalal Uddin Mahmood, Mahbubur Rahman Khan. Mesch Ong’uti, Anthony Wongs Associate Professor and Head, Department of Periodontology, Dhaka Dental College. Bangladesh

Introduction

Cleft lip and palate is a congenital malformation. Baby born with cleft lip and palate brings unhappiness to the family. Prevalence and incidence of cleft and palate is high in Bangladesh. It affects the function and esthetics of individual. Timely treatment of these defects increases social acceptance and preventsMaterials complication and Methods in adult life.

Every year we work in rural cleft lip and palate camp in Madaripur district in Bangladesh. Camp consists of six days –first day opening ceremony and case selection . From second day we start surgical procedure of both infant and adult cases. Unilateral cleft lips were repaired by rotational advancement with small triangular flap- method. Bilateral clefts were repaired using one stage repaired method. Cleft palate were repaired by mucoperiosteal push back or furlow technique. Adult cases were repaired with local anesthesia with sedation and infant treated under GA. Simultaneous palatoplasty performed very carefully as riskResults of complication is high.

Surgical repair results of cleft lip and palate was very good. Post operative care after six days were taken in Madaripur district hospital and few complicated cases follow up given in Dhaka dental college hospital. Adult cheiloplasty under localDiscussion anesthesia save cost , time and manpower.

Cleft lip and palate repaired is a single or two stage surgical procedure. The real problem begain from the day baby born. Several problems faced by the parents, for instance feeding, nursing, regurgitation, esthetic, hearing and speech92 problem. Overall family criticisim and social acceptance of the child. To 10th World Cleft Lip, Palate & Craniofacial Congress

solveConclusion these problem needs multidiciplinary team approach.

Surgeon’s little efforts can bring smile and happiness to the family. Social awarenessIntroduction is equally important to reduce the incidence of adult cleft patients. palateCleft lip and palate is most common congenital malformation. An estimated 300,000 Bangladeshi are suffering with unrepaired cleft lips and

1. Around 4000 to 5,000 child born with this deformity in every year in Bangladesh 2. A significant number of this cases do not receive any treatment because lack of knowledge. Even they do not know that cleft can be fully corrected and its treatment totally free of cost. Baby born with cleft lip and palate brings unhappiness to the family. It affects the function and aesthetics of individual. Timely treatment of these defects increase social acceptance and prevent complication in adult life. The purpose of this paper to share experiences of treatment of cleft lips and palates in a rural Madaripur camp Materialsin Bangladesh and Method

Every year we work in a rural cleft lip and palate camp at Madaraipur district in Bangladesh. The camp last for 6 days. Cleft camp was publicise through local news paper, banners ,posters and loud speaker transmission. Our cleft team comprises Bangladeshi, Nepales, Kenyan and U.S surgeons , anesthesiologists and nurses. Foreign doctors leaving Dhaka city at the evening before the day of commencing camp. Ministry of health Govt. of Bangladesh allow us to use Madaripur district hospital’s operation theatre and other hospital facilities. Asmat Ali Khan Research Foundation, Bangladesh, organize the cleft camp . Fig 1 & 2

Fig 1. Location Madaripur District in Bangladesh Fig 2. Cleft team Doctors

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First day opening ceremony and case selection. Patients are selected the days before operation by cleft team and given appropriate pre operative advice. Fig. 3 Fig. 3 Opening ceremony, people gathering, patient selection and planning to work. From second day we start surgical procedures of both infant and adult cases. Fig. 4, Fig.5, Fig. 6 Fig 4. Cleft team working. At a time 3 patient’s operation was performed. Fig5. Pre and post ope. Cleft lip children

Fig 6. Pre and Post Ope. Cleft and Palate Repair Adult

94 10th World Cleft Lip, Palate & Craniofacial Congress Results

In 2012 total 104 case of clefts have been operated. Of them age group

3 months to 12 years was 77 (74.03%) cases and 67 (87%) were underwent cheiloplasty and 10 (13% ) were underwent cleft palate repair . Twenty seven patients (25.96%) were aged range 13 to 47 years. Twenty of these patients (74.07% ) had untreated cleft lip and underwent primary cleft lip repair. The remaining seven patient (25.92%) had a cleft palate underwent primary palatoplasty .Table 1 Surgical repair results of cleft lip and palate was very good. Out of 104 cases 95 (91.34%) cases wound healing was uneventful. Three cases( 2.88%)

have bleeding complain from palate , four cases (3.8%) case wound dehiscence and two cases (1.92%) infection noted, which was subsequently managed . Post operative care after six days were taken in Madaripur district hospital and few complicated cases follow up given in Dhaka manpower.dental college hospital. Adult cheiloplasty under local anesthesis save cost, time and

Table 2 . Complication Unilateral cleft lips were repaired by rotational advancement with small triangular95 10th World Cleft Lip, Palate & Craniofacial Congress flap method. Bilateral clefts were repaired using one stage repaired method. Cleft palate were repaired by mucoperiosteal push back or furlow technique 3,4). Adult cases were repaired with local anesthesia with sedation and infants treated under GA. Simultaneous palatoplasty performed very carefully as risk Fig 7. Children Pre and Post Ope. Cleft Lip and Palate. of complication is high.

After operation patient taken to recovery room and then back to the ward where nurses monitar them. They are kept in overnight and advised to takeDiscussion fluids for the rest of the day.

Cleft lip, alveolus and palate (hard and soft ) is common congenital deformity of the orofacial structures. A cleft occurs when certain midline soft tissues do not fuse together during development of the fetus. Nasolabial and bilateral muscles discontinuation on one side causes unilateral cleft lip, whereas bilateral cleft is the result of symmetrical defects.

Cleft palate is the result of incomplete development and fusion of the5,6 palatine shelves limited to only the soft palate , only the hard palate or both ).

Bangladesh has between 275,000 and 300,000 people1,7 currently living with CLAP amongest a population of almost 150 million ) . This calculate to just over 1 child in every thousand families .Though the majority7 of the sufferers come from the destitute and poorest segment of society ).

The incidence in UK and US is approximately8, 9,10 1 in 700 as opppsed to 1 in 500 in regions of Africa and South America ) . This shows incidence of cleft lip and palate ( CLAP) tends to be much higher in Bangladesh compared to the developed countries. Exact causes of the cleft lip and palate is unknown. But experts believe -clefts have multifactorial etiology namely: a) Genetic predisposition, b) Drugs intake in pregnancy, c)Alcohol and smoking, d)Maternal illness and infection,e) Early marriage and teen age pregnancy, f) Malnutrition (Lack of nutrition, Lack of vitamins and folic acids.) In Bangladesh all the factors may play role especially , early marriage and teen age pregnancy, maternal illness and infection. Malnutrition has significant role because majority of sufferers come from poor family 8) . In Madaripur camp any age group cleft lip and palate patients were operated.96 In many surgical textbooks recommended repair of cleft lip at 10 to 10th World Cleft Lip, Palate & Craniofacial Congress

12 weeks of age, followed by primary palatoplasty at 9 to 12 months of age . Surgical results of cleft lip and palate repair was good. Only nine cases have complications which was subsequently managed. There are limited number of oral and maxillofacial surgeons and plastic surgeons in Bangladesh 9,10) . As a result, an estimated 300,000 Bangladeshi are suffering with unrepaired cleft lips and palates 7) . In addition every year 4000 to 5000 babies born with Clefts 2) . Follow up, observation of patient, comprehensive care and absence of cleft board is an obstacle to establishing modern cleft lip and palate services in Bangladesh 1) . In the cleft camp as many as possible cleft repaired was done and in this respect it is a complete success. But we need to do observation , follow up of the patient and independence –financial support and more trained doctors to deal with the situation. Social awareness is equally important to reduce incidence of adult cleft patients. We also need comprehensive care . This include speech therapy, ortho-prosthodontics service and psychological counseling. This mean a multidisciplinary team approach is essential to bring CLAP patients to productive member of the Bangladeshi society.

We thanks all for support and contribution to CLAP camp at Madaripur inReferences Bngladesh 01. Ghani S, Mannan A.Sen S et al: The problem of establishing modern cleft lip and palate services in Bangladesh. J Surg.2:43.2004 02. Hope foundation for women and children in Bangladesh. News Bulletine.http//www. hopeforbangladesh.org.Nov-Dec.2010 03. Mulliken JB: Pricipal and techniques of bilateral complete cleft lip repair. Plast Reconstructy Surg. 75:477.1985 04. Salyer K. Primary bilateral ceft lip/ nose repair, in Bardach J, Salyer K (eds): Salyer and Bardach’s atlas of craniofacial and cleft Surgery. Volume 2. Philadelphia. PA. Lippincortt, 1999 . 543-607 05. Wll L: Growth and development in patients with untreated clefts. Cleft palate Craniofac. J 37:523.2000 06. Ward C. James I: Surgery of 346 patients with un operated cleft lip and palate in Sri Lanka. Cleft Palate J. 27:11.1990 97 10th World Cleft Lip, Palate & Craniofacial Congress 07. Sen SL ([email protected]). Mobile Cleft Lip Cam pre-assessment Data. Personal communication 2001. 08. Ahmed LA. Mending their fate. The Star Magazine.Bangladesh Daily Star. August12th 2001. (online) http://www.thedailystar.net/magazine(accessed 24.06.2003) 09. International Association of Oral and Maxillofacial Surgeons: History of IAOMS: 1992- 2001.Available at :http//www.iaoms.org/history-1992-2001. Aspx. Accessed January 9, 2009 10. International Association of Oral and Maxillofacial Surgeons: available at :http/wwww. iaoms.org/IAOMSDIRECT/images/membership/membership_top.jpg. Accessed January 9. 2009 Partners : Asmat Ali Khan Research Foundation, Mr. Shahjahan Khan MP, Minister Shipping Govt.of Bangladesh and Dr. M.R Khan MOH Govt. of Bangladesh support with hospital facilities. Help a Child face tomorrow, Kenya Friends of Grace International , U.S.A

98 10th World Cleft Lip, Palate & Craniofacial Congress

International Foundation for Children with Cranial-Facial Disorders

John Westine 250 Dixie Boulevard. Suite 100.Delray Beach, FL.33444.USA

What is the IFCCD? It is a charitable corporation formed to enable needy children with cranial facial disorders to have free care. The foundation might provide money to pay for a hospital operating room and recovering room. It may buy instruments, it may enlarge clinics, or it may pay travel expenses, room and board for surgeons who donate their time. After surgery to correct his/her deformity we provided follow up care whether it’s orthodontics or other types of dentistry, psychological or even post- op financial support for the families transportation and medical costs. The Foundation is primarily a children’s foundation and not an educational foundation for training surgeons. Often Surgeons like to be itinerant and gain experience at the expense of these children. Then they leave the follow up care to others. A surgeon’s educational training is not the purpose of this foundation. It is strictly to help children with deformities around the world. We have developed the first cranial facial center in Peru. The Foundation has helped children in Nepal, Africa, Mexico, and Peru and is always looking for catchment areas where needy children can be found. Anyone listening to this lecture may be able to help locate these children. Thus far, we have not Ventured into the Middle East because of the danger however we might still look into this area to see if there are child victims of the war who can use our expertise. The spinoff of this foundation are numerous. Hospital’s that have anesthesia residency programs enjoyed the benefits of treating large numbers of children to improve their techniques. The IFCCD helps develop centers of excellence within hospitals. Concentrating care gives opportunities for research and publications as well.

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Those in attendance who are interested in this project can help can help by telling me where they believe the foundation can help and whether or not they are willing to lend their expertise to the project.

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Celebrating the Past, Looking to the Future: New Educational Projects of the EACMFS

Manlio Galiè EACMFS Education & Training Officer, Unit of Cranio Maxillo Facial Surgery, Center for Orbital Pathology & Surgery, ( Director and Chief: Prof. Luigi C. Clauser ), St. Anna Hospital and University, FERRARA – Italy.

ABSTRACT

The evolution of OMF and CMF surgery has been dramatic and the Specialty today takes the lead among colleagues of related disciplines in an integrated interdisciplinary patient care system. The future of Education in Oral & Maxillo-Facial Surgery could be considered as a learning revolution and I am convinced that education and training are a source of future growth of the discipline and are a key success factor for our association, as well as a clear mandate for us.

●● As a specialty and as a profession, we have faced the changes occurring ●over● the last years in this very dynamic and ever-changing field: ●● No “school of thought” but Evidence; ●● Huge increase in number of Journals; ●● Courses; ●● Information online: global access; ●● Generational attitudes; ●● Standards of care; Ethics; Accreditation. The recent launch of the EACMFS website has represented a significant step forward both as regards communication with our members and a more modern education and training system. The key strategy of the EACMFS is based on a strong support to the countries demanding cooperation and help to defend the prerogatives of our discipline, well aware that spending on education is an investment, but not a cost. 101 10th World Cleft Lip, Palate & Craniofacial Congress

Management of cleft lip and palate ; are we delivering the best?

Parakrama Wijekoon Consultant OMF surgeon, Founder President Sri Lanka Association of Cleft Lip ,Palate &Craniofacial Anomalies Past president (SLOMFS), Director, Smile Train Project, Department of Oral and Maxillofacial Surgery,Faculty of Dental Sciences, University of Peradeniya, Peradeniya, Sri Lanka

The man

agement of cleft lip and palate showed a tremendous development in the last two decades During early days, it was a performance of a single surgeon .Cleft lip and palate is the commonest congenital abnormality in the head and neck region. Globally, cleft lip and/or palate occurs about 1 in every 1000 live births on average. In Sri Lanka, the incidence rate of cleft lip and/or palate is about 418 births per year (population: 20million), which compares with the global and local region rates (e.g., USA – 6169/293 million; UK – 1266/60 million; India - 23,375/1,065 million; Bangladesh – 2969/144million; Pakistan – 3344/159 million). When a child is born with cleft lip, palate or other craniofacial anomalies, the challenges faced by the child and the caregivers need continued support and guidance by a team of professionals in many different areas of expertise. Pediatricians, feeding specialists/nutritionists, oral and maxillofacial surgeons, plastic surgeons, anesthetists, orthodontists, ENT surgeons/specialists, speech and language therapists, genetics specialist/genetic counselors, psychiatrists/ psychologists, audiologists, social workers and many others are, or should be involved in providing specialized and much needed care for this patient population. The care for this population does not stop with successful surgery; in fact, their journey to achieve typical development in all aspects begins at that potentialpoint. The multidisciplinary team involved must make sure that each child is provided with the best of care and opportunities so that he reaches his highest

Multidisciplinary management of cleft lip and palate is a well-established practice world-wide which but its applicability varies from country to country. Factors such as infrastructure, availability of team members and many other factors govern this. Different aspects of multidisciplinary management of cleft lip and palate,its advantages ,disadvantages and its applicability to our countries will be discussed in detail in the presentation. 102 10th World Cleft Lip, Palate & Craniofacial Congress

Three decades of excellence in Cleft Orthodontics for complex Secondary cleft deformities

OP Kharbanda Professor and Head, Division of Orthodontics and Dentofacial Deformities, ALL India Institute of Medical Sciences New Delhi, India

In India, about 40,000 new cleft patients are born every year and only a few of them receive optimum coordinated interdisciplinary care. A large number of patients get suboptimum surgical /dental /speech care leading to severe and complex malocclusion. AIIMS being tertiary care hospital treats patients with secondary cleft deformities referred from several states of the country. The thechallenge family. in management lies in dealing with the complexity of the malocclusion and the multitude of other problems including limited resources available with

AIIMS has initiated orthodontics cleft clinic in 1972 and author joined this institute in 1985. Since last three decades, we have learnt, tested and instituted orthodontics with excellent outcome with self initiate protocols to fix the complex malocclusion and needs of the patient with least burden of care. Use of pre calibrated NiTI expanders, early chin cup therapy, fixed appliance only after eruption of permanent dentition, use of long term rigid metal retainers, and three in one prosthesis to serve as obtutaror, retainer and denture has optimised treatment outcome with least burden of care. This presentation will deal with issues in cleft care in developing countries and show case AIIMS protocols on management of complex malocclusion with a variety of representative cases.

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Cleft Care Orthodontics

A. ARIF YEZDANI Professor and Head, Department of Orthodontics and Dentofacial Orthopedics, Bharath University, Sree Balaji Dental College and Hospital, Narayanapuram, Pallikaranai, Chennai, India

Abstract

Clefts of lip and palate are one of the most common craniofacial anomalies with a multifactorial inheritance with genetic and environmental factors responsible for its occurrence with a 70% incidence of nonsyndromic cleft lip with or without cleft palate and a 30% syndromic cleft lip with or without cleft palate. The incidence of cleft lip and palate in India is 1:500 live births with unilateral cleft lips predominant over bilateral cleft lips with left sided unilateral clefts more common than right sided ones. In females isolated cleft palate is more common as compared to males in whom the incidence of cleft lip is higher. Unoperated cleft lip/palate subjects exhibit normal facial growth whereas surgery dependent on its timing could produce midfacial growth problems. Nevertheless, esthetics, psychosocial reasons and speech mandates a multidisciplinary treatment approach for management of such patients. This presentation would deal with the role of orthodontist in the treatment of cleft patients. The orthodontist is one of the first few specialists to meet the parents and infant. Informed reassurance, parental counseling, dietary counseling, and fluoride prescription is the first step towards management of cleft cases. In bilateral clefts, premaxillary segments must be retruded. Presurgical infant orthopedics carried out at 3-6 weeks makes use of intraoral appliances to separate the collapsed lateral maxillary segments which alongwith an elastic strapping across prolabium restrains premaxillary growth. Acrylic obturators can be used for feeding difficulties. Presurgical nasoalveolar molding, a nonsurgical technique, is effective in infants to reduce the size of intra-alveolar cleft by molding of bony segments, surrounding deformed soft tissues and cartilage in the cleft nose. The orthodontist has to effectively manage the anteroposterior, transverse and vertical dimensions of the maxillary and mandibular dental arches in the mixed and permanent dentition. Skeletal Class III malocclusions with retrognathic maxilla can be treated with protraction headgear104 therapy around 6-8 years of age. W-arch, quadhelix, nickel titanium 10th World Cleft Lip, Palate & Craniofacial Congress

palatal expanders or other types of expansion appliances at 9-10 years of age are used to correct anterior and posterior crossbites. A secondary bone graft (8-11years) is performed prior to cleft canine eruption and active orthodontic treatment is started after eruption of canine. Comprehensive orthodontic treatment with preadjusted edgewise appliances can be used around 12-13 years of age for orthodontic traction of impacted teeth, precise positioning of teeth and arch coordination. Chin cup headgear therapy can be used to restrain//redirect mandibular growth. After completion of growth period, presurgical and postsurgical orthodontics may be deemed necessary for the cleft patients who warrant orthognathic surgery.

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Specifics of Orthognathic surgery patients with cleft lip and palate

O.Z. Topolnickij, R.N. Fedotov, S.A. Nikolaeva Head of the Department of Pediatric Maxilofacial and Dental Surgery, Moscow State Medicine and Dentistry University, Russia

Applicability

According to International and Russian medical researches the frequency of cleft pathology is 1-1,5 in 1000 newborn children. It is an important aspect to all the patients with cleft pathology to undergo subsequent treatment in special medical centers for complex rehabilitation, with all required medical professionls, but still according to international and our institute’s researches - from 10 to 30 percent of cleft patients need supplementaryAim orthognatic surgery. and palate. Providing better quality orthognathic surgery of patients with cleft lip Materials and Methods

120 patients with full cleft lip, alveolar bone and palate received treatment and orthognathic surgery, 58% percent have a unilateral cleft and 42% percent have a bilateral cleft. The patients were checked-up thoroughly. All the patients have received a pre-surgical orthodontic treatment. 32% underwent preliminary repeated bone grafting of alveolar bone defect. Depending on the character of the deformation and bone tissue defect the orthognatic surgery was performed for upper jaw, lower jaw and bimaxillary. Upper jaw osteotomy was performed among 25% of patients, lower jaw osteotomy was performed among 5% patients and bimaxillary osteotomy was performed among 70 % patients.

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anterior wall defect of maxilla and alveolar bone was performed for 18 patients. . Among 18 patients upper jaw osteotomy were performed using an immediate bone transplantatgrafting of anterior inability. wall defect of maxilla and alveolar bone. 6 patients received the bone grafting twice, as the first time bone grafting failed due to

12 patients received the immediate bone grafting for the first time, that allowed to exclude the proximate bone grafting of alveolar bone defect during the pre-surgical treatment and to establish additional stabilization for theupper future. jaw complex during and after the surgery, to create good conditions for implementation secondary rhino- and cheiloplastic and rational prosthetic in

The main criteria of completeness of complex rehabilitation of patients with cleft lip and palate is social adequate speech, which quality is much depended of full palatopharyngeal closing. The check-up of speech therapy status and palatopharyngeal closing were completed before the orthognatic surgery and 6 months after. 68 nasopharyngoscope and speech therapy checkups of the function of palatopharyngeal closing were completed (34 before and 34 after surgery). The following speech malformations common for patients with cleft lip and palate combined with mesial occlusion were defined - rhinolalia, residual effects of rhinolalia and rhinophonia. The results of speech quality of all patients were divided as excellent, good, satisfactory and non-satisfactory. The stage of palatopharyngeal closing were appraised as: ratio – defect of palatopharyngeal closing is 0-20%, I degree – 21-40%, II degree – more then 41%. On 100% the convention surfaces of palatopharyngealResults closing in inertness was taken.

1. The stable result of occlusion was achieved for 81% cases. The relapse occurred for 19 percent patients. Correlation between relapse and the following orthodontic treatment is (p<0,05). 2. According the following checkups in post-surgery period no patients received retrogression of speech quality. The speech quality of 6% of patients improved after the surgery and speech therapy correction classes. The results of nasopharyngoscope showed that the condition of palatopharyngeal and it’s function didn’t change for 56 % of patients during more then 6 months after orthognatic surgery. 44 % has improvements.107 10th World Cleft Lip, Palate & Craniofacial Congress

No retrogression of palatopharyngeal closing is shown. The percent of improvement is proved statistically (p<0, 05). 3. The orthognatic surgery creates stable anatomical and functional results which leads to better social adaptation and less disability.

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Distraction of Cleft Maxilla

Krishna Nayak US Principal & Dean, A.B.Shetty Memorial Institute of Dental Sciences, Nayak's Dental Speciality Clinic & Orthodontics Centre, Mangalore, India

Cleft lip and palate are common congenital deformities in the oromaxillary area. This birth defect causes several functional and aesthetic problems because of defects in the soft and hard tissues leading to nose deviation and formation of scar tissue after surgery. Distraction osteogenesis (DO) was first used for correction of the craniofacial skeleton in the early 1990s. Figuero and Polley reported success with no significant complications when DO was used to advance the maxilla in children with cleft lip and palate. Recently, DO was used to advance the maxilla in patients with cleft palate; however, little has been reported on the use of DO for maxillary expansion and closure of the cleft alveolus. This presentation review distraction in cleft palate with case reports.

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Management of Maxillary cleft deficiency - Long term results

Adi Rachmiel, Dekel Shilo, Dror Aizenbud, Omri Emodi Department of Oral and Maxillofacial Surgery, Rambam Medical Center, Haifa, Israel and Faculty of Medicine, Technion-Israel Technion of Technology, Haifa, Israel

Abstract Aim

Correction of the hypoplastic maxilla secondary to cleft patients is a great challenge due to a significant vertical and horizontal deficiency and difficulty in mobilizing the hypoplastic maxilla as a result of scarring from previous operations. In addition there is a great tendency for relapse following the major movements which are required. Moderate or severe maxillary deficiency can be better corrected using distraction osteogenesis due to the major possible movements in anterior and downward directions. In this study we will preset our experience using internal andMaterial external and devices methods in maxillary cleft deficiency.

Maxillary distraction was performed on 56 cleft patients, 21 patients with rigid extraoral devices (RED) and 35 patients with internal devices (IDD). The rate of distraction was 1mm per day until achieving a Class I occlusion yearswith partial will be overcorrection presented. and creating facial profile convexity. Following the latency period, distraction devices were removed. Long term follow up of 5 Results

The RED system offers greater distraction length, offers a control on the vector of lengthening and is easily removed. However, it is uncomfortable parietalfor the patient bone penetration. when wearing for long periods of several months, the device is exposed to external trauma forces during that period and there is a risk of

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Internal distraction devices (IDD) are very efficient in advancement of the deficient maxilla and are safer to wear for long periods of several months, they do not create social discomfort and therefore permit longer retention periods which may contribute to better stability than external devices. However their major disadvantage is that they require a second operation under general anesthesiaSummary for device removal.

In conclusion, The hypoplastic maxilla in cleft patients is associated with moderate to severe retrusion, and is better treated by distraction osteogenesis then by conventional orthognathic surgery. The internal devices should be considered first even when taking into account the major disadvantage of a second operation for device removal. Based on research and clinical experience, distraction osteogenesis has minimal tendency to relapse even following great skeletal movements, due to the new bone formed in the distraction gap.

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Current Issues in Cleft related orthognathic surgery

Jong Woo Choi Department of Plastic and Reconstructive Surgery, Ulsan University College of Medicine, Seoul Asan Medical Center, Seoul, Korea

As the maxillary hypoplasia exists in cleft related dentofacial deformities, the midface retrusion has been the main target in planning. Therefore, the maxillary advancement with or without the mandibular setback procedure was the standard treatment objective. However, I found out the maxillary vertical shortening would be another major problem as well as the maxillary retrusion in cleft related dentofacial deformities. Although the deficient incisor show due to the deficient maxillary vertical growth as well as the horizontal growth exists, many surgeons have mostly focused on the maxillary advancement in order to overcome this problem using the orthognathic surgery or maxillary distraction. The maxillary vertical lengthening is known to be the least stable procedure among the various maxillary movement planning. So this is not regarded as the recommendable procedure. If we underwent the maxillary vertical lengthening, the bone contact surface between the maxillary segments would be the least in spite of the bone graft. But, as the deficiency of the maxillary vertical growth in the cleft related dentofacial deformities exist, I thought I should overcome this obstacle anyway. In addition, in terms of the harmonious lips relationship, cleft-related jaw-deformity patients often do not receive the same cosmetic benefits as non-cleft class III patients after the orthognathic surgery. Several reasons could be suggested. First is the possibility of not enough amount of maxillary advancement. Second is the upper lip tightness related cleft lip repair. Third is the possibility of the existence of lower lip deformity itself in cleft patients. Although the cleft lip is mostly known to be related to the growth and function of the upper lip, I believe the lower lip itself could be deformed via the impairment of the orbicularis oris muscle sling. Aim of this study was to know whether the lower lip deformity itself may exist based on the comparison of the outcomes of orthognathic surgery between cleft and non-cleft class III malocclusion patients focusing112 on the lip relationship. 10th World Cleft Lip, Palate & Craniofacial Congress

Finally, despite an ongoing debate regarding premaxillary osteotomy, it has been recently recognized as a probable solution for severe forms of bilateral cleft lip and palate. Accordingly, we hypothesized that premaxillary repositioning into the correct position would help to restore the maxillary arch coordination in severe forms. In our present study, we investigated the effect of premaxillary repositioning on the restoration of maxillary arch coordination in three dimensions, as well as its effect on midfacial growth. There are few reports of the orthognathic surgery based on the maxillary vertical lengthening for the correction of the cleft related dentofacial deformities, premaxillary repositioning and lower lip deformity in cleft dentofacial deformities. This presentation will discuss the various current issues including the importance of the maxillary vertical lengthening accompanied by the maxillary horizontal advancement, premaxillary repositioning and the lower lip deformity when we plan the cleft related two jaw surgery.

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The Key Points for the Good Final Result of Cleft Lip and Palate Patient

Lian MA Cleft lip and palate treatment center, Department of Oral and Maxillofacial Surgery, School of Stomatology and Dental Hospital, Peking University, Beijing, China

The final results for cleft lip and palate patient should be good appearance, good speech, and good growth (maxilla).For approaching the aims, cleft lip and palate patients are needed to be treated from born to adults. Even then not everyone can achieve the ideal result. Several points are indicated in this talk for how to gain good final results for cleft lip and palate patients according to the01. author’sDoctor: over 30years experience. including the insistence of doctor, principle of treatment plan, and based evidence. Most important point which should always be in mind is 02. notPatients: to try new method until the evidence of long term result is gained. including the insistence of patients, therapeutic compliance and demands. Keeping change doctor and setting treatment plan by patients 03. themSocieties: shelf based on internet website is main factor for failure. including foundation support, society acceptance and discrimination against. The Vast numbers of case samples are demonstrated in this talk and the schedule for treatment cleft lip and palate special points in PKU are indicated and discussed.

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Diagnosis, treatment and outcome of patients with submucous cleft palate- a retrospective data analysis

1A. Hemprich, 2F. Schiefke Head, Department of Maxillofacial and Facial Plastic Surgery of the University of Leipzig, Germany

Introduction

The submucous cleft palate is often diagnosed very late. It frequently causes velopharyngeal insufficiency, which can induce speech abnormalities, as well as conductive hearing loss. The aim of this study was the systematic evaluation of our patients with submucous cleft palate of the past 20 years, as wellMaterial as a thematicand methods literature review.

In a retrospective study the medical records of 80 patients (11 syndromic, 69 non syndromic), who were referred to our department due to a suspicion of a submucous cleft palate in the period from 1995-2015, were evaluated. 54 of them had a surgery-requiring submucous cleft palate. In 26 cases only a non-functionally relevant bifid uvula was observed. 38 patients underwent a primary palatoplasty with complete reconstruction of the cranial and caudal velar muscle rings. Palatoplasty with simultaneous velopharyngoplasty took place in 11 children. The cleft palate closure was performed in the sense of a v. Langenbeck procedure. A hearing assessment was carried out pre-and postoperatively. Moreover, the influence of the size of the cleft and the operative therapy on the speech wereResults evaluated.

The median age of children with submucous cleft at initial presentation was 46.5 months. 39.9% of the patients had a history of already one or multiple ENT-interventions without previous diagnosis of the cleft. Velopharyngeal115 10th World Cleft Lip, Palate & Craniofacial Congress insufficiency was the leading clinical symptom in 63.9% of children. 51.9% had a chronic sound conduction defect. In 40.7% of cases a speech therapy was thealready patients. done. The clinical investigation revealed a bifid uvula in 79.6%, a bifid posterior nasal spine in 77.8% and a muscle gap (zona pellucida) in 81.2% of

The majority of all cases showed speech abnormalities (81.4%, hypernasality, 100% nasal emission and 69.2% a centripetal shift. The primary palatoplasty respectively palatoplasty with velopharyngoplasty took place in the median age of 42 respectively 75 months. In 54% of children a simultaneous paracentesis and insertion of tympanostomy tubes was carried out. 12 patients had post-surgical wound healing disorders. In two patients (5.4%) a reoperation due to an oronasal fistula was necessary. A functionally not relevant oronasal fistula persisted in three cases (8.1%). In two patients, a surgical haemostasis had to be carried out. After palatal reconstruction an improvement of speech could be shown. It proved to be especially in a decrease of the extent of speech pathology. In six children (15.8%) noticeable speech despite speech therapy persists after palatoplasty,Summary so that the indication for a velopharyngoplasty was made.

The submucous cleft palate is a common congenital malformation of the palate, which can lead to a velopharyngeal insufficiency and tube ventilation failure. The present study demonstrates that a surgical correction of cleft initiatecontributes an appropriate to improving diagnosis speech and and treatment. hearing of the affected children. Thus patients with these symptoms should be presented early in a cleft centre to Literature 01. Pensler JM, Bauer BS: Levator repositioning and palatal lengthening for submucous clefts. Plast Reconstr Surg. 1988; 82:765-769. 02. Stewart JM, OTT ME, Lagace R: Submucous cleft palate. Birth Defects Orig Artic Ser. 1971; 7:64-66. 03. Sullivan SR, Vasudavan S, Marrinan EM, Mulliken JB: Submucous cleft palate and velopharyngeal insufficiency: comparison of speech outcomes using three operative techniques by one surgeon. Cleft Palate Craniofac J. 2011; 48:651-670. 04. Scheuerle J, Habal MB: Discussion of Isotalo et al.: speech in 6-year-old children with submucous cleft palate. J Craniofac Surg. 2007; 19:723-724.

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Surgical Treatment of children with cleft lip and palate in newborn period

Mammadov Ad.A., A.B. MacLennan, Zh. A. Blinder, L.A. Mazurina Department of Pediatric Dentistry and Orthodontics I.M. Sechenov Medical University First Moscow Medical State University Russian Ministry of Health, Moscow, Russia

mostly dependent on the timing. Diagnosis and planning and the success of surgical treatment in medicine

However, in the cleft surgery there is no consensus on rational terms of surgical care to children with CLP. Operation time is almost always determined by the maxillofacial surgeon based on his experience, anesthetic, the possibility of intra and post-operative complications, somatic status, presence and asseverity a whole. of comorbidities, as well as psycho-emotional impact of the presence of maxillofacial region pathology in the relationship of parents and the family

The study included 30 children aged between 0 and 29 days with non- syndromic unilateral cleft lip, alveolar process and palate before surgery, and 45 children aged 4 to 6 months with same pathology. For both groups, the study developed a method of prenatal diagnosis and counseling of parents, as well as a survey of parents to assess the results of prenatal diagnosis. In the postnatal period in children with unilateral cleft lip and palate surgery performed by modified surgical method of cheoloplasty in the neonatal period.

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Re-thinking Bilateral Clefts

S. Anthony Wolfe Chief, Division of Plastic Surgery, Miami Children’s Health System, 3100 S.W. 62nd Avenue, Miami, Florida, USA

Ralph Millard developed the rotation-advancement procedure for unilateral cleft lip repair, which along with primary nasal correction (we use the McComb method)’ is used around the world by the majority of cleft surgeons with gratifying and reproducible results. Millard and others (Manchester, Mulliken) however treated bilateral clefts, both complete and incomplete with a one-stage repair which trimmed and elevated the prolabium, and brought the lateral muscles to the midline. The nose---columella and lobule---remained a problem, dealt with forked flaps, sewing alar cartilages together, excising Kilner triangles, depending on the author. Lips ended up being tight, short, with rectangular prolabia and indistinct white rolls. Incidence of maxillary growth retardation was high, as evidenced by Le Fort 1 osteotomies in 50 to 75 % of patients. For the past 15 years, we have been treating all bilateral clefts, complete and incomplete, with precisely the operation we use for unilateral clefts---the rotation advancement with McComb nasal correction, following NAM, and closure of alveolar clefts and anterior palate. This is done in a staged approach, three months apart, for obvious circulatory concerns. The resultant lips are full and projecting beyond the lower lip, and longer, with preliminary mixed dentition evaluation showing good maxillary growth (see presentation of Dr. Martha Mejia elsewhere in program). The noses are normal. The remaining palatal cleft is closed at 18 months, giving an entirely intact palate due to the prior closure of the anterior palate. Alveolar bone grafting at 5 or 6 is extremely easy since there are no fistulae to close. Since bilateral clefts are nothing more than two unilateral clefts, with the sole exception that there is no muscle in the prolabium in the complete clefts-- --why not close them with the same procedure that gives such excellent results in the unilaterals? 118 10th World Cleft Lip, Palate & Craniofacial Congress

Operation Smile Comprehensive Care approach: scientific and humanitarian vision

Domenico Scopelliti D., Amodeo G. U.O.C. Maxillo-Facial Department, San Filippo Neri Hospital – ASL Roma 1 -Rome Operation Smile Italy Foundation – Smile House Milan, Via Panama 52, 00198 Rome – Italy, Via Giuseppe Martinotti 20, 00135, Rome

Abstract

Operation Smile Italy Onlus is a foundation created in 2000, made up of volunteer doctors, nurses and health workers, participating in humanitarian medical missions, to correct with reconstructive plastic surgery severe facial deformities such as cleft lip and cleft palate, outcomes of burns and trauma. KeywordsThe organization was founded in 1982 in USA and in 2000 in Italy.

Operation Smile; scientific approach; humanitarian approach; training andIntroduction education

Operation Smile was founded in 1982 when Dr. William (Bill) P. Magee, Jr., a plastic surgeon, and his wife, Kathleen (Kathy), a nurse and clinical social worker, traveled to the Philippines with a group of medical volunteers to repair children's cleft lips and cleft palates. What they found was overwhelming. Approximately 300 families arrived hoping their children would receive surgery, but the team could only treat 40 children. As they decided to leave, the Magees promised they would return to help more children. This represented the beginning of Operation Smile. Operation Smile is an international medical charity that has provided hundreds of thousands of free surgeries for children and young adults in developing countries who are born with cleft lip, cleft palate or other facial deformities. It is one of the oldest and largest volunteer-based organization dedicated to improving the health and lives of children worldwide through119 10th World Cleft Lip, Palate & Craniofacial Congress access to surgical care. Operation Smile helps to fill the gap in providing access to safe, well-timed surgeries by partnering with hospitals, governments and ministries of health, training local medical personnel, and donating much- needed supplies and equipment to surgical sites around the world. Founded and based in Virginia, U.S., Operation Smile has extended its global reach to more than 60 countries through its network of credentialed surgeons, pediatricians, doctors, nurses, and student volunteers. Since 1982, Operation Smile has developed expertise in mobilizing volunteer medical teams to conduct surgical missions in resource-poor environments while adhering to the highest standards of care and safety. Operation Smile’s Global Standards of Care were initially developed and agreed on by the global medical leadership in 2006. Subsequent meetings have been held to review and refine these standards, which establish minimum and absolute requirements for any Operation Smile surgical program. These standards are supported by medical policies and procedures which are developed, reviewed, refined and approved by the Operation Smile Medical Oversight team and which are referenced in each standard as appropriate. Underlying all standards and policies is the Operation Smile Charter of Patient Rights and Responsibilities. The 2014 Operation Smile Global Standards of Care below reflect the work at the Global Standards Summit convened in May 2014, and review processes that preceded and followed that meeting. The current structure of each standard has evolved to include every aspects of the humanitarian action: the preoperative patient screening and assessment; anaesthesia equipment and supplies; surgical equipment; post anaesthesia care unit; postoperative intensive care; postoperative ward; patient consent; surgical priority; preventing transmission of blood borne pathogens; pain management; operation smile team; qualifications for volunteers; patient followup; translation; documentation; hospital facility; quality assurance. This is fundamental to provide care to child respecting quality and safety. The patient hasn’t to become an instrument to learn medical practice. To effort this aspect the sanitary operators have to demonstrate that they are able to do what is necessary. One of the most important aspect among the foundation activities is to create local independence through training local team not only helping people through surgical procedure. To build a long-term sufficiency, the organization trains doctors and local medical professionals, in our partner countries, to make them able to treat, by themselves, their local communities. In this vision the foundation donates medical equipment and supplies and provides year-round 120 medical treatments through worldwide centers. 10th World Cleft Lip, Palate & Craniofacial Congress

The program development allows the creation of several programs aimed to help children with malformations. During the several years of activity many initiative were born to support people all around the world. After 15 years of activity the organization start help people victims of war or natural disasters. This is the reason why in 2004 it stroke a deal with the Ministry of Foreign Affairs to support the Iraqi people that brought the devastating outcomes of the Gulf War. Thus the "Smiles initiative in Iraq" was born to provide the opportunity for children and young people affected by outcomes of burns, traumas or malformations of the face, to come to Italy. This program gave the possibility to Iraqis doctors to accompany the patients to have the opportunity to participate to training courses (Fig.1).

In 2010 the organization arrived to Haiti to help people after the terrible heartquake (Fig 2). In Friday, May 24, 2013 on the Cavour aircraft carrier moored in Taranto, began "A Sea Of Smiles" project, which merges the Navy doctors and volunteers of the Operation Smile Italy Onlus Foundation. The common goal was helping palatechildren and and fromadults head suffering and from cleft lip and/or cleft 121 10th World Cleft Lip, Palate & Craniofacial Congress

hospitality to young patients and their families, as well as to adults who may neck malformations or their outcomes (Fig.4). The Cavour aircraft carrier gives be submitted to surgical treatment. The aircraft carrier represented the core of the project with its operating theater and the medical staff that cooperated with the Foundation's volunteer doctors. The "A Sea Of Smile" project was born after the positive experience of February 2010, when Operation Smile and the Navy collaborated on board the aircraft carrier Cavour, as part of a mission authorized by the Ministry of Defense and under the coordination of the Civil Protection. During this period 35 surgeries were performed on children and adults affected by serious facial trauma, resulting from the earthquake that struck the country on January of the same year. "A Sea of Smiles", the humanitarian project in collaboration with the Italian Navy, landed in Africa, always on board the Cavour aircraft carrier to help children and adults with cleft lip and palate and facial deformities. The volunteer doctors and health professionals of Operation Smile gave free health care to children in Kenya, Madagascar, Mozambique, South Africa, Ghana, Morocco and Algeria, as part of the Navy's campaign "Country moving system". The surgical procedures were carried out by Italian surgeons in collaboration with American colleagues and colleagues of Operation Smile Foundation in Africa (Fig. 5).

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The foundation program is too wide. It tries to cover each aspect of actions: medical programs through International and local medical missions, creation of care centers, research programs, training and education and advocacy. Not every country or community around the world is the same. That's why the foundation works with local medical professionals, governments, hospitals and other NGOs to create various models of surgical care. Together, it is possible to determine which solutions work best in order to reach as many children as possible wherever the foundation works. Sometimes patients required a relatively simple surgical procedure. Sometimes care is more complex. Operation Smile recognizes that the need for care doesn’t stop when a medical mission ends. So wherever possible, the foundations established centers to ensure a continuative care for patients who require comprehensive or more complex treatment than a single medical mission can provide. This is the reason why in Italy during the last decade, following the Memorandum of Understanding signed by the Foundation Operation Smile Italy Onlus and the San Paolo Hospital, Regional Reference Center for cleft lip directed by Prof. Roberto Brusati, the Smile House was born in 2011 in Milan (Fig.3). It represents the first Operation Smile multispecialty center in Europe for the treatment of congenital maxillofacial malformations. The Smile House offers, in a single structure, a multispecialized team able to follow the patient throughout the diagnostic and therapeutic process. The Smile House is a treatment center and a research and training center pointed towards malformation.the medical and health personnel of the partner countries of the Organization, as well as a reference center for couples who have adopted children with a face

The exact cause of cleft remains unknown and Operation Smile is trying to find the answers. One of the most important aspects is the research program. The foundation research has to identify new ways to deliver surgical care and to exceed obstacle that divide patients to surgery. This could be possible listening the patients to really understand what prevents them from accessing care. Training and education are another fundamental foundation aspect. Them represent the heart of foundation actions. On every engagement, in fact, there is a growth, through the exchange of ideas and knowledge among our medical specialists, from different backgrounds, with varied levels of expertise. Together patients.it is possible to have the same goal: improving surgery while empowering local communities. The local people have to be sufficient in management this kind of

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The foundation is active in establishing events that have the aim of found raising. Smile champions around the world are doing creative thinks to help create new smiles for children all around the world. Volunteers’ birthday or marriage, organization of athletic or other special events are the ideas that the foundation’sConclusion member realized to provide the necessary care.

Operation Smile has been committed to increase access to surgical services for more than three decades and our patients are a testament of that. Unfortunately there are too many patients who still need help. In alignment with the World Health Organization, we believe that safe and welltimed surgery is a basic service that should be accessible to all families and it’s our responsibility to advocate for their right to surgical services.

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Alveolar Cleft Repair with Demineralized Dentin Graft Material

Masaharu MITSUGI Japan Auto-Tooth Bone Bank, Secretary General, Div. OMS, Kochi Health Sciences Center, Japan

Secondary autogenous iliac cancellous bone grafting at the age of 8~11 years during the transitional dentition phase is a widely used method for alveolar clefts repair. However, it needs extensive surgeries, and failure of iliac bone grafting sometimes occurs due to inadequate covering with the surrounding soft tissue and marked scar formation, or large clefts. Auto-Tooth Bone is bio-recycled extracted teeth as Bone Graft Materials developed in Korea and Japan. It is a good grafting material without harvesting donor site and it promotes rapid new bone remodeling and it does not induce immune rejection or postoperative infection. It has not only osteoconduction property but also osteoinduction property which other synthetic grafting materials don't have. Auto-Tooth Bone is incomplete demineralized dentine matrix including Type - Collagen, BMPs, Hydroxyl Apatite that is an essential component used during bone remodeling. The frequency of wound rupture occurred in the cases using Auto-Tooth Bone were the same as that in other cases using other synthetic grafting materials, however postoperative infection and complete loss of Auto- Tooth Bone materials grafted has never been reported. We used Auto-Tooth Bone for the reconstruction of alveolar bone defects without harvest surgery and infection risk. We think Auto-Tooth Bone will be a successful choice of 2nd grafting material in cleft patient. I will show the successful result of these procedures.

125 10th World Cleft Lip, Palate & Craniofacial Congress

Secondary Alveolar Grafting with Tibial Graft – A simple technique

Sadique Hussain M Principal, N.I. College of Dental Science, Thrissur, Kerala, India

Bone graft usually harvested from illiac bone, which is rich source of corticocancellous bone for secondary alveolar grafting also, Corticocancellous graft is taken from illiac bone. However this makes the post operative stay in the hospital for a longer time and other complications like pain for a longer duration and discomfort for the normal gait. So harvesting bone graft from medial condyl of tibia through a simple technique makes revolving solution for the●● above said complications like, ●● Procedure is simple ●● Patient can walk from the very next post operative day No post operative discomfort.

126 10th World Cleft Lip, Palate & Craniofacial Congress

ALVEOLAR CLEFT GRAFTS

Mzubanzi Mabongo Head of Clinical Unit, Department of Maxillo-Facial and Oral Surgery, School of Oral Health Sciences, Faculty of Health Sciences, University of Witwatersrand, South Africa

Introduction

Cleft lip and palate is the common craniofacial anomaly with prevalence of 1 in 5000 to 1 in 25000 of live births. The incidence differs depending on the population group and geographical area. Embryological basis for cleft lip and palate is the of failure of palatal shelves to fuse or failure of fusion between primary and secondary palate or both depending on the extent of the cleft. Hereditary and environmental factors are believed to be the main contributing actors in the development of cleft lip and / or palate. Environmental factors include several prescription drugs (anticonvulsants, corticosteroids and retinoids), maternal smoking and consumption of large volumes of alcohol. Metabolic imbalances like folic acid deficiency are also associated with facial clefts1 (Watkins et al 2014). While MSX1 has been implicated as a cause in patients with cleft lip and/or palate. Candidate genes and loci have been identifiedAlveolar Cleft in chromosome Grafts 1,2,3,4,6,11,14,17 and 19 (Blanton et al 2004).

An Alveolar cleft refers to a space between the maxillary segments anterior to the incisive3 foramen, and therefore presents with absence of dental arch continuity . Alveolar cleft may occur as part of complete cleft lip and palate or as an isolated cleft. It is the last part of the complete cleft to be repaired. Timingafter repair for repair of the3 lipof alveolarbut before cleft the is repair generally of the classified palate into primary repair and secondary repair . Primary repair is done within the first4,5 24 months after birth, . Skoog et al reported the first boneless6 graft in 1965, which was later referred to as gingivoperiostoplasty (GPP) . The main goals for primary repair are to improve arch form, preserve lateral incisors and stabilise the premaxilla 7in cases with bilateral clefts and. the need for orthognathic surgery is reduced . While the disadvantages of GPP7 are restriction of maxillary growth due to the scars and periosteal stripping Some clinicians have reported high incidence of malocclusion, inadequate bone127 10th World Cleft Lip, Palate & Craniofacial Congress formation and need for additional bone-graft procedure later in patient’s life3. While others reported a 72% success rate with GPP7,8. Criteria for success of GPP is the presence of a bony bridge in the previous cleft, more than 75% bony height and less than 25% residual bony defect on occlusal radiographs7. Secondary alveolar cleft grafting was first introduced by Boyne and Sands in1972, and it is. a widely accepted procedure as standard care for patients with cleft lip and palate. For success of this procedure, a collaborative planning between two members of the craniofacial team, a surgeon and an orthodontist is mandatory. Secondary alveolar cleft repair is further classified into early secondary repair at 2 to 5 years, early mixed dentition at 6 -8 years and late mixed dentition at 9-12 years and late secondary at 13 years and above3,4. The major problem with early repair of alveolar graft is the disturbance of growth of the maxilla7. The decision for alveolar bone grafting should depend on balancing the need for functional and aesthetic outcomes against the potential for restriction growth and development of the maxilla4,9. Since maxillary growth is almost complete at the age of 8 years, secondary alveolar cleft repair is usually performed at the age of 9 years and above. While Some clinicians recommend alveolar grafting to be done during eruption of central incisors, during early mixed dentition, to improve periodontal support of lateral incisors10. The timing of surgery is dependent on the patient’s dental development of the patient, not on the chronological age4. The most important factor for success of alveolar bone grafting is the eruption stage at the time of grafting and the position of the canine. Optimal timing for alveolar cleft grafting is when the root of the canine of the cleft side one third to half formed11. Goals of alveolar cleft repair are to establish continuity of the maxillary segments, to provide bone for eruption of lateral incisors and or canines, to facilitate orthodontic and prosthodontic treatment. Alveolar cleft grafts also provide support for the alar of the nose, facilitates adequate oral hygiene and dental and periodontal health and helps in closure of oro-nasal fistulae3.8,12. Although this procedure is popular and the success rate is high, like any surgical procedures, complications and failures do occur. Factors that are associated with high success rate of unilateral clefts are presurgical and postsurgical orthodontics,Surgical technique and in cases where alveolar cleft is done before eruption13.

Various surgical approaches have been described by Cohen, Hall and Posnick, Precious and Cendrun11. These different incisions arise as a result on an attempt to preserve the keratinised mucosa of the gingivae. Although there is128 consensus on superiority of autogenous graft, some clinicians prefer to use 10th World Cleft Lip, Palate & Craniofacial Congress

cancellous bone4 while others prefer corticocancellous bone block9 which providesRole of orthodontist immediate mechanical stability required for healing14.

Alveolar cleft grafting is usually preceded by some form of presurgical orthopaedics. Various methods of presurgical orthopaedics have been reported in literature. Santiago et al (2014) classifies presurgical orthopaedic into two stages presurgical infant orthopaedics and maxillary arch preparation for secondary grafting3. The history of presursical infant orthopaedic date back from use of extroal devices to nasoalveolar moulding (NAM). Presurgical orthopaedic therapy is used for restoring correct skeletal, cartilage and soft tissue relationships. This provides optimal conditions for primary lip, alveolus and nose surgery3,13. Since teeth adjacent to the cleft tend deviate towards the cleft especially central incisors, orthodontic alignment of the teeth is required before secondary grafting3,13. Postsurgical orthodontic treatment is also required to move erupted teeth into the grafted bone and creating a space for a prosthesis12,13. Liao et al (2015) found that presurgical and postsurgical orthodontics was associated withGrafting superior materials secondary / Materials alveolar used cleft for graft grafting outcomes13.

Grafting materials should have the following properties, osteoinductive, osteoconductive and osteogenic. Osteoinductive refers to the ability of the graft material to recruit pluripotetial mesenchymal stem cells that differentiate into bone- forming osteoblast and chondrocytes. Osteogenic material refers to grafts material which promote primary bone formation while osteoconduction refers to the geometric scaffolding, which provides an environment for new bone apposition by supporting host capillary ingrowth, perivascular tissue, and osteoprogenator cells14. Grafting materials are classified as autogenous, allogenic, xenograft and alloplast. Allografts, Xenografts and alloplastic materials have been used in repair alveolar clefts. Recently, tissue engineering, which does not involve autologous graft, by using the use of bone morphogenic proteins Autogenous (BMP) grafts and stem cells has produced good results.

Autogenous bone is the gold standard for grafting of the alveolar clefts because of their osteoinductive, osteoconductive and osteogenic properties. The donor sites for autogenous grafts are classified as local and distant sites. The local sites include maxillary tuberosity, mandibular symphysis and retromolar area15. 129 Since bone from the local donor sites bone is intramembranous in origin, bone 10th World Cleft Lip, Palate & Craniofacial Congress

graft is expected to have minimal15,16 resorption and show rapid revascularisation than endochondral bone grafts . However, the disadvantages of these local sites are small quantity of bone and the mandibular donor sites provide mainly cancellous. Autogenous15 bone from extraoral sites can be harvested from iliac crest, calvarium, rib . The main disadvantage of these is the second procedure site and unsightly scars. Iliac crest graft is easy surgical access, large quantity of bone can be harvested and superior outcomes. The concerns about iliac crest are its effect on growth, gait disturbance, neurological fallout, heamatoma and pain4. Mandibular symphysis is a preferred donor site by some clinicians because it is the area of familiarity. Other advantages are easily accessed, single site of intraoral operation, short stay in the hospital, an invisible scar in the lower alveolar sulcus and there is minimal postoperative pain or discomfort. However, the disadvantages of mandibular are small quantity of bone and that the bone is mainly cancellous in nature. Secondly, there is high risk of damage minimalto adjacent bleeding teeth, andmental allows nerve rapid and ambulation soft tissues.. Patients are referred back to the orthodontist four weeks after surgery. Tibial14 bone is easy to access, there is

Allografts are grafts taken from the member of the same species as the host but genetically dissimilar. These include cadaver, free dried bone and decalcified bone. Allografts contain biological active proteins found17,18 in extracellular matrix of bone, are osteoconductive and osteoinductive . Possible complications associated with allografts include infections. Xenografts: grafts derived from genetically different species from the host. These are de-proteinised skeletal tissues from bovine or porcine origin, and only inorganic part of bone is used18. The commonly used xenograft is bio- Oss, hydroxyapatite Alloplasts are synthetic bone substitutes that mimic the inorganic component of bone and allow new bone with connective tissue to form. Bioceramics need to have osteoconductive that they and are biogradable brittle and properties.therefore are These not include calcium sulphate, tri-calcium. phosphates, hydroxyapatite, bioactive glasses. Shortcoming of alloplasts17 is Distractionappropriate infor cleft load-bearing management

Binger et al 2003 reported use of distraction osteogenesis before grafting. of the alveolar cleft. They recommended the use of distraction osteogenesis19 in patient with wide cleft defects where soft would be difficult to close130 10th World Cleft Lip, Palate & Craniofacial Congress Tissue Engineering

Tissue engineering (Te) has been described as an alternative method managing bone defects. TE aims to restore function to or replace damaged or diseased tissues through the application of engineering and biologic principles5. It uses three dimensional scaffolds, undifferentiated mesenchymal stem cells and osteoinductive proteins called, bone morphogenetic proteins (BMP). Scaffolds provide the solid framework for cell growth and differentiation5,20. The scaffolds should be biocompatible and be absorbable with rates comparable to rate of new bone formation5. Scaffolds should also be osteoinductive, osteoconductive, provide mechanical support and non-immunogenic20. Various sources for mesenchymal cells have been described. These include autologous graft, embryonic stem cells5. BMPs recruit mesenchymal stem cells and differentiate them into osteogenic linage5. Approaches to the use of Te in alveolar cleft grafting are enhancing autologous graft, with platelet rich plasma, barrier membranes and fibrin glue21,22. The second approach to use of Te is extending autologous graft by adding Calcium phosphate scaffolds. Lastly, Te can be used as a replacement graft by using BMP-2, mesenchymal withstem hydroxyapatite cell and scaffolds21. showed Studies better onresults outcomes than when of use autologous of tissue graft engineering is used to enhance autologous graft with membranes and extending autologous graft alone22. Reports of spontaneous eruption of teeth through tissue engineered bone23 and orthodontic movement of teeth into the tissue engineered bone24 have been published. Te offers various advantages like absence of donor site morbidity, shorter hospital stays and operating time, and reduced cost. Craven et al (2007) regard the following as keys to success of alveolar grafting: delay bone grafting until maxillary growth is complete but before eruption of canines, coordination of efforts and planning between the surgeon and orthodontist, appreciation of three-dimensional nature of the defect and stagingOutcomes of the of alveolarprocedure cleft where grafting necessary12.

Criteria for successful alveolar cleft is restoration of the alveolar bone height and width, eruption and periodontal health of permanent incisors and canine, successful placement of an implant supported restoration4 and periodontal support of tooth adjacent to the cleft13. A number of validated tools that are being used to assess the outcomes of alveolar bone grafting are Bergland index score, Chelsea index score and Kindelan index score (..). The major shortcoming of these tools is that they are based on a two dimensional radiographs. Currently a three-dimensional imaging like cone beam computed131 10th World Cleft Lip, Palate & Craniofacial Congress tomography (CBCT) are highly recommended for both diagnostic and assessing outcomes. CBCT have far less radiation dose than conventional CT scans, their diagnosticComplications quality25.

A review of secondary alveolar cleft grafting revealed the following complications seroma (4%), superficial donor site abscess (1%), postoperative pyrexia of unknown origin (2%), gingival bleeding (2%), and oral infection (2%)26. Other complications that have been reported are loss of graft and residual fistula11. Failures in alveolar cleft reconstruction may be related to factors such as exposed tooth enamel during an operation, postoperative infection, and poor compliance27. Although alveolar bone graft is a widely accepted procedure of managing clefts, controversies exist on timing for grafting, choice of donor sites and surgicalReferences: approaches 01. Watkins SE, Meyer RE, Aylsworth AS. Classification, epidemiology and genetics in orofacial clefts. Clin Plastic Surg 41(2014) 149- 163 02. Blanton et al 2004 03. Santiago P E, Schuster L A, Levy-Bercowski D. Management of alveolar cleft. Clin Plast Surg 2014; 41:219-232 04. Miller LL, Kuaffmann, St. John D, Waite PD. Retrospective Review of 99 Patients With Secondary Alveolar Cleft Graft Repair. 2010; J Oral Maxillofac Surg 68:1283-1289 05. Moreau JL, Caccamese JF, Coletti DP et al. Tissue Engineering solutions for Cleft Palates. J Oral Maxillofac Surg 2007; 65:250-11 06. Skoog T. The use of Periosteal Flaps in the Repair of Cleft of the Primary Palate. Cleft Palate J 1965; 2:332-9 J Oral Surg 1972;30:87-92 07. Wang YC, Liao YF, Chen PK. Comparative outcomes of primary Gingivoperiotoplasty and Secondary Alveolar Bone Grafting in Patients with Unilateral Cleft Lip and Palate. Plast. Reconstr Surg 2016; 137(1):218-27 08. Sato Y, Grayton BH, Garfinkle J, Barillas I, Maki K. Cutting CB. Success rate of gingivopesostoplasty with and wiothout secondary bone graft compared with secondary alveolar bone grafts alone. Plast. Reconstr Surg. 2008;121(4):1356-67

132 10th World Cleft Lip, Palate & Craniofacial Congress 09. Cho-Lee G-Y, Garcia-Diez EM, Nunes R-A, Marti-Pages C, Sieira-Gil R, Rivera-Baro A.. Review of secondary alveolar cleft repair. Annals of Maxillfacial Suurgery 2013; 3:46 10. Precious DS. Alveolar bone grafting. Oral Maxillofac Surg Clin North Am 2000;12: 501 11. Cendrum JLL, Gonzalez-Landa G, Figueroa A. Isolated keratinised gingiva incision in alveolar cleft bone grafts improves qualitative outcomes: A single surgeon’s 23 years experience Journal of Cranio-Maxillo-Facial Surgery 2014; 42:1692-1697 12. Craven C, Cole P, Hollier L, Stal S. Ensuring success in Alveolar Bone Grafting: A Three- Dimensional Approach The journal of Craniofacial Surgery 2007 18; (4): 855-859 13. Liao Y-F, Huang C-S. Presurgical and postsurgical orthodontics are associated with superior secondary alveolar bone grafting outcomes. Journal of Cranio-Maxillofa-Facial Surgery 2015;43:717-723 14. Myerof C, Archdeacon M. Autogenous Bone Graft: Donor Site and Techniques. J Bone Join Surg Am. 2011; 93:2227-36 15. Dolanmaz D, Esen A, Yildirim G, Inan O. The use of autogenous mandibular bone block graft for reconstruction of alveolar defects. Annals of Maxillofacial Surgery 2015; 5:71-75 16. Kusiak JF, Whitaker LA. The early vascularisation of membranous bone. Plast. Recon Surg 1985; 75:510- 6 17. Deatherage J.Bone Materials Available for Alveolar Grafting. Oral Maxillofacial Surg Clin N Am 2010; 22:347-352 18. Sharif F, Rehma IU, Muhammad N, MacNeil S. Dental materials for cleft grafting. Materials Science and Engineering C 2016; 61:1018-1028 19. Binger T, Katsaros C, Rucker M, Spitzer W J. Segment distraction to reduce a Wide Alveolar Cleft before Alveolar Bone Grafting. Cleft Palate-craniofacial Journal 2003; 40:561-65 20. Gladysz D, Hozyasz K. Stem cell regenerative therapy in alveolar cleft reconstucion. Archives of Oral Biology 2015; 60:1517-1532 21. Berger M, Probst F, Schwarts C et al. A concept for scaffold-based tissue engineering in alveolar cleft osteoplasty. Journal of Cranio-Maxillofac-Surgery 2015; 43:830-836 22. Xiao WL, Zhang D-ZChen X.-J, Yuan C, Xue L.F. Osteogenesis effect of guided bone regeneration combined with aveolar cleft grafting: assessment by cone beam computed tomography. Int J. Oral Maxillfac. Surg.2016; 45:683-687

133 10th World Cleft Lip, Palate & Craniofacial Congress 23. Pradel W, Tauche E, Gollogly J et al. Spontaneous tooth eruption after alveolar cleft osteoplasty using tissue -engineered bone: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endo 2008; 105:440-4 24. Behnia H, Khojasteh A, Soleimani M, et al . Secondary repair of alveolar clefts using human mesenchymal stem cell. Oral Surg Oral Med Oral Pathol Oral Radiol Endo 2009;108:e1- e6) 25. Dissaux C, Bodin F, Grollemund B. et al. Evaluation of success of alveolar cleft bone graft performed at 5 years versus 10 years of age. Journal of Cranio-Maxillofacial-Surgery 2016; 44:21-26 26. Amin K, Khor WS, Rosich-Medina A, Beale V. Alveolar Bone Grafting: Donor Site Review of 100 Consecutive Cases in Cleft Lip and Palate. Cleft Palate Craniofac J. 2015 Oct 27 27. Wiedel AP, Svensson H, Schönmeyr B, Becker M. An analysis of complications in secondary bone grafting in patients with unilateral complete cleft lip and palate. J Plast Surg Hand Surg. 2016;50(2):63-7

134 10th World Cleft Lip, Palate & Craniofacial Congress

Perspectives in the management of Complex Craniofacial Anomalies

Krishna Shama Rao Founder and Chief Trustee, Director of Maaya Cleft & Craniofacial centre at Bangalore, Karnataka

Abstract

Congenital Craniofacial deformities are very common and occur one in every 2000 live births in India. This means an estimated 15000 to 20000 non cleft craniofacial deformities are prevalent in India every year. The deformities can be broadly classified as pure cranial, largely facial with or without Orbital involvement and Craniofacial which involves the entire skull. Current technology using 3D printing CT Scans Orbital and Cranial venograms allow us to plan the management of these deformities accurately in anomalies.spite of a degree of financial and infrastructural difficulties. Case illustrations will be provided to illuminate aspects in the correction of these complex

135 10th World Cleft Lip, Palate & Craniofacial Congress

One-piece frontoorbital advancement with distraction but without a bandeau for craniosynostosis

Jong-Woo Choi Department of Plastic and Reconstructive Surgery, University of Ulsan, College of Medicine, Seoul Asan Medical Center, Seoul, Korea

To adopt the traditional frontoorbital advancement technique designed by Tessier in the application of a distraction technique, the frontal bone flap should be detached before the distraction. In order to maximize the merit and potency of the distraction, we have applied the “one-piece cranioplasty” technique without bandeau for coronal craniosynostosis. Our new surgical technique used to treat 10 unilateral and 16 bilateral craniosynosis patients between February 2005 and August 2014. Satisfactory results achieved in all patients. An average distraction of 25.2 mm was possible without detachment from the dura mater. The average cephalic index (width/length × 100) decreased from 98.3 to 89.9 after 3 months postoperatively and was maintained at 88.6 until 6.0 years on average after the operation. In 10 unilateral coronal synostotic patients, the endocranial angulation of the anteroposterior axis was improved from 165.3° to 174.8° after 3 months postoperatively and was maintained at 174.5° until 5.5 years on average after the operation. Our present findings indicate that the novel one-piece frontoorbital advancement with distraction approach appears to be less invasive and suitable for correcting single-suture coronal craniosynostoses, except for the complex form of this condition, even at longer follow-up intervals. In addition, this presentation will discuss the various applications of the one-piece frontoorbital advancement without Bandeau and its outcomes.

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Localised “Vertical Relapse” or Failure of Eruption of the dentition in the Bone Grafted area following Orthodontic Correction in young patients with Cleft Lip Palate: Case Studies

Sikki Singh Private Orthodontic Practice / Wentworth Foundation for Cleft Lip palate, Durban North, South Africa

Introduction

Most Cleft lip palate (CLP) patients are treated from birth to adulthood, going through various phases of treatment by many role players. One of the very important aspects of treatment is orthodontic alignment of the dentition in late childhood with bone grafting of the cleft area. Secondary alveolar bone grafting for patients with clefts involving the maxillary alveolus has transformed dental management of these patients. There has been a steady improvement of the bone grafting procedures and success of grafts (Boyce and Sands, 1972, 1976; Bergland et al, 1986a; Kalaaji et al, 1996; Enemark et al 2001; Micheal Paterson et al 2016) However, a major complication of bone grafting techniques is the resorption of the grafted bone in the cleft area, especially if there are no teeth holding it in place (Feichtinger et al, 2007). Bone grafts in the cleft areas are normally done around the ages of 9 years to 11 years, to coincide with the eruption of the canine into the grafted area. Various studies have indicated that the erupted canine with good periodontal support helps maintain the grafted bone. This allows for orthodontic tooth movement (De Winter et al, 2003). Today most grafts are harvested from the iliac crest. Other grafts include mandibular symphyseal , retromolar pad and allografts. A study in Netherlands (Ad de Ruiter et al,2010) showed a positive outcome with mandibular symphyseal bone grafted into the cleft area. This study mentions life time fixed upper and lower retention for cleft lip palate patients. 137 10th World Cleft Lip, Palate & Craniofacial Congress

Although most clefts lip palate patients have missing lateral incisors in the cleft area, those that have lateral incisors can be considered for earlier bone graft at age 6-7, rather than waiting for the canine to erupt at ages 9-11years. This is still controversial (Dafna S and M. Lena Omnell, 2000). Search of the literature shows few studies or research undertaken to show stability of the dentition in the bone grafted cleft area following orthodontic treatment in young prepubertal and pubertal cleft lip palate patients. The type of relapse shown here is localised to the cleft area. This has not been investigated previously.Objective of Case Studies

To show the rapid localised relapse of well aligned upper teeth in young cleft lip palate patients who had bone grafts placed in the cleft area. The relapse, inspite of good retention appliance wear is vertical, suggestive of failure of eruption. This also questions the method of providing retention after orthodontics.Materials and Methods

Three cases will be demonstrated. They include two unilateral cleft lip alveolus and one unilateral cleft lip palate .These are patients from a joint private practice / public service cleft lip palate clinic in the province of Kwa Zulu Natal in South Africa . These patients have been treated from birth. All ethical considerations have been taken into account. The necessary permission for publication of patient information has been obtained. This is a photographic evaluation and depicts dental changes in the post orthodontic treatment phase (i.e. retention phase). It is confined to those cleft lip palate patients who received bone grafts during the growing phase of life in line with eruption of the canine. All grafts were from the iliac crest. An outline of the active treatment phase and the passive post treatment phase (retention) will be given, showing the changes in the dentition. “Debanding” of patients refers to removal of fixed braces. The patients comprised of two young females andCase one I : Unilateralmale. All are Cleft based Lip inAlveolus Kwa Zulu Natal, South Africa.

This patient is a young male. He presented with a Unilateral Cleft Lip and Alveolus. The primary operation for the lip was done at +/- 4 months of age. A preliminary first phase orthodontic treatment was commenced at the age of 9 years with a removable expansion device (Fig 1 A, B). The patient had a138 supernumerary in the Cleft area, on the mesial side of the cleft. The lateral 10th World Cleft Lip, Palate & Craniofacial Congress

incisor (22) was on the distal side of the cleft.

Figure 1A - Pre-treatment Figure 1B – Intra-Oral Pre-treatment The second fixed phase commenced at the age of 12 years. The bone graft taken from the iliac crest, was done within 3 months of commencement of fixed orthodontic treatment. The supernumerary was extracted at the same time as placement of the graft. Space closure began +/- 6 months after placement of the graft, to allow for proper healing (Fig 2 A, B).

Figure 2A – During Treatment Figure 2B –Panorex During Treatment On completion of fixed orthodontics at age 14 years, the patient was debanded (fixed braces removed) and placed on removable upper and lower retainers (Fig 3 A, B, C).

Figure 3A – Day of Deband Figure 3B – Intra-Oral Day of DebandFigure 3C – Intra-Oral Day of Deband

Six months into the retention phase the failure of the eruption of the teeth adjacent to the cleft became apparent (Fig 4). This trend has continued and the vertical relapse has worsened. The situation worsened one year into retention139 10th World Cleft Lip, Palate & Craniofacial Congress

(Fig 5 A, B). To prevent further relapse a fixed retainer was placed on the teeth adjacent to the cleft. This will be there until retreatment at the age of 18 years to correct overbite (Fig 6 A, B).

Figure 4 – Intra-Oral six months post deband

Figure 5A – One year post deband Figure 5B – One year post deband

Case 2 : UnilateralFigure 6A –Cleft Fixed Lip Retention Alveolus Figure 6B – Fixed Retention

This patient is a young female and she presented with a Unilateral Cleft Lip Alveolus (Fig 7 A, B). Fixed orthodontic treatment started at age 9 ½ years. The cleft was on the left side with the lateral incisor on the distal aspect of the cleft. The bone graft, from the iliac crest, was done +/- 12 months after beginning fixed orthodontics. No movement of the teeth into the cleft area was undertaken for +/- 6 months to allow for the consolidation of the graft. The patient was debanded at the age of 11 ½ years (Fig 8 A, B) and placed on upper and lower retainers. One year post retention the patient presented with vertical relapse of the teeth adjacent to the cleft (Fig 9 A, B). 140 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 7A – Pre-Treatment Figure 7B – Intra-Oral Pre-Treatment

Figure 8A – Day of Braces removal (Deband) Figure 8B – Intra-Oral. Day of Deband

Figure 9A – One year post-deband Figure 9A – One year post-deband The patient was retreated with fixed to realign the upper teeth (Fig 10 A, B). A fixed retainer was fitted to the teeth in the cleft area (Fig 11). The patient Casemay need 3 : Uni-Lateral retreatment Cleft at age Lip 18Palate: years.

This young patient is a female and she presented with a Unilateral Cleft Lip Palate. The cleft was on the left side. The Panorex showed presence of a supernumerary on the distal aspect of the cleft and a lateral incisor on the mesial side of cleft. A preliminary phase orthodontic treatment was started at the age of 8 years with a removable expansion device (Fig 12 A, B).

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Figure 10 A - Retreatment Figure 10B - Retreatment

Figure 11. A fixed retainer was fitted to the teeth in the cleft area

Figure Figure 12A – Pre-Treatment Figure 12B – Intra-Oral Pre-Treatment The second phase fixed orthodontics was started at the age of +/- 9 years. A bone graft from iliac crest was placed in the cleft area when the patient was 1 year into the fixed orthodontic treatment. Patient was +/- 10 years of age (Fig 13 A, B). The supernumerary was actively erupted through the graft in an effort to consolidate the graft. This was not too successful. A second bone graft and extraction of supernumerary was done almost a year later at age 11 years (Fig 14 A, B). This was followed by space closure. The patient was debanded a year later (after almost 40 months of fixed orthodontic treatment). Patient was placed on a removable upper retainer and lower fixed retainer (Fig 15A, B, C). At the end of six months of retention wear there was a definite vertical relapse (failure of eruption) of teeth adjacent to the cleft (Fig 16 A, B). After 12 months of retention the teeth adjacent to the cleft showed no signs of eruption, with a worsening of anterior overbite (Fig 14217A, B). Two years post deband the trend continued with vertical movement 10th World Cleft Lip, Palate & Craniofacial Congress

of the teeth in the cleft area (Fig 18A, B). A fixed retainer was placed to prevent further relapse. It was decided to retreat patient at 18 years of age to establish a good overbite.

Figure 13A – During Treatment First bone graft Figure 13B – During Treatment First bone graft

Discussion:Figure 14A –During Treatment Second Bone graft Figure 14B – During Treatment Second Bone graft

Although orthodontic relapse is a major problem in cleft lip palate patients, very few studies have been undertaken to determine the causes of relapse. Much has been written about surgical relapse following orthodontics and orthognathic surgery (Guy D. Watts et al, 2016, Saltaji et al 2012) and collapse of the maxillary arch post orthodontics and orthognathic surgery (Talat Al-Gunaid et al, 2008). Although the timing of bone grafts with eruption of canine produced more stable grafts, no studies have shown how teeth erupt through the graft. Also, we seem to know very little about movement of teeth through the various types of grafts (autogenic /alloplastic grafts). This study focused on localised vertical relapse or failure of eruption of the teeth in the cleft area following orthodontic treatment and bone grafting. It’s causes can be varied, example , loss or shrinkage of grafted bone, type of bone or scar tissue. This article is a review of cases from an orthodontic practice specializing in cleft lip palate work. To validate this observation, further research involving longitudinal and cross sectional studies using certain143 10th World Cleft Lip, Palate & Craniofacial Congress yardsticks (example Goslon Yardstick) will need to be undertaken with many cases and from many centres.

Figure 17A – One year post deband Figure 17B – One year post deband

Conclusion:Figure 18A – Two years post deband Figure 18B – Two years post deband

The failure of eruption of teeth in the grafted cleft area of the maxilla in the growing patients is a common observation in our cleft clinic. In view of this we have added a new retention protocol. In addition to the post orthodontic removable retainer a fixed bonded retainer is placed on all teeth adjacent to the cleft. It is important that the bonded retainer be placed from non-cleft area to non-cleft area, incorporating 2/3 teeth on either side of the cleft.

144 10th World Cleft Lip, Palate & Craniofacial Congress

Transversal problems in cleft patients

Martín Romero Maroto Profesor titular de Odontopediatría y Ortodoncia, Universidad Rey Juan Carlos, Spain

Transversal problems are one of the main problems in cleft lip segments.and palate patients. One of the objectives of presurgical orthopedics is to reduce the width of the cleft and in some cases it is necessary to expand the

In cleft patients the major cause of maxillary dental arch constriction is scar tissue distribution on the palate. In the initial healing phase, transverse growth of the alveolar bone is affected by wound contraction and the contractile force exerted by myofibroblasts in granulation tissue. Palatal expanders may be used in all phases of treatment and in some cases expansion has to be achieved with orthognatic surgery.

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Craniofacial Deformities and Orthodontics: Perils and Plights: Are we Crying Wolf?

Amish Mehta Consulting Orthodontist,, Chandan Orthodontic Clinic and Superspeciality Dental Care,, Krishaaryan, Professor, Head, and Post Graduate Guide, Department of Orthodontics and Dentofacial Orthopaedics, Faculty of Dental Science, Dharmasinh Desai University, Vice President, Indian Orthodontic Society, India

Abstract

The Speaker has 25 years experience in managing Orthodontic Problems in Craniofacial Deformities Including CLPs. The Lecture will encompass contemporary International Protocols in managing Craniofacial Deformities as a team in General and with Orthodontics in Particular. An over view of the Indian scenario and understanding on the topic will be exemplified with plethora of CLP and select craniofacial cases involving mild to severe conditions managed with simplest mechanics to complex appliances. The differential biomechanics and its modifications for these conditions will be highlighted. In tandem with the theme of the congress, the Lecture will also focus on challenges we face still in Prevention of Orthodontic Problems in CLPs in our country. At the end of the lecture, the attendee shall learn basic treatment planning and time scheduling in Orthodontic management of CLPs, complications arising out of lack of timing and chronologic interventions, Biomechanical and appliances employed in treatment of CLPs, and have a sound overview of current Understanding and scenario in India with its advantages and limitations.

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Social media in education towards cleft prevention

1Amin Pasha Samadian, 2Marie M. Tolarova 1Cleft Prevention International Foundation, USA, 2University of the Pacific, San Francisco, California, USA, 2ANAMAYA Indo-Pacific Cleft Prevention Program, Chennai, INDIA

Introduction

Social media, nowadays, is one of the most powerful tools for spreading news. From official news networks sending breaking news on the notification centers of our cellphones with links to two-minute videos that quickly convey a clear message, to people individually broadcasting their personal recordings of an event, to politicians debating over their twitters and to people sharing their memories on Facebook. Education has undergone a huge improvement with the help of social media. Today, many people have instant access to almost all the knowledge over internet and can share that information withinMethod their virtual networks. Existing information that was accessible through social media about prevention of birth defects and cleft prevention, specifically, was reviewed and theResults content, outreach, and efficacy critically evaluated. The goal of this presentation is critically look at pros and cons of using social media and internet for awareness building. We will present several examples that can be used in social media for different age, gender, social strategies.economic status, and other population groups to disseminate knowledge and information related to causes of cleft lip and palate and about cleft prevention Discussion

Through access to social media, we can convey our message useto billions a format of and people language who, that otherwise, is interesting would and not easily be able understandable. to hear us. We can specifically target different populations and, thus, educate them for free and Conclusion

We strongly believe in a significant role of social media in our cleft prevention activities worldwide. In developing countries, its importance is even more critical. There are strong platforms that we can benefit from. Now is the time to join them and change the traditional ways of spreading our message. 147 10th World Cleft Lip, Palate & Craniofacial Congress

Cleft prevention from patient – doctor perspective

Héctor Villegas Álvarez1, 2, Roberto Ruiz1, Eileen Uribe-Querol1, Marie M. Tolarova2, 3 1National Autonomous University of Mexico (UNAM) School of Dentistry, Mexico City, Mexico, 2Cleft Prevention International Foundation, USA, 3University of the Pacific, San Francisco, California, USA

Objective

To describe the process of adaptation and expectations of family-patient- clinicianDescription in the treatment of cleft lip and palate (CLP) from a personal experience.

Each stage of treatment is accompanied by doubts, fears and specific conditions of each individual case. It is important that the team treating the patient, will finally resolved them. Presentation will focus on the process of sharing expectations, data from the literature, and personal experience in process of treatment in patient-family-doctor setting. Critically important in the treatment of CLP - in their respective order - are love, psychology and voice. Patient should know that he/she is not alone - orofacial clefts (OFC) are the second most common malformations in humans. Every two minutes a baby with OFC is born into the world; only in India 3 babies are born with OFC every hour. Patients should know that 70% of CLP are non-syndromic cases and significant proportion of these are preventable - as it has been shown in some studies and populations. Thus, while improving OFC treatment outcome and supporting access to treatment for every individual with a cleft in the world – the best approach to reducing the incidence of OFC is investment in cleft prevention.Conclusions

Empowering families in participation during the treatment of OFC is crucial for successful treatment; not all cases behave during treatment as literature indicated. As OFC are one of the biggest public health problems worldwide, cleft prevention strategies are important toward its solution.

148 10th World Cleft Lip, Palate & Craniofacial Congress

Orthodontist’s role in the treatment of patients with nonsyndromic cleft lip and palate. Importance of prevention

Héctor Montoya1,2,3; Cecilia Cantero1,3; Lorena Orellana1,3,4 1University of Concepción, Concepción, Chile, 2Rotary Club Concepción, Concepción, Chile, 3Cleft Prevention Program, University of Concepción, Concepción, Chile, 4Special Dental Care Unit, School of Dentistry, University of Concepción, Chile

Introduction

The treatment of cleft lip and palate is complex and therefore requires the participation of several specialists. The intervention of orthodontists in the comprehensive treatment of these patients is prolonged, therefore it is important to maintain good coordination with other professionals. Considering the complexity of treating of these patients is necessary to consider how to prevent its occurrence. The aim of this presentation is to release the role of the orthodontist in the care of patients with cleft lip and palate in Concepcion, Chile andMetodology the importance of its prevention.

Cases of patients with nonsyndromic cleft lip and palate who come to the Cleft Unit of the Regional Hospital of Concepción, Chile, some results of a cross-sectional study of patients with cleft lip and palate and presentation of a brochureResults on preventing congenital malformations.

Most patients with this condition attending the Regional Hospital of Concepción had unilateral or bilateral cleft lip and palate (81%). There are several factors that determine the complexity of orthodontic treatment of a complicated uni / bilateral cleft. This treatment requires several stages such as pre-surgical, in primary, in mixed and permanent dentition. Most of these patients treatment begins before the month of life (75%). To promote the prevention of congenital malformations a brochure was prepared.

149 10th World Cleft Lip, Palate & Craniofacial Congress Discusion

The complexity of the treatment, its long duration and low socio- economic and cultural conditions generate, often, that these patients do not complete their comprehensive treatment being severely anatomical, functional and psychological consequences. There is little awareness in the community aboutConclusions prevention of congenital malformations.

It is important to carry out prevention campaigns of congenital malformations for the community through different means, because a significant percentage of cleft lip and palate can be prevented.

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Prevention as Solution of Global Health Issues Related to Cleft Lip and Palate

Marie M. Tolarova1, 2, 4, Terezie T. Mosby2, 3, 4, Mirek Tolar1, 2, 4, Amin Samadian1, 2, Shreekala Kandapalanivel1, 4, Hector Montoya1, 5 1Cleft Prevention International Foundation, USA, 2University of the Pacific, San Francisco, California, USA, 3Mississippi State University, Starkville, Mississippi, USA, 4ANAMAYA Indo-Pacific Cleft Prevention Program, Chennai, INDIA, 5University of Concepcion, Concepcion, CHILE

The oral health is, and always has been, on the top of initiatives of medical and dental professionals, pharmaceuticals, many NGOs, and even investors. According to ADA oral health is a functional, structural, aesthetic, physiologic and psychosocial state of well-being and is essential to an individual’s general health and quality of life. Thus, when it comes to public health issues worldwide, oral diseases represent the most common diseases with significant socio-economic impacts, however still frequently neglected in public health policy (Jin et al, 2016). The oral data extracted from the GBD Study indicate that dental caries, periodontal disease, edentulism, oral cancer, and cleft lip and palate collectively accounted for up to 18,814,000 disability adjusted life-years (DALYs) in 2010, an average of 20.4% increase from 1990 (Murray et al, 2012; Marcenes et al, 2013). These major oral diseases are with no doubt a serious global burden. Many common risk factors with non-communicable and systematic diseases are well defined for dental carries, periodontal diseases, edentulism, cancer, and also for cleft lip and palate. Therefore it is very important to integrate oral health into general global health agenda focusing on common risk factors (Jin et al, 2016). embryo and early fetus, thus also more than any other areas prone to abnormal The orofacial region is one of the most sensitive part of developing human development that leads to congenital anomalies. Cleft lip and palate anomalies are the most common and most severe congenital anomalies of the face and mouth and the second most common birth defects in general.

151 10th World Cleft Lip, Palate & Craniofacial Congress “One size does not fits all”

Data on the prevalence of orofacial clefts reported in the literature vary according to the investigator and the country (Tolarova and Cervenka, 1998a). There is no doubt, and it has been well documented, that real differences exist by ethnicity (Croen et al 1998; Tolarova, 1998b) and even within country, based on SE status (Womersley and Stone, 1987; Carmichael et al., 2009) or different altitude, or just different state in the same country. IPDTOC Initiative presented six-fold variation in the prevalence at birth of cleft lip with or without cleft palate (IPDTOC Working Group, 2011) and thee-fold variation in the prevalence at birth of cleft palate (Mossey et al 2011). In our population based study of 2,509,881 California births 4,433 cases of all types of orofacial clefts (nonsyndromic CL, CLP, CP; clefts in syndromes and in multiple congenital anomalies and chromosomal aberrations) were ascertained giving birth prevalence 17.66 per 10,000 birth - one in every 566 newborns (Tolarova and Cervenka, 1998a). Translated into time, a baby will be born with a cleft somewhere in the world every two minutes, 648 per day, and 236,400 per year. Data from IPDTOC collaborative project that involved 54 registries in 30 countries during the period of 2000-2005 and reported 7704 CL+-P in 7.5 million births giving birth prevalence of 9.92 per 10,000 birth (IPDTOC Working Group, 2011). In our California population based study the birth prevalence for the same group was a little higher 10.49 per 10,100 birth (Tolarova and Cervenka, 1998a). IDCFA reporting data for 2001-2003 from International Clearing House reporting birth prevalence of CP as 4.9 per 10,000 birth and total for oral clefts 16.7 per 10,000 birth (Mastroiacovo et al, 2005) which is very similar to our Californian data. Differences in orofacial clefts prevalence that we are seeing in different ethnicities, countries, locations, and SE groups, really correspond differences that we are seeing when analyzing genetic and environmental (non-genetic) factors in orofacial clefts etiology. Most orofacial clefts – about 65-70%, like most common congenital anomalies, are caused by the interaction between genetic and environmental factors (Figure 1). In those instances, genetic factors create a susceptibility for clefts. When environmental factors (i.e., triggers) interact with a genetically susceptible genotype, a cleft develops during an early stage of development (Tolarova, 2015).

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Figure 1. Schema etiology of cleft lip and palate

The proportion of environmental and genetic factors varies with the sex of the individual affected with cleft. In CL and CP, it also varies with the severity and the unilaterality or bilaterality of the cleft anomaly; the highest proportion of genetic factors are in the subgroup of females with a bilateral cleft, and the smallest proportion is in the subgroup of males with a unilateral cleft. Thus, the classic multifactorial threshold (MFT) model of liability can be applied to nonsyndromic cleft lip with or without cleft palate as the multifactorial model of liability with four different thresholds (Tolarova, 1984). The present genomic era enable us to study a large number of genetic polymorphisms and loci and numerous studies were published in the last 10 years. However, there is a lot of inconsistency in published data (Table 1).

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One reason for the ongoing uncertainty in relation to interactions include low statistical power to detect or exclude interactions and differences between studies in individuals who have been genotyped (Mossey et al 2011). However, we believe that of the same importance is not to overlook the real differences in both – genetic (susceptibility genes polymorphisms) as well as in non-genetic/ environmental factors (mothers’ nutrition, maternal cigarette smoking, exposure to secondhand tobacco smoke in periconceptional period, life style factors, etc.) among different populations. Thus “one size does not fits all”. And this is very important to keep in mind when developing cleft prevention protocols. When looking at the same etiological factors in three samples from different cleft populations, only a small proportion will overlap, in other world will be the same for all three samples (Figure 2). The vast majority is “location” and “population” specific. Results on many studies of genes, mothers’ nutrition, lifestyle factors, suggest that a different spectrum of genetic factors and also a different spectrum of environmental factors, specifically those related to mothers’ diet during pregnancy, exist in different populations (Tolarova, 2014). Several nutrients, folate, zinc, and B6 in low doses and Vitamin A in high doses seems to be critical and we introduce for them a term “candidate nutrients”. We can find them commonly in several populations studied (see overlapping area on Figure 2). Therefore, prevention approach must address these differences. Figure 2. Diagram of fictive etiological factor for cleft lip and palate in three different population samples that are overlapping in very small area. What we can expect? Thus, let us estimate that

results from these studies, there when we combine and averaging

is birth prevalence of orofacial clefts (CL, CLP, and CP) about 17 per 10,000 birth or about 1 in every 600 birth. The Table 2 shows World Vital Events for 2016 according US Census and we add as the last column an estimate for number of individuals with a cleft. This year 135,308,628 babies are/will be born and out of them 230,025 are/will be born with any type on orofacial cleft. In other words, every 2 minutes is one baby born with a cleft. If we focus only on 154individuals with a cleft and no other anomalies (nonsyndromic clefts), we can 10th World Cleft Lip, Palate & Craniofacial Congress

conservatively estimate that one individual with a cleft is among every 1,000 people. Thus, if natural increase this year (Table 2) for year 2016 is 78,165,166 individuals, 78,165 are those with a cleft.

Thus, there are about 7.32 million individuals with a cleft living on this planet at present. Twenty years from now, with the projected population growth, there will be 1.36 million more of them. And in the year 2050, the cleft population will reach 9.37 million – 2 million more than today (Figure 3 and Figure 4).

Figure 3. Projection of world population

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WhereFigure is the 4. situation Projection most of cleftcritical? population

When looking more in detail at estimate of population growth (Table 3) the most populated countries are China, India, and USA. While population, when comparing 2015 and projected data for 2050, is for more developed countries practically the same, the biggest change is expected in least developed countries (1.89 times) when population will almost double. Increase for less developed countries (LDC) is estimated 2.9 billion which translate to additional 2.9 million of individuals with a cleft in less develop countries. The only way how to deal with this is investment in development of cleft prevention strategies and programs! Table 3. Projection of populations in more, less, and least developed countries.

156 10th World Cleft Lip, Palate & Craniofacial Congress Historical studies of cleft prevention

Medical community as well as public are, nowadays, aware of prevention of neural tube defects by folic acid supplementation. However, even earlier than attempts to prevent NTD were carried on, early findings of Warkany and Nelson inspired plastic surgeons operating on babies with cleft lip and palate to administer multivitamins to pregnant women. Dr. Joseph Warkany, who is well known as cofounder of , developed experimental animal models for testing various dietary vitamin deficiencies during pregnancy that led to development of birth defects. It was his astonishing finding that pregnant mice fed a diet lacking vitamins delivered offspring with various malformations (Warkany and Nelson, 1940; Warkany and Shraffenberg). A decade later, attention was turned to antimetabolites and their effects on fetal development (Nelson, 1955). In late1950’, several papers were published on use of folic acid antagonist, 4-aminopteroylglutamic acid, in therapeutic abortions (Thiersch, 1952) and on congenital anomalies that developed after use of 4-amino PGA (Meltzer, 1956; Warkany et al, 1959; Hernandez-Diaz et al, 2000). Motivated by Warkany studies first studies to prevent cleft lip and palate were done by Peers, Conway, and Douglas (Conway, 1958; Douglas, 1958; Peer et al, 1958; Peer et al, 1963; Peer et al, 1964). Soon, Burian initiated a cleft prevention study in Prague (Burian, 1964) Studies in the US continued (Briggs, 1976) and more European colleagues were also searching for the answer, whether cleft lip and palate anomalies could beNeural prevented tube defect (Kreybig prevention et al, 1978; studies Gabka, and 1981; our firstSchubert cleft etprevention al, 1990). results

But not only unanswered questions about etiology of NCLP created curiosity among clinicians and researchers. Another serious and common congenital anomaly severely debilitating child’s life – neural tube defects (NTD) - became intensively studied in relation to environmental triggers and specifically to a role of folic acid. Already in 1930’s and 1940’s, the studies on experimental animals indicated that mother’s vitamin deficiency could cause congenital malformations in offspring (Hale, 1930; Warkany and Nelson, 1940; Warkany and Shraffenberg, 1943). In 1960’s, it was demonstrated that formiminoglutamic acid excretion test for defective folate metabolism was more often positive in women pregnant with a child with NTD or other congenital anomaly than in controls (Hibbard and Smithells, 1965). Later, it was shown that periconceptional supplementation with multivitamins (Smithells157 et 10th World Cleft Lip, Palate & Craniofacial Congress

al, 1981) or folic acid (Laurence et al, 1981) had a role in prevention of NTD. However, it seemed impossible to consider prevention of congenital anomalies as an ultimate goal of teratology (Warkany, 1981) and a need for randomized clinical trials (RCT) was apparent. We published the first results of our cleft prevention study in the Lancet in 1982 (Tolarova, 1982a). We demonstrated a significant decrease of recurrences in the case group of 85 supplemented pregnancies (p=0.023, Fisher exact test). Our study was ongoing and several presentations and publications followed reporting progress of this study (Tolarova, 1987; Tolarova, 1990a, Tolarova, 1982b; Tolarova, 1988; Tolarova, 1989, Tolarova, 1990b; Tolarova, 1993). A randomized, controlled, double-blind, multicenter clinical trial sponsored by the British Medical Research Council (MRC) showed a 72% decrease in the recurrence of NTD when women took 4 mg/day of folic acid periconceptionally (MRC Vitamin Study Research Group, 1991; Wald, 1993; Wald, 1994). Thus, results clearly demonstrated the role of folic acid (FA) supplementation in the prevention of NTD. Moreover, there appeared numerous articles pointing to a preventive effect of FA in other dysraphic congenital birth defects (Czeizel, 1993; Czeizel, 1996). Some also suggested a possible preventive effect of FA in respectFolic acid to chromosomaland prevention aberrations of nonsyndromic (Hobbs cleft et al, lip2000). and palate (NCLP)

A number of observational and interventional studies focused on a preventive effect of FA on orofacial cleft anomalies (Tolarova and Harris, 1995; Tolarova, 1982a; Czeizel, 1993; Czeizel, 1996; Shaw et al, 1996; Amitai Y, 1999; Bienengraber et al, 2001; Czeizel et al, 1999; Loffredo et al, 2001; Botto et al, 2003; Czeizel, 2004; Rooij van et al, 2003; Wilcox et al, 2007; Little et al, 2008; Bille et al, 2007; Shaw et al, 1995; Werler et al 2007; Itikala et al, 2001; Hayes et al, 1996; Johnson and Little, 2008; Badovinac et al, 2007; Czeizel and Dudas, 1992). While the vast majority presented suggestions or evidence for a preventive effect, an unequivocal agreement was not found. An extensive overview of these studies was compiled recently by Wehby and Murray (2010). All these findings confirm an urgent need for a randomized, double-blind, controlled multicenter trial to rigorously and conclusively establish whether periconceptional vitamin supplementation prevents NCLP and, if it does, whether the effective agent is folic acid, and more, what is the effective dose needed. Over the past eight years, Wehby and Murray have developed the Oral Cleft Prevention Program as a double-blinded RCT that has been run in Brazil to158 estimate the effect of periconceptional supplementation with a high dose of 10th World Cleft Lip, Palate & Craniofacial Congress

folic acid (4 mg/day) on prevention of NCLP recurrences. In our non-randomized interventional study, we found a dramatic reduction of cleft recurrences after periconceptional supplementation with multivitamins and a high dose of FA. Our first results were published in Lancet in 1982 (Tolarova, 1982a) and the complete final evaluation later (Tolarova, 1995). To assess effects of periconceptional multivitamin and folic acid supplementation on recurrence of NCLP, we prospectively evaluated 221 pregnancies of women at risk for a child with NCLP. A ten-step protocol included multivitamin supplementation with SPOFAVIT (vitamins A, B1, B2, B6, C, D3, E, nicotinamide, calcium pathothenicum) and folic acid (10 mg/day) beginning at least two months before a planned conception and continuing for at least three months thereafter. A comparison group comprised 1,901 women at risk for a child with NCLP who received no supplementation and gave birth within the same period as the study group. In the supplemented group, 3 of 214 informative pregnancies ended with infants with NCLP, a 65.4 % decrease (P=0,031, Fisher’s exact test); the expected value of 8.67 was calculated based on the incidence of clefts among first-degree relatives in the comparison group (Table 4). Table 4. Czech cleft prevention study - nonrandomized prospective trial of prevention of recurrences by multivitamins and high folic acid (10 mg/day) periconceptional supplementation

Subset analysis by proband’s sex, severity of NCLP, and both variables showed the highest supplementation efficacy in probands with unilateral cleft (82.6 % decrease, P=0.024, Fisher’s exact test.). No efficacy was observed for female probands with bilateral NCLP. Generally, the efficacy was greater for subgroups with unilateral cleft in comparison with bilateral clefts and for male probands in comparison with female probands. 159 10th World Cleft Lip, Palate & Craniofacial Congress

Similarly, a large population-based case control study in California demonstrated that periconceptional use of multivitamins, which usually contained 0.4 mg or more of FA, reduced the risk for NCLP by approximately 27-50 %. This was based on data derived from a population-based case- control study on fetuses and live-born infants with orofacial anomalies in the 1987-89 cohort of births in California. Interviews were conducted with 731 (84.7 %) eligible mothers having an infant with NCLP and with 734 (78.2 %) control mothers having non-malformed infants. Results showed a reduced risk of orofacial clefts, if the mother had used multivitamins containing FA during the period starting one month before conception and continuing for two months after conception. Women who used multivitamins containing FA periconceptionally had 25-50 % reduction of the risk for an offspring with NCLP compared to women who did not use such multivitamins. However, this association may not be attributable to FA specifically, but may be a consequence of other multivitamin supplement components, or behaviors, that are highly correlated with the use of multivitamins containing folic acid (Shaw et al, 1995). In 1996, prompted by our published studies that were suggesting preventive effect of FA on recurrence of NCLP (4) and on occurrence of NCLP (36), we organized the symposium “Approach to the Prevention of Orofacial Clefts” in Emeryville, California (1996). Several meetings discussing extensively an optimal design for a NCLP prevention trial followed (WHO Meeting on the Prevention of Orofacial Anomalies in 2001, Global Strategies to Reduce the Health-Care Burden of Craniofacial Anomalies in 2002). We are aware that there are several key questions that need to be addressed in future scientific studies in order to clarify an association between NCLP and a lack of folate and other vitamins in the maternal diet. The complex etiology of NCLP in humans is still poorly understood. The vast majority of orofacial clefts (cleft lip, cleft lip and palate, and isolated cleft palate) are nonsyndromic (Tolarova, 1998a; Tolarova, 1998b). Increasing evidence suggests that NCLP has a multifactorial etiology and both genetics and environment play significant roles in cleft development. It is believed that while genetic factors create susceptibility for clefts, environmental factors trigger development of the cleft through their interactions with a susceptible genotype. Later, data from RCT study in Hungary (Czeizel et al, 2004) and population- based observational Atlanta studies (Botto et al, 1996, 2000, 2003) showed that periconceptional multivitamins and high dose of folic acid also significantly decrease rate of congenial heart defects. Another observation of a significant reduction160 of birth prevalence of congenital heart defects has been reported 10th World Cleft Lip, Palate & Craniofacial Congress

from Quebec, Canada folic acid fortification of grain products (Ionescu-Ittu et al, 2009). Further steps that will help efficacy of cleft prevention are focusing on processes, by which a specific DNA genotype is translated into a specific phenotype. These will include analyses of mechanisms regulating gene expression – epigenetics (methylation of CpG, acetylation of histones, regulatory microRNAs), gene-gene interactions (balance in gene networks, metabolic pathways), and gene-environment interactions. Thus, we will need to consider very seriously one more player in etiology of orofacial clefting: epigenetics (Figure 5). There is no doubt that investment in prevention of birth defects has the highest return. It has been shown clearly in the US when folic acid fortification project was evaluated in respect to NTDs. The food fortification of all cereal grain products (0.14mg FA/100g) started in the US in January 1998. The last evaluation (Grosse et al, 2005) estimated the benefit-cost ratio as 40:1, which year.represented an estimated economic benefit as $312-425 million annually and the savings (net reduction in direct costs) in range of $ 145-588 million per Conclusion

In conclusion, several birth defects that are known as multifactorial – i.e., in their etiology both genetic factors (susceptibility or candidate genes) and environmental factors are involved – are preventable. Bioavailability of folic acid is critical for normal development of human embryo. Also other nutrient deficiencies (like zinc and vitamin B6) and a high content of vitamin A in mother’s diet during embryonic period of development are necessary to be taken in consideration (Tolarova et al 2012). Preventive measures in general population will involve in addition to activities like folic acid fortification of grains, also addressing risk factors that are common with common diseases (consumption of alcohol, diet with high sugar, salt, and high saturated and trans fat, life style with stressful activities, etc.). However, the very first step in any kind of prevention is education carried on all social levels: professionals (Elavenil et al. 2010), health care workers, parents, patients, adolescents, NGOs, families with an individual with a cleft, patients, and general public, and other groups. We need to create an educational structure that will involve all of those groups. Each will need specific educational tools, material, approach, and strategies. 161 10th World Cleft Lip, Palate & Craniofacial Congress

There is already enough scientific evidence suggesting that a significant proportion of cleft lip and palate anomalies are preventable. These anomalies are the second most frequent among all birth defects and the first and most serious one in the orofacial region. The fact that cleft lip and palate anomalies belong to those that are preventable is especially significant in less and least developed countries (see Table 3). Let us work together toward prevention of birth defects, share our knowledge and resources – and we will be able to follow the Hippocratic Oath notReferences only to treat but even to prevent an abnormality and to help many! 1. Amitai Y. Periconceptional folic acid and multivitamins for prevention of congenital anomalies and improving maternity health. In: Multivitamins for Prevention of Congenital Anomalies. Publication of the Ministry of Health of Israel, http://www.health.gov.il, 1999 2. Approach to the Prevention of Orofacial Clefts. Workshop organized by the California Birth Defects Monitoring Program and Centers for Disease Control and Prevention. Emeryville, CA, March 17-18, 1996 3. Badovinac RL, Werler MM, Williams PL, Kelsey KT, Hayes C. Folic acid-containing supplement consumption during pregnancy and risk for oral clefts: a meta-analysis. Birth Defects Res A Clin Mol Teratol 79:8-15, 2007 4. Bienengraber V, Malek FZ, Moritz K-U, Fanghanel J, Gundlach KKH, Weingartner J. Is it possible to prevent cleft palate by prenatal administration of folic acid? An experimental study. Cleft Palate Craniofacial J 38:393-398, 2001 5. Bille C, Olsen J, Vach W et al. Oral clefts and life style factors – a case-cohort study based on prospective Danish data. Eur J Epidemiol 22:173-181, 2007 6. Botto, L.D.; Khoury, M.J.; Mulinare, J.; Erickson, J.D. Periconceptional multivitamin use and the occurrence of conotruncal heart defects. Results from a population based case- control study.Pediatrics 1996, 98, 911–917. 7. Botto, L.D.; Mulinare, J.; Erickson, J.D. Occurrence of congenital heart defects in relation to maternal multivitamin use. Am. J. Epidemiol. 2000, 151, 878–884. 8. Botto, L.D.; Mulinare, J.; Erickson, J.D. Do multivitamin or folic acid supplementation reduce the risk for congenital heart defects? Evidence and gaps. Am. J. Med. Genet. 2003, 121A, 95–101. 9. Briggs R. Vitamin supplementation as a possible factor in the incidence of cleft lip/palate deformities in humans. Clin Plast Surg 3:647-652, 1976

162 10th World Cleft Lip, Palate & Craniofacial Congress 10. Burian F. A research on prevention of inborn malformations. Acta Univ Carol Med Suppl (Prague) 19:43-46, 1964 11. Carmichael SL, Ma C, Shaw GM (2009). Socioeconomic measures, orofacial clefts, and conotruncal heart defects in California. Birth Defects Res A Clin Mol Teratol 85:850-857. 12. Czeizel, A.E.; Dobó, M.; Vargha, P. Hungarian two-cohort controlled study of periconceptional multivitamin supplementation shows reduction in certain congenital abnormalities. Birth Defects Res. 2004, 70, 853–861. 13. Czeizel AE. Controlled studies of multivitamin supplementation on pregnancy outcomes. Ann N Y Acad Sci 678:266-275, 1993 14. Czeizel AE. Folic acid and prevention of birth defects. JAMA 275:1635-6, 1996 15. Czeizel AE, Timar L, Sarkozi A. Dose-dependent effect of folic acid on the prevention of orofacial clefts. Pediatrics 104: e66,1999 16. Czeizel AE, Dudas I. Prevention of the first occurrence of neural-tube defects by periconceptional vitamin supplementation N Engl J Med327:1832-1835, 1992 17. Conway H. Effect of supplemental vitamin therapy on the limitation of incidence of cleft lip and cleft palate in humans. Plast Reconstr Surg 22:450-453, 1958 18. Croen LA, Shaw GM, Wasserman CR, Tolarová MM. Racial and ethnic variations in the prevalence of orofacial clefts in California, 1983-1992. Am J Med Genet. 1998 Aug 27;79(1):42-7 19. Douglas B. The role of environmental factors in etiology of “so-called” congenital malformations. II. Plast Reconstr Surg 22:214-229, 1958 20. Elavenil P, Murugavel C, Kannadasan K, Krishnakumar Raja VB, Gnanam A, Kanimozhi G, Davis D. Folic acid in cleft lip, alveolus and palate prevention: awareness among dental professionals. Indian J Dent Res. 2010 Jul-Sep;21(3):360-3. 21. Gabka J. Verhutung von Lippen-Kiefer-Gaumenspalten. Klinische Erfahrungen. (Prevention of cleft lip and palate. Clinical experience) Munc Med Wochenschr 123:1139- 1141, 1981 22. Grosse, S.D.; Waltzman, N.J.; Romano, P.S.; Mulinare, J. Reevaluating the benefits of folicacid fortification in the United States: Economic analysis, regulation, and public health. Am. J. Public Health 2005, 95, 1917–1922 23. Hayes C, Erler MM, Willett WC, Mitchell A. A case-control study of periconceptional folic acid supplementation and oral clefts. Am J Epidemiol 143: 1229-1334, 1996

163 10th World Cleft Lip, Palate & Craniofacial Congress 24. Hernandez-Diaz, S.; Werler, M.M.; Walker, A.M.; Mitchell, A.A. Folic acid antagonists during pregnancy and risk of birth defects. N. Engl. J. Med. 2000, 343, 1608–1614. 25. Hibbard ED, Smithells RW. Folic acid metabolism and human embryopathy (Preliminary communication). Lancet 1:1254, 1965 26. Ionescu-Ittu, R.; Marelli, A.J.; Mackie, A.S.; Pilote, L. Prevalence of severe congenital heart disease after folic acid fortification of grain products. Time trend analysis in Quebec, Canada. Br. Med. J. 2009, 338, b1673. 27. IPDTOC Working Group (2011). Prevalence at birth of cleft lip with or without cleft palate. Data from the International Perinatal Database of Typical Oral Clefts (IPDTOC) Cleft Palate-Craniofac J 48:66-81. 28. Itikala PR, Watkins ML, Mulinare J, Moore CA, Liu Y. Maternal multivitamin use and orofacial clefts in offspring. Teratology 63:79-86, 2001 29. Jin LJ, Lamster IB, Greenspan JS, Pitts NB, Scully C, Warnakulasuriya S. Global burden of oral diseases: emerging concepts, management and interplay with systemic health. Oral Diseases (2016) 22, 609–619 30. Johnson CY, Little J. Folate intake, markers of folate status and oral clefts: is the evidence converging? Int J Epidemiol 37:1041-1058, 2008 31. Kreybig TV, Stoeckenius M, Fhibiidungen BM. Lippen-Kiefer-Gaumenspalten. Entstehung, Ursachen und Praventionsmassnahmen. (Cleft lip and palate. Development, etiology and prevention) Med Monatsschr Pharm 1:243-249, 1978 32. Laurence KM, James N, Miller MH, Tennant GB, Campbell H. Double-blind randomized controlled trial of folate treatment before conception to prevent recurrence of neural tube defects. BMJ 282:1509-1511, 1981 33. Leslie EJ, Marazita M. Genetics of cleft lip and palate. Am J Med Genet C Semin Med Genet. 2013 Nov;163C (4):246-58. 34. Little J, Gilmour M, Mossey PA et al. Folate and clefts of the lip and palate – a UK- based case-control study: Part I: Dietary and supplemental folate. Cleft Palate Craniofac J 45:420-427, 2008 35. Loffredo LC, Souza JM, Freitas JA, Mossey PA. Oral clefts and vitamin supplementation. Cleft Palate-Craniofac J 38:76-83, 2001 36. Marcenes W, Kassebaum NJ, Bernab_e E et al (2013). Global burden of oral conditions in 1990-2010: a systematic analysis. J Dent Res 92: 592–597.

164 10th World Cleft Lip, Palate & Craniofacial Congress 37. Mastroiacovo P, Robert Gnansia E, Castilla EE. IDCFA. International Centre on Birth Defects. 2005 38. Meltzer, H.J. Congenital anomalies due to attempted abortion with 4-aminopteroylglutamic acid. J. Am. Med. Assoc. 1956, 161, 1253–1254. 39. Mossey PA, Shaw WC, Munger RG, Murray JC, Murthy J, Little J (2011). Global oral health inequalities: challenges in the prevention and management of orofacial clefts and potential solutions. Adv Dent Res 23: 247–258. 40. MRC Vitamin Study Research Group. Prevention of neural tube defects: results of the Medical Research Council Vitamin Study. Lancet 338:131-137, 1991 41. Murray CJ, Vos T, Lozano R et al (2012). Disability-adjusted life years (DALYs) for 291 diseases and injuries in 21 regions, 1990-2010: a systematic analysis for the Global Burden of Disease Study 2010. Lancet 380: 2197–2223. 42. Nelson, M.M. Mammalian Fetal Development and Antimetabolities. In Antimetabolites and Cancer; Rhoads, E.P., Ed.; American Association for the Advancement of Science Monograph: Washington, DC, USA, 1955; pp. 61–79. 43. Peer LA, Strean LP, Walker JC, Bernhard WG, Peck GC. Study of 400 pregnancies with birth of cleft lip-palate infants: protective effect of folic acid and vitamin B6 therapy. Plast Reconstr Surg 22:422-429, 1958 44. Peer LA, Gordon HW, Bernhard WG. Experimental production of congenital deformities and their possible prevention in man. J Int Coll Surg 39:23-35, 1963 45. Peer LA, Gordon HW, Bernhard WG. Effects of vitamins on human teratology. Plast Reconstr Surg 34:358-361, 1964 46. Rooij van IA, Vermeij-Keers C, Kluijtmans LA et al. Does the interaction between maternal folate intake and the methylenetetrahydrofolate reductase polymorphisms affect the risk of cleft lip with or without cleft palate? Am J Epidemiol 157:583-591, 2003 47. Schubert J, Schmidt R, Raupach HW. New findings explaining the mode of action in prevention of facial clefting and first clinical experience. J Craniomaxillofac Surg 18:343- 347, 1990 48. Shaw GM, Lammer EJ, Wasserman CR, O’Malley CD, Tolarova MM. Risk of orofacial clefts in children born to women using multivitamins containing folic acid periconceptionally. Lancet 346:393-396, 1995 49. Shaw GM, Wasserman CR, Lammer EJ, O’Malley CD, Murray JC, Basart AM, and Tolarova MM. Orofacial clefts, parental cigarette smoking, and transforming growth factor-alpha gene variants. Am J Hum Genet, 58; 551-561, 1996 165 10th World Cleft Lip, Palate & Craniofacial Congress 50. Smithells RW, Sheppard S, Shorah CJ, Seller MJ, Nevin NC, Harris R, Read AP, Fielding DW. Apparent prevention of neural tube defects by a periconceptional vitamin supplementation. Arch Dis Child 56:911-918, 1981 51. Thiersch, J.B. Therapeutic abortions with a folic acid antagonist, 4-aminopteroylglutamic acid (4-amino PGA) administered by the oral route. Am. J. Obstet. Gynecol. 1952, 3, 1298–1304 52. Tolarova MM. Periconceptional supplementation with vitamins and folic acid to prevent recurrence of cleft lip. Lancet (July 24, 1982), II/8291, p. 217. 1982a 53. Tolarova MM. Supplementation en vitamines et en acide folique dans la periode periconceptionelle en prevention des recidives de fissure labiale. Le Journal International de Medicine, Gynecologie Mondiale. Suppl 34, pp. 60-61, 1982b 54. Tolarova MM. Spontaneous abortions and facial clefts. Letter to Editors. Clinical Genetics; 1984: 26:77-80 55. Tolarova MM. Cleft Lip and Palate in Man - Epidemiology, Genetics and Prevention. DrSc Thesis. (in Czech). Czechoslovak Academy of Sciences, Prague 1988 56. Tolarova MM. Primary prevention of cleft lip and palate. Department of Dental Research University of Chapel Hill, June 6,1989 57. Tolarova MM. Genetics, gene carriers, and environment. In: Bader JD ed. Risk Assessment in Dentistry. Chapel Hill: Univ. of North Carolina Dental Ecology, 1990a, 116-148 58. Tolarova M. Genetic findings in cleft lip and palate in a Czech population. In: Multidisciplinary Management of Cleft Lip and Palate. J Bardach and HL Morris eds. WB Saunders, pp 113-121, New York 1990b 59. Tolarova MM. Prevention of cleft lip and palate in humans: Results and proposal for a multicenter clinical trial. – HFSP Craniofacial Morphogenetics Workshop, Iowa City, April 1993 60. Tolarova MM, Harris J. Reduced recurrence of orofacial clefts after periconceptional supplementation with high-dose Folic Acid and multivitamins. Teratology 51:71-78, 1995 61. Tolarova MM, Cervenka J (1998a) Classification and birth prevalence of orofacial clefts. Amer. J Med. Genet, 75:126-137 62. Tolarova MM: Cleft lip and palate among Hispanics in California (1998b). Biomedicina 2, 2:65-72

166 10th World Cleft Lip, Palate & Craniofacial Congress 63. Tolarova MM, Mosby T, Capozzi, Tolar M. Thirty years of studies, experience, and nuts and bolts on our journey toward primary prevention of cleft lip and palate. In: Management of Cleft & Craniofacial Deformities. Current Techniques, Research & Future directions. Proceeding of 7th Biennial World Cleft Lip and Palate Congress, 2012 64. Tolarova MM. Genetic Updates Related to Craniofacial Anomalies. Symposium of Emirates Society of Orthodontics in Dubai, UE, November 2014. Keynote lecture. 65. Tolarova MM. Tolarova, MM. Pediatric cleft lip and palate. MedScape November 2015. E-Medicine Journal Pediatrics: Surgery - Genetics. http://emedicine.medscape.com/ article/995535-overview 66. Wald NJ. Folic acid and prevention of neural tube defects. In Keen CL, Bendich A, Whillhite CC (ed). Maternal Nutrition and Pregnancy Outcome. Ann NY Acad Sci 6/8:112-29, 1993 67. Wald NJ. Folic acid and neural tube defects: the current evidence and implications for prevention. In: Bock G, Marsh J (eds.) Ciba Foundation Symposium Nr. 181: Neural Tube Defects. Chichester, pp.192-211, Wiley 1994 68. Warkany J. Prevention of congenital malformations. Teratology 23:175-189, 1981 69. Warkany J, Nelson RS. Appearance of skeletal abnormalities in the offspring of rats reared on a deficient diet. Science 92:383-384, 1940 70. Warkany J, Shraffenberg E. Congenital malformations induced in rats by maternal nutritional deficiency. V. Effects of a purified diet lacking riboflavin. Proc Soc Biol Led 54:92-94, 1943 71. Warkany, J.; Beaudry, P.H.; Hornstein, S. Attempted abortion with 4-aminopteroylglutamic acid (aminopterin): Malformations of the child. Am. J. Dis. Child. 1959, 97, 274–281. 72. Wehby GL, Murray JC. Folic acid and orofacial clefts: a review of the evidence. Oral Diseases 16: 11-19, 2010 73. Werler MM, Hayes C, Louik C, Shapiro S, Mitchell AA. Multivitamin supplementation and risk of birth defects. Am J Epidemiol 150:675-682, 2007 74. Wilcox AJ, Lie RT, Solvoll K et al. Folic acid supplements and risk of facial clefts: national population based case-control study. BMJ 334:464, 2007 75. Womersley J, Stone DH (1987). Epidemiology of facial clefts. Arch Dis Child 62:717-720.

167 10th World Cleft Lip, Palate & Craniofacial Congress

Hemifacial Hypertrophy – A Case Report

A. Thangavelu, R. Murugan1, V.N. Kavitha Devi2 Head, Department Of Oral And Maxillo Facial Surgery, R M D C & H , Annamalai University, 2Final Year Post Graduate, Rmdch, Annamalai University

Abstract

Hemifacial hyperplasia is a rare developmental anomaly exhibiting asymmetric growth of one or more parts of body. It is characterized by hyperplasia of tissues rather than a hypertrophy, so it is better to call it hyperplasia rather than hypertrophy. It involves the hard and soft tissues of the face. The cause is unknown, although several predisposing factors have been described. Thus in this present article a case report of a 14-year-old girl with hemifacial hyperplasia is presented to highlight the clinical and radio-imaging findings.Keywords

IntroductionHemifacial hypertrophy, Facial asymmentry, developmental anomaly

Hemifacial hyperplasia (HFH), described initially by Merckel J in 1822 and Wagner in 1839, is a sporadic congenital[1] condition. HFH is a segmental form of congenital hemihyperplasia . This condition is also known as softhemihypertrophy, tissues, bones, congenitaland teeth hemihypertrophy, hemimacrosomia, or partial gigantism; the classic presentation[2] is a unilateral overgrowth of the orofacial . The right side of the face is affected more often than the left side. HFH is more common[3] in men than in women and in white peopleof the body than in. other racial groups . It may be associated with other conditions, such as acromegaly[4] and pituitary gigantism, or with hypertrophy of other parts

Many theories have been proposed on the cause of hemifacial hyperplasia, including hormonal imbalances, diseases involving the neural system, vascular conditions (such as hemangiomas and arteriovenous malformations), abnormalities (such as lymphangioma, incomplete twinning, abnormal intrauterine environment, somatic mutations, and central nervous system lesions),168 mechanical influences, and congenital syphilis. Thus, many authors 10th World Cleft Lip, Palate & Craniofacial Congress malformations consider the development[4] of prenatal teeth to be a good indication of future . In patients affected by hemi facial hyperplasia, asymmetry afteris more birth marked than in normal individuals. Most of the time, this disorder is congenital [5]but few cases have been recorded where the condition developed . Thus this report describes a rare case of hemifacial hyperplasia on the right side of the face of a 14- year-old girl who presented with unilateral facialCase reportenlargement.

A 14-year-old girl was referred to Rajah Muthaih Dental College and Hospital, Chidambaram, Tamil Nadu with the chief complaint of restricted asymmetry.mouth from pastThe onemother year also [Figure reported 1]. The that girl’s she father neither reported had problems that she was during the first of the two siblings born to him without any previous family history of facial pregnancy nor during delivery. No significant postnatal illness was reported. The developmental milestones were normal. The physical examination revealed werea healthy, in normal active limit. and mentally active girl. General examination showed, patient was having normal gait, average built and moderately nourished. All vital signs

Figure 1: Female patient presented with unilateral facial enlargement with pre-operative mouth opening of 1 finger breadth

Patient was apparently normal before 6 years after which she developed a gradual decrease in mouth opening that progressed to attain the present state. She gave a history of ulcers in right side of buccal mucosa for which she visited a private clinic, where extraction of lower 32 to 45 was done before 1 year. Following extraction, she developed significant reduction in mouth opening (one finger breadth). [Figure 1] 169 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 2: Pre-operative OPG of the patient

On extra oral examination facial asymmetry was present on right side (diffuse swelling was present in right lower third of face). The soft tissues of the right cheek and the lip were thick and lip was incompetent. There was also an obliteration of lower buccal vestibule from 43-47 region. The region is firm and non-tender on palpation. Right and left submandibular lymph nodes were firm, mobile and tender. On intra oral examination teeth erupted in maxilla were 7,6,5,4,3,2,1/1,2,3,4,5,6,7 and in the mandible 7,6,/,3,4,5,6,7. On palpation palpable thick fibrotic tissues present in right buccal mucosa. The mouth opening from upper central incisor to lower alveolar ridge is 14 mm, there was grade III mobile teeth present from 21-22 Other striking features were the deviation of the maxillary midline. The radiograph confirmed the clinical findings.MRI findings OPG showed,view showed that generalized periodontitis. [ Figure 2]

bo 01. Muscle hypertrophy with fatty replacement noted in the right masseter, th medial and lateral pterygoids, buccinators, mylohyoid, anterior & posteriorDiffuse soft belly tissue of digastrics proliferation and zygomaticnoted in the major right muscles. side with predominant 02. fatty replacement. 03. Mild osseous hypertrophy of the right half of the mandible. 04. Mild shrinkage of the right parotid gland with relative increase in the right parapharyngeal space. 05. Right Sternocleidomast oid and temporalis muscle appear normal. [Figure 3] 170 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 3: MRI scan showing muscle hypertrophy on right side of the face. Discussion

HFH may be associated with deformities of the skeletal system, including macrodactyly,[1] polydactyly, syndactyly, scoliosis, tilting of the pelvis, and clubfoot . Central nervous system defects may include cerebral enlargement, epilepsy, strabismus, and mental retardation in 15-20%[6] of patients, and in some cases dilatation of the pupils on the affected side . Genitourinary system disorders, such as hypospadias,[7] cryptorchidism, and medullary sponge kidney, are also noted occasionally . Our patient had the characteristic clinical features of HFH, such as unilateral overgrowth of the orofacial soft tissues. Imaging features of HFH had shown an alteration in dental development with earlier dental maturity on the affected side, early eruption,[8] and idiopathic root resorption have been described in past literature . But panoramic and periapical radiographs of the present patient showed excessive root resorption with chronic periodontitis. HFH had a higher prevalence among males than in women and more commonly involves the right side of the face [3,9,10]. In keeping with this, the present case was a girl, and the right side of the face was affected. These alsoasymmetries well reported are often by many noted author at birth, . but in this case it is not noted by the parents. Precocious eruption and premature [11,12] development of involved teeth are

When evaluating a patient suspected to have HFH, numerous entities have to be considered in the differential diagnosis. Some of them where congenital causes of cranial asymmetry which includes a variety of syndromes such as Beckwith- Weidemann, Proteus, McCune-Albright, epidermal naevus, Klippel-Trenaunay and Maffucci syndromes. However, all of these forementioned syndromes have distinguishing features other than body asymmetry. Other conditions171 10th World Cleft Lip, Palate & Craniofacial Congress

that manifest at an early age such as neurofibromatosis, hyperpituitarism[13] and infiltrating lipomatosis are largely bilateral and widespread . Acquired causes of cranial asymmetry include Romberg syndrome, vascular anomalies and fibro-osseous lesions. Romberg syndrome is characterised by an acquired unilateral[14] underdevelopment, with muscle weakness and neurological deficit . Vascular anomalies (e.g. haemangioma) and multiple arteriovenous aneurysms of the bone are distinguished by their characteristic clinical and investigatory (e.g. Doppler[14] ultrasonography, magnetic resonance imaging and angiography) findings . Fibro-osseous lesions (e.g. fibrous dysplasia, Paget’s disease and ossifying fibroma) and other bony tumours usually[15] have no soft tissue component and may have serum chemistry changes . But our patient showedManagement no other systemic or syndromic alterations.

Generally, treatment of HFH is for cosmetic purposes. Emotional support and counselling during the developing years is important in order to help the individual cope, as reconstructive procedures such as soft tissue debulking, face lifts, osteotomies and/or orthognathic[9] surgery can only be planned andbeen performedreported in when the literature. growth ceases. Other options including facelifts and orthognathic surgical procedures[16] in conjunction with orthodontic therapy have

HFH is generally associated with a good prognosis and literature also revealed no formal reports of malignant degeneration. The asymmetry usually remains constant up to the end of adolescence and the condition stabilizes thereafter [1]. Thus in the present case treatment was done to improve cosmetic and functional purpose, so condylectomy, coronoidectomy and release of fibrotic tissues of right buccal mucosa was done and reconstruction of the defect was done using buccal pad of fat [ figure 4]. Post operatively mouth opening was 35 mm from upper alveolar ridge to lower ridge [ figure 5]. After surgery to improve patient aesthetics, we rehabilitate the patient with removable partial denture. Post-operative mouth opening with removable partial denture was 20mm from upper incisor to lower incisor. [figure 6]

Figure 4: Post-operative OPG of the patient

172 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 5: Post-operative mouth opening of 35mm

Figure 6: Post-operative mouth opening with partial denture of 20mm Conclusion

Thus in this case primarily coronoidotomy, condylectomy and release of fibrotic tissues in right buccal mucosa was done to improve the mouth opening. Preoperatively mouth opening was one finger breadth after primary surgery mouth opening of 35mm was achieved. In the second phase we planned rehabilitation of patient using implant prosthesis in both arches to improve patientReference aesthetics. 01. Pollock RA, Newman MH, Burdi AR, Condit DP. Congenital hemifacial hyperplasia: an embryologic hypothesis and case report. Cleft Palate J 1985;22, 173-184. 02. Islam MN, Bhattacharyya I, Ojha J, Bober K, Cohen DM, Green JG. Comparison between true and partial hemifacial hypertrophy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:501-509. 173 10th World Cleft Lip, Palate & Craniofacial Congress 03. Rowe NH. Hemifacial hypertrophy. Review of the literature and addition of four cases. Oral Surg Oral Med Oral Pathol 1962;15:572-587. 04. Bergman JA. Primary hemifacial hypertrophy. Review and report of a case. Arch Otolaryngol 1973; 97:490-494. 05. Miranda RT, Barros LM, Santos LA, Bonan PR, Martelli H Jr. Clinical and imaging features in a patient with hemifacial hyperplasia. J Oral Sci 2010;52(3):509-512. 06. Hayashi S, Tomioka T, Aoki H, Nakakuki K, Mekaru K. Hemifacial hypertrophy. Report of two cases. Oral Surg Oral Med Oral Pathol 1973; 35:750-761. 07. Eisenberg RL, Pfister RC. Medullary sponge kidney associated with congenital hemihypertrophy (asymmetry). A case report and survey of the literature. Am J Roentgenol Radium Ther Nucl Med 1972; 116:773-777. 08. Yoshimoto H, Yano H, Kobayashi K, Hirano A, Motomura K, Ohtsuru A, Yamashita S, Fujii T. Increased proliferative activity of osteoblasts in congenital hemifacial hypertrophy. Plast Reconstr Surg 1998;102, 1605-1610. 09. Neville B, Damm D, Allen C, Bouquot J. Developmental defects of oral and maxillofacial region. In Neville et al oral and maxillofacial pathology. 2nd Edition. Philadelphia, 2002. Pg.37-38. 10. Austin RD, Srivastava KC, Pranavadhyani G, Shrivastava D. A rare clinical case of true hemifacial hyperplasia. Singapore Med J 2013; 54(8): e160-e163. 11. Lawoyin JO, Daramola JO, Lawoyin DO. Congenital hemifacial hypertrophy. Report of two cases. Oral Surg Oral Med Oral Pathol 1989; 68:27-30. 12. Horswell BB, Holmes AD, Barnett JS, Hookey SR. Primary hemi- hypertrophy of the face: review and report of two cases. J Oral Maxillofac Surg 1987; 45:217-22. 13. Sapp JP, Eversole LR, Wysocki GP. Contemporary Oral and Maxillofacial Pathology. St Louis: Mosby, 2004. 14. Mazzeo N, Fisher JG, Mayer MH, Mathieu GP. Progressive hemifacial atrophy (Parry- Romberg syndrome). Case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 79:30-5. 15. Regezi JA, Sciubba JJ. Oral Pathology: Clinical Pathologic Correlations. 4th ed. Philadelphia: Saunders, 2003. 16. Hall HD. An improved method for treatment of facial asymmetry secondary to jaw deformity. J Oral Maxillofac Surg 1984;42:673-679.

174 10th World Cleft Lip, Palate & Craniofacial Congress

3D planning in craniomaxillofacial surgery

Josep Rubio-Palau1, Alejandra Prieto-Gundin2, Asteria Albert Cazalla3, Miguel Bejarano Serrano3, Gemma Garcia Fructuoso4, Francisco Parri Ferrandis3, Alejandro Rivera Baró5 Maxillofacial Surgeon1, Paediatric Anaesthesiologist2, Paediatric Surgeon3, Neurosurgeon4, Orthodontist5, Maxillofacial Surgery, Department of Pediatric Surgery, Barcelona, Spain

ABSTRACT INTRODUCTION

3D planning in oral and maxillofacial surgery has become a standard in the planification of a variety of conditions such as dental implants and orthognathic surgery. By using custom made cutting and positioning guides the virtual surgery is exported to the operating room, increasing precision and improvingMATERIALS results. AND METHODS

We present our experience in the treatment of craniofacial deformities with 3D planning. Software to plan the different procedures has been selected for each case, depending on the procedure (Nobel Clinician, Kodak 3DS, Simplant O&O, Dolphin 3D, Timeus, Mimics and 3-Matic). The treatment protocol is exposed step-by-step from virtual planning, designCONCLUSIONS and printing of the cutting and positioning guides, to patients’ outcomes.

3D planning reduces the surgical time and allows predicting possible difficulties and complications. On the other hand, it increases preoperative planning time and needs a learning curve. The only drawback is the cost of the procedure. At present the additional preoperative work that can be justified because of surgical time reduction and more predictable results. In the future the cost and time investment will be reduced. 175 10th World Cleft Lip, Palate & Craniofacial Congress

3D planning is here to stay. It is already a fact in craniofacial surgery and theKEYWORDS investment is completely justified by the risk reduction and precise results.

Craniofacial Surgery; 3D planning; Virtual surgery; Craniosynostosis OrthognathicINTRODUCTION Surgery; Distraction.

3D planning is a routine technique for some procedures such as dental implants and orthognathic surgery. Its advantages include more precision, fewer complications and reduction of the operative time.[1, 2, 3] In the 1990’s the construction of stereolithographic models begun. They were used for evaluation and treatment planning of complex facial deformities. [4] A few years ago, surgical cutting and positioning guides were developed in order to treat a wider spectrum of maxillofacial deformities and conditions. Some patients undergo surgical treatment with suboptimal results despite well-planned operations by experienced surgeons. One of the reasons for a poor outcome is the surgeon’s reliance on 2-dimensional imaging for treatment planning of a 3-dimensional problem.[4] Virtual surgery, a surgical revolution,●● I could help avoid risks. Some of its multiple advantages are: ●● ncreased precision ●● Possibility to operate on the same patient endless times ●● Simulate different approaches and procedure types ●● Compare different vectors and hardware in distraction osteogenesis ●● Avoid damaging neurovascular structures or teeth ●● Reduce operative time and improve postoperative recovery ●● Reduce complications and reoperations More precise and predictable results Its main disadvantages are the cost of the software and 3D printing and the longer preoperative preparation and procedure planning. However, the cost tends to reduce and so does the planning time after the learning curve. On the other hand, the operative and hospitalization time is reduced.[5] Virtual presurgical planning may be complemented with intraoperative navigation.[4] Adding intraoperative CT/MRI imaging, the result can be 176 10th World Cleft Lip, Palate & Craniofacial Congress

future.checked, further increasing the accuracy of the procedure. Augmented reality with devices such as Google glasses could revolutionize surgery in the near MATERIALS AND METHODS

Our preoperative work-up consists of craniofacial CT-scan, facial and intraoral photographs, and dental casts. In order to increase the resolution of the oclusal surfaces, dental casts are scanned and fused to the CT scan. [6] Different softwares have been used depending on the condition. Virtual surgery was transfered to the operating room with surgical splints or cutting and positional guides. In selected cases 3D-models were printed in order to simulate the surgical procedure and the placement of the cutting guides and the hardware or distractors. Initial and follow-up photographs are taken to evaluateOrthognathic results. surgery Intra and postoperative complications have been recorded.

3D planning and the use of CAD-CAM splints are a routine procedure in orthognathic surgery, thanks to its multiple advantages. The software used in our institution is Dolphin 3D Surgery (Dolphin Imaging & Management Solutions. 9200 Eton Ave. Chatsworth, CA 91311 U.S.A.) which allows simulation of the procedure, positioning of the maxilla and mandible and print CAD-CAM

splints[6] [Figures 1-2]. This technology is especially relevant for the correction of facial asymmetries because it analyzes the transverse plane, which is one of the limitations of 2D cephalometry. Regarding orientation, 2D allows the measurement of pitch, but not of roll or yaw.[7] It also allows checking the need of prosthesis or grafts to achieve symmetry. This is especially important in severe asymmetries, such as hemifacial microsomia.

177 10th World Cleft Lip, Palate & Craniofacial Congress Distraction osteogenesis

Distraction is a useful procedure to lengthen facial bones. We use this technique in neonates with Pierre-Robin sequence (micrognatia, glossoptosis and respiratory distress[8]), older children with hemifacial microsomia and severe maxillary or mandibular hypoplasia that cannot be corrected with orthognathic surgery. In neonates we use two Molina uni-directional external distractors (KLS-Martin. Ludwigstaler Str. 132. D-78532 Tuttlingen, Germany) and two Kirschner needles.Osteotomies are performed through an intraoral approach using piezoelectric surgery device determine(VarioSurg3, the NSK. osteotomy Nakanishi path Inc. line Japan) and [Figure the position 3]. In these cases virtual planning is especially important to

of the needles, preserving dental germs, as well as to predict the position of the mandible that will enlarge the upper airway. No surgical splints or positioning guides are used in these patients, as there are no teeth to fix them. Simplant® O&O has been used for planning the osteotomies and reposition the bones. It allows studying the anatomy -distorted in most patients- and choosing the best osteotomy while avoiding damage to neurovascular

structures (i.e: inferior alveolar nerve), teeth and germs [Figures 4 and 5]. A sequel of cleft lip and palate early surgery is reduced maxillary growth, especially in bilateral cases, which can be treated with orthognathic surgery. When the sagittal discrepancy is higher than 10-12 mm it is difficult to advance the maxilla in a single stage, due to the long gap and the limited movement of the scarred178 soft tissues. In case of such a severe maxillary hypoplasia we are using 10th World Cleft Lip, Palate & Craniofacial Congress

the internal Zurich Pediatric Maxillary distractors (KLS-Martin. Ludwigstaler Str. 132. D-78532 Tuttlingen, Germany) which allows an advancement of the maxilla up to 20 mm. In these cases, and in mandibular distraction for hemifacial microsomia, the software we use is Timeus (Laboratorio Ortosan, Madrid, Spain) which adds the possibility to create 3D cutting and positioning guides to transfer virtual

planning to the operating room [Figures 6-8]. This is especially important in order to plan the desired position of the maxilla and vector of movement. It is possible to try different kinds of distractors to achieve what has been planned. This software is also useful to correct asymmetric patients by placing the distractors differently to work more on one side and center the midline. We use 3D printed models in these cases to simulate the LeFort I osteotomy withPremaxillary the cutting osteotomy guide and the distractors position with the positioning guide.

Premaxillary osteotomy is done only in a selected group of complete bilateral cleft patients who have an abnormal position of the premaxilla that cannot be corrected orthopedically because it protrudes or is overerupted with hanging and retroinclined upper incisors.[9] 179 10th World Cleft Lip, Palate & Craniofacial Congress

The work-up consists of dental casts, intraoral and facial photographies, CT-scan and virtually positioning of the premaxilla in the correct position, which is transfered to a surgical acrylic splint. [Figure 9] 3D planning is very useful andin thesethe design cases of the to osteotomy. decide the It bestalso approach (intraoral or endonasal[10])

provides information about possible interferences and the need of a second osteotomy to achieve the desired Rhinoplasty torque and position of the premaxilla. single or double osteotomies and simulate the mobilization of the bones in Severe cases of nasal bone asymmetry can be virtually planned by doing order to achieve the best possible result. The software used is Simplant® O&O (Materialise Dental/Dentsply Implants, Mölndal, Sweden). Although it is not possible to predict the soft tissues response, 3D planning shows the best way to

perform180 the nasal osteotomies and septoplasty. [Figures 10-12] 10th World Cleft Lip, Palate & Craniofacial Congress Craniofacial surgery

Complex craniofacial malformations and craniosynostosis can be treated successfully with this technique. Simulating the final result and printing the cutting and positioning guides are great aids to achieve a good, consistent result. The proposed surgery can be based on quantitative anthropometric guides, a superimposed normal side or a mirrored normal side.[11] Operative time and bleeding is reduced, which is especially important in young patients, allowing a better recovery with a shorter hospitalization period. The software used has been Timeus (Laboratorio Ortosan, Madrid. Spain) and 3D-models have been very useful to simulate the various steps of the surgical procedure [Figures 13-15]. In craniosynostosis two different

softwares have been used: 3-matic (Materialise. Technologielaan 15, 3001 Leuven, Belgium) for the design and planification, and Mimics (Materialise. Technologielaan 15, 3001 Leuven, Belgium) for the segmentation of the skulls. Cutting and positioning guides have also been used to reposition the bones in orderSoft tissues to normalize the anatomy of the skull.

The behavior of the soft tissues after a bone movement is very unpredictable because of its different components and status, so the simulation181 10th World Cleft Lip, Palate & Craniofacial Congress

is not a good predictor of soft tissue outcome. Depending on the skin, fat tissue or muscle the response can be different in every patient; the dynamics and mimics of the patient’s face have an influence as well. We have used the softwares Simplant® O&O and Dolphin 3D surgery for this purpose, as well as the 3D plastic surgery simulator Crisalix (Lausanne, 1015. Switzerland). Simulation of rhinoplasty and genioplasty can be done, as well as facelift and lip surgery, among others [Figure 16]. This commercial

software focuses in showing the patient’s possible results, like 3D Photoshop. toIt canthe beoperating very helpful theater. to increase surgeon-patient communication and to show the patient what can be expected, but there is no way to transfer the simulation DISCUSSION

Virtual surgery offers indisputable advantages. It is a big step forward in the treatment of craniomaxillofacial conditions. A step we don’t want to step back from, as one feels that an incalculable amount of information is lost with 2D. Since 2010 we have been planning 3D all the orthognathic surgery patients and some other conditions. Preoperative planning is time consuming and has a learning curve. Nevertheless, these inconveniences are widely surpassed by its advantages: enhanced visualization, precise information, better tools for instruction and accurate documentation5. Little research has been done on virtual planning of midfacial distraction since182 the description of Gateno in 2003.[12] Virtual surgery simplifies the 10th World Cleft Lip, Palate & Craniofacial Congress

procedure and allows achieving good results in very complex patients. One example could be maxillary distraction in bilateral cleft patients with severe maxillary hypoplasia. In such patients we have treated discrepancies up to 20mm from upper incisor tip to lower incisor tip, the final position of the maxilla reaching as far as had been planned. The use of cutting and positioning guides reduces considerably the operation time and the risk of errors, since the position and the vector are transferred to the guides, and from these to the patient, as planned. If an error occurs in the final position, it will happen before in the preoperative planning. Virtual surgery helps improve accurate diagnosis, optimise treatment planning and transfer of the planning to the patient.[5,13] Virtual planning has helped decide the approach or the osteotomy line in other surgical procedures, even when no cutting or positioning guides were made. This happens for instance in neonatal distraction with external distractors for Pierre Robin sequence, while Steinbacher has treated these patients using cutting and positioning guides and internal distractors.[11] Postoperative CT-scans offer a good quality control of the procedure, comparing the 3D planning with the final surgical outcome.[5] This could be a useful research line to validate the accuracy of virtual surgery in other craniomaxillofacial procedures. Regarding the soft tissues, only approximate results can be expected[5]; virtual surgery may be helpful to simulate the result after surgery, especially after rhinoplasty, and the position of the lips after orthognathic surgery, but has a lower precision when compared to the bones. A useful advantage of 3D planning is that it increases the knowledge of the anatomy of each particular patient; this is because the real surgical intervention is not the first time the surgeon operates on this particular patient -the procedure has been performed several times before on “the same” virtual patient and with 3D printed models-. Virtual surgery is also a very powerful tutorial and communication tool to present, train and document cases and proceduresCONCLUSION for colleagues, trainees and patients.

The purpose of this article is to show new applications of 3D planning in craniomaxillofacial surgery. This technology is here to stay. Additional preoperative time and costs can be justified by highly precise bone movements and more predictable results, while reducing surgical time and postoperative complications. 183 10th World Cleft Lip, Palate & Craniofacial Congress

On the other hand, it increases preoperative planning time and needs a learningREFERENCES curve. 01. Gateno J, Xia JJ, Teichgraeber JF, Christensen AM, Lemoine JJ, Liebschner MA, Gliddon MJ, Briggs ME.Clinical feasibility of computer-aided surgical simulation (CASS) in the treatment of complex cranio-maxillofacial deformities. J Oral Maxillofac Surg 2007;65:728-34. 02. Swennen GR, Mollemans W, Schutyser F. Three-dimensional treatment planning of orthognathic surgery in the era of virtual imaging. J Oral Maxillofac Surg 2009;67:2080- 92. 03. Aboul-Hosn Centenero S, Hernández-Alfaro F. 3D planning in orthognathic surgery: CAD/ CAM surgical splints and prediction of the soft and hard tissues results - our experience in 16 cases. J Craniomaxillofac Surg 2012;40:162-8. 04. Bell RB. Computer planning and intraoperative navigation in cranio-maxillofacial surgery. Oral Maxillofac Surg Clin North Am 2010;22:135-56. 05. Adolphs N, Haberl EJ, Liu W, Keeve E, Menneking H, Hoffmeister B. Virtual planning for craniomaxillofacial surgery--7 years of experience. J Craniomaxillofac Surg 2014;42:e289- 95. 06. Rubio-Palau J, Hueto-Madrid JA, González-Lagunas J. Planificación 3D en cirugía ortognática. Rev Esp Ortod 2012;42:17-21. 07. Gateno J, Xia JJ, Teichgraeber JF. A New Three-Dimensional Cephalometric Analysis for Orthognathic Surgery. J Oral Maxillofac Surg 2011; 69: 606–622. 08. Sesenna E, Magri A, Magnani C, Brevi B, Anghinoni M. Mandibular distraction in neonates: indications, technique, results. Ital J Pediatr 2012; 38: 7. 09. Jensen J, Kuseler A, Klit Pedersen T, Norholt S. Premaxillary osteotomy and bone grafting for secondary BCLP repair—long-term evaluation of growth. J Oral Maxillofac Surg 2011;69:e16–e17. 10. Ferrer Fuertes A, García Díez E, Rivera Baró A, Sieira Gil R, Cho-Lee GY, Martí Pagés C, Rubio Palau J. Endonasal approach in premaxilla osteotomy of complete bilateral cleft lip palate. Int J of Oral Maxillofac Surg 2013;42:10, 1197. 11. Steinbacher DM. Three-Dimensional Analysis and Surgical Planning in Craniomaxillofacial Surgery. J Oral Maxillofac Surg 2015;73:S40-56. 12. Gateno J, Teichgraeber JF, Xia JJ. Three-dimensional surgical planning for maxillary and midface distraction osteogenesis. J Craniofac Surg 2003;14:833-9. 184 10th World Cleft Lip, Palate & Craniofacial Congress 13. Markiewicz MR, Bell RB. Modern concepts in computer-assisted craniomaxillofacial reconstruction. Curr Opin Otolaryngol Head Neck Surg 2011;19:295-301. FIGURE LEGENDS

Figure 1: 3D planning of a bimaxillary surgery + genioplasty. Preoperative lateral teleradiograph, 3D-planning and postoperative result. Figure 2: Pre and postoperative lateral views of the patient in Figure 1. Figure 3: Mandibular distraction in a neonate affected with Pierre Robin sequence. Figure 4: 3D planning of bilateral mandibular osteotomies for distraction. Figure 5: Virtual surgery for mandibular distraction. Figure 6: Virtual surgery for maxillary distraction. a) Initial situation. b) Lefort I osteotomy using cutting guides. c) LeFort I and maxillary advancement using Zurich internal distractors and positioning guides. Figure 7: Lateral teleradiographs for maxillary distraction. a) Initial situation. b) Beginning of maxillary distraction. c) End of maxillary distraction. d) Final situation after distractors removal, bilateral sagittal split, genioplasty and rhinoplasty, all in one procedure. Note the change of the nasal tip after distraction. Figure 8: Preoperative (a), after maxillary distraction (b) and after removal of maxillary distractors, BSSO, genioplasty and rhinoplasty (c) postoperative lateral views of the patient in Figure 6 and 7. Figure 9: Premaxilla osteotomy. a) Lateral teleradiograph before surgery. b) Preoperative CT-scan. c) Virtual osteotomy and reposition of the premaxilla. d) Postoperative lateral cephalometry. Figure 10: Virtual planning for rhinoplasty. Figure 11: Open septorhinoplasty of a crooked nose in a Turner syndrome patient with cleft palate. a) 3D planning of the first left osteotomy. b) 3D planning of the second left osteotomy and segment to be removed in order to symmetrize the nasal bones. c) First left osteotomy performed with piezoelectric device. d) Second left osteotomy. e) Removal of the left nasal bone segment. rhinoplasty. Figure 12: Preoperative (a) and postoperative (b) frontal images after

185 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 13: CT scan of a frontal defect causing associated with craniofacial cleft. a) Preoperative view. b) Virtual simmulation for the reconstruction of the frontal bone. c) Postoperative view. Figure 14: Cutting and positioning guides for frontal reconstruction of a frontal defect associated with craniofacial cleft. the same patient. Figure 15: Intraoperative view of the cutting guides for cranial osteotomies in

Figure 16: 3D simulation of orthognathic surgery and rhinoplasty using Crisalix. a) Preoperative view. b) Simulation of the surgical procedure. c) Final result.

186 10th World Cleft Lip, Palate & Craniofacial Congress

Distraction Osteogenesis versus Traditional Orthognathic Surgery in the Management of Craniofacial Deformities

G. E. Ghali Chancellor & Dean, LSU Health Sciences Center, Gamble Professor & Chairman, Oral & Maxillofacial Surgery, Shreveport, Louisiana, USA

ABSTRACT

Relative to craniofacial surgery distraction vs. non-distraction, a systematic approach to the evaluation of patients with craniomaxillofacial disorders will be presented. As part of this presentation, those conditions in the upper, middle and lower one-third of the craniofacial skeleton will be analyzed relative to indications for distraction vs. non-distraction in the management of these deformities. Various distraction and non-distraction techniques will be described in detail and contrasted relative to traditional orthognathic surgery.

187 10th World Cleft Lip, Palate & Craniofacial Congress

Challenges in airway management in children with craniofacial abnormalities-Case Capsules

Dr.S.Ramesh Consultant Paediatric Anaesthesiologist, Chennai, India

Airway management for patients with craniofacial disorders poses many challenges. The anaesthesiologist must be familiar with thenormal bony and soft-tissue anatomy in the airway and how anatomyis altered by various congenital disorders.Anatomical abnormalities may affect only intubation, only airway management, or both. Another complicating factor in paediatric patients is how abnormalities change with growth. Airway abnormalities may improve, worsen, or remain the same as craniofacial structures mature Specific areas to assessinclude the oral cavity, anterior mandibular space, maxilla, temporomandibular joint and vertebral column. Congenital conditions that may alter normal anatomy and therefore anaesthetic management include cleft lip and palate with or without Pierre Robin syndrome,craniofacial dysostosis, mandibulofacial dysostosis/Treacher Collinssyndrome, hemifacial microsomia, Klippel-Feil syndrome, Beckwith-Wiedemann syndrome, trisomy 21/Down’s syndrome, Freeman–Sheldon/whistling face syndrome/craniocarpotarsal dysplasia, fibrodysplasiaossificans progressiva, mucopolysaccharidosis and vascular malformations. The lecture will be highlighting the various techniques and the devices used to manage the difficult airway in various craniofacial disorders which I came across during the last 25 years of my paediatric anaesthesia practice.

188 10th World Cleft Lip, Palate & Craniofacial Congress

Evidence based chair-side preventive dentistry for cleft patients

Vijay Mathur Additional Professor, Pedodontics and Preventive Dentistry, Centre for Dental Education and Research, WHO Collaborating Centre for Oral Health Promotion, All India Institute of Medical Sciences,, New Delhi, India

Abstract

The children with CLP suffer from dental caries and hypoplastic teeth extensively. This leads to effect on non only their mastication, esthetics and speech development but also on overall development and self-esteem. Most of the time they are being treated by the plastic surgeons. Oral surgeons and pediatric surgeons during early age and not much emphasis is given to prevention of dental caries and control of further damage to the hypoplastic teeth. However, smaller steps taken by any professional for diet counseling, hygiene and chair side prevention can help in reducing the significant problem to the CLP children. This presentation will highlight the evidence of each preventive procedure and summarize the recommendations for prevention of dental caries in CLP children.

189 10th World Cleft Lip, Palate & Craniofacial Congress

A simple and novel splint to prevent reattachment of reconstructed auricle in microtia patients

Dr.C.J.Venkatakrishnan Consultant Maxillofacial Prosthodontist, Chennai, India

Abstract

Microtia a congenital anomaly of the ear can occur as an isolated birth defect or as part of a spectrum of anomalies or as a syndrome. Microtia is often associated with impaired hearing and or total loss of hearing. Such patients typically require treatment for surgical ear reconstruction and for hearing impairment. The reconstruction of a normal looking ear is one of the most challenging problems in plastic surgery. Maintenance of ear projection and post auricular sulcus in staged ear reconstruction in microtia is a trying problem. So also is the maintenance of the patency of the external auditory meatus following recanalization and meatoplasty. This case report describes a simple effective way of fabrication of ear splint prosthesis to prevent reattachment of reconstructed auricle in microtia patient.

190 10th World Cleft Lip, Palate & Craniofacial Congress

Images and Measurements During Speech Using a 320-Row Area Detector CT

Koichi Satoh Dept. of OMFS, Saiseikai Matsusaka General Hospital, Japan

Abstract

Background and Objective; The continuing advances in technology of CT provide a greater understanding of the human body. The purpose of this study is to evaluate dynamics of speech organs using a 320-row area detector CTDesign (ADCT).

This CT system has the 16cm wide area detector, and the velopharynx can be scanned within one rotation. The CT gantry was tilted back. The subjects sitting on the custom-made chair was behind the gantry and spoke a speech sample (/bampa/). The radiationCT images exposure were taken was with estimated 80kV and 10mA, and the individual

at 0.5 mSV. Four-dimensional dynamic CT images were reconstructed at 0.05 seconds intervals. The lips on the frontal view, the velopharyngeal function and the tongue on the sagittal plane, and the glottis on the axial plane were observed. The velopharyngeal volumeParticipants and the glottal area were measured. age and free of oropharyngeal abnormalities. The subjects were five normal adult females between 23 and 35 years of Results

The percentage of minimum to maximum of the velopharyngeal volume ranged from 43.9 to 67.4 %. The backward movement of tongue acted as a contributing factor to the change of pharyngeal volume. The percentage191 10th World Cleft Lip, Palate & Craniofacial Congress of minimum to maximum of the glottal area ranged from 13.4 to 60.0%. Measurements of the glottal area were uneven, but five subjects were divided into two groups. Measurements stayed the same during speaking /bampa/ in three subjects, measurements increased between the voiced sound and the voicelessConclusions sound in two subjects.

Images of all speech organs during speech can be taken, this is the strongest advantage of this CT. The multiple view images worked well to understand the mutual movements among speech organs. This study revealed that a 320-row ADCT has the full potential of studying the time series movements of speech organs. The application range of this CT can be extended to speech orders in patients with repaired cleft palate, postoperative malignant tumor, or brain infarction.

192 10th World Cleft Lip, Palate & Craniofacial Congress

Anatomical and Functional basis of the production of the Spoken sound. The impact of Middle ear status, in the Speech of a Cleft Palate child.

Balakrishnan D Professor of ENT & HOD Audiology Speech Language Pathology SRM Medical College, SRM University.

Abstract

Recent technological advancements in imaging, recording, acoustic analysis etc, has produced voluminous knowledge, in the field of phonetics and sound production. It is virtually impossible to learn all that. However, out of necessity, we have to learn. In this lecture, I will describe the production of speech sounds. More importantly, I will demystify the terminology. The grains and pearls of knowledge, in this field, will be presented to you. The descriptions, which do not have a direct impact to the actual practice, will be kept out. In general, this presentation, which will focus on the essentials only. Speech consists of a string of sounds. Each unit of sound is called a phone or a phoneme. The lungs produce air stream. The Vocal cords open to allow breathing. They close to cut the air stream into columns. With some phonemes, the vocal cords also vibrate. The phonemes, wherein the vocal cords vibrate are called ‘voiced’ sounds. The structures, situated above the vocal cords, dynamically rearrange themselves into differing configurations and produce distinctive phonemes. This activity is called ‘articulation’. The vocal tract comprises of the cords, pharynx, oral cavity and the nose. The phonemes, which exit from the vocal tract without any impediment i.e. without the tongue touching any other part of the vocal tract namely the lips, teeth or the roof of mouth, are called ‘vowels’. The consonant phoneme have a constriction at some of the vocal tract. The process of producing such constrictions is complex and is called articulatory mechanism. In a child with cleft, we will be concerned only with the consonants. 193 10th World Cleft Lip, Palate & Craniofacial Congress

When the air stream exits through the oral route, the phonemes are described as oral consonants’. In a similar vein, the sounds which escape through the nose are termed nasal consonants. Because, in a cleft child, by alsovirtue exit of throughthe ever the present nose. free way between the oral and the nasal cavities, all the sounds automatically get ‘nasalized’ i.e. part of the air in oral consonants

By just listening to the child speaking a few consonant phonemes, a discerning listener can assess the underlying derangement of structure and function. Several instruments are now available to assist the listener. In addition to speech defects, almost every child with cleft palate, will have Secretory otitis and suffer from its consequences. This will have a serious impact upon the hearing comprehension and upon restoration of clear speech. This must be deliberately looked for and be factored into the treatment protocol.

194 10th World Cleft Lip, Palate & Craniofacial Congress

Perceptual and Instrumental Assessment of Speech parameters in Cleft lip and palate.

Vedhasorubini K Asst. Prof. of Audiology & Speech Language Pathology, SRM Medical College, SRM University

Abstract

Human being communicates mainly by speech. The defining parameters of Speech are voice, articulation, fluency, resonance and prosody. Individuals with●● cleft lip & palate present with ●● Deviant speech characteristics involving articulation, voice and resonance swallowing Inadequate vegetative functions viz. sucking, chewing, biting and ●●

●● Nasal regurgitation, affecting nutrition ●● Middle ear problems due to velo pharyngeal incompetence, and Delayed language development in the case of children. The above issues prevent meaningful communication. Every person with a cleft palate, must have a detailed assessment, regardless whether they have undergone surgical repair or not. Such an assessment, enables the Speech therapist to set appropriate and realistic therapy targets for correction and restoration●● of intelligible speech. The methods employed are ●● History taking ●● Examination of the Oral peripheral mechanisms ●● Perceptual assessment (low tech technique) Instrumental assessment (relatively high tech and with speech software) Perceptual assessment is low tech and does not require any instruments. In practice, this method has been found to be sufficient and successful. In this activity, the skill and experience of the speech pathologist (SLP) counts a lot. Success depends on the listening and inferential skills of the SLP. Familiarity with standardized subjective test tools and algorithms would be essential. Word195 10th World Cleft Lip, Palate & Craniofacial Congress lists, which correspond to the place and manner of articulation are invaluable. They render the life of the SLP simpler. Instruments help in documentation. Instruments viz. video cine fluoroscopy, nasendoscopy, nasometer, speech recorders, and software e.g. Pratt, Dr Speech are employed. Reliable records of speech parameters can be obtained by skillful use of these techniques. Such records aid in monitoring the progress of the child, during the course of therapy. After the comprehensive assessment, as above, we should generate a report. This report shall contain a definite set of obligatory articulatory errors, compensatory articulatory errors, hypernasality, nasal air emissions theand diagnosis errors of will language be presented. acquisition. Additionally, wrongly learnt manners of speech production must be specifically identified. Case studies, exemplifying

196 10th World Cleft Lip, Palate & Craniofacial Congress

Management Strategies in Restoration of Speech and Language in Children

Usha A Dalvi Associate Prof. of Audiology & Speech Language Pathology, SRM Medical College, SRM University

Abstract

In patients with communication disorders due to cleft lip and palate, early intervention of communication disorders is a critical component. Occasionally, after surgery, a physiological or an anatomical deficit may remain. This will preclude clear speech. When compared to their normal peers, they may exhibit (i) articulation defects viz. substitutions, omissions and glottal stops, and (ii) resonance defects viz. hyper nasality, nasal emissions and hypo nasality (iii) defects in their receptive and expressive language, limited vocabulary and limited socio-communicative interactions The Speech Language Pathologist must be able to identify underlying specific articulatory, resonance and language defects and to provide early intervention. The speech therapist must make an effort to correct the errors and overcome any residual nasal leak (nasal emissions) by appropriate therapeutic techniques. The therapist must also intuitively decide and prognosticate success.●● The strategies used for restoring speech and language are the targeted phoneme, one phoneme at a time. This would be followed by Phoneme specific approach used for achieving the correct production of

●● words and sentences. Focused stimulation in which the child is presented with vocabulary ●● targeted at particular sound and stabilized with drills work.

monitor.Improving the Auditory discrimination of the child helps to become aware of his wrong speech production techniques and enables the child to self ●●

Modeling is another commonly used strategy for developing speech and language. In this, the Speech Language Pathologist (SLP) models the correct197 10th World Cleft Lip, Palate & Craniofacial Congress

●● speech and language behavior that the child has to learn. Stimulability is a strategy which uses multisensory stimulation (i.e.) a sound is taught using visual, auditory and tactile cue. This is also used in ●● reducing the nasal emissions and phoneme specific nasal emission. Oro-motor exercises like blowing, puffing the cheek, which even though not associated directly with speech movements, facilitate correct flow of breath stream for speech production and building up of intraoral breath pressure. For any of the therapy technique to be effective, it is very important program.to empower the parent or the significant other by teaching them few simple techniques or observing the therapy session and execute the home training

198 10th World Cleft Lip, Palate & Craniofacial Congress

Compensatory errors of Speech, assessment and management

Thamizharasan S, MSc (Audiology & Speech Language Pathology) Associate Prof. of Audiology & Speech Language Pathology, SRM Medical College, SRM University

Abstract

Well before any surgical intervention, the Speech Language Pathologist must be able to identify specific articulatory, resonance and language defects and to provide early intervention. The speech therapist must make an effort to correct the errors. The child might have learnt some trick movements to overcome feeding defect and speech defects. After surgical closure, the child may find it difficult to adjust to the newly restored anatomy, and continue to harbour speech defects. The speech therapist must be able to identify the specific speech defects, which might be the consequence of wrong learning by the child. Such children rewarding.need precise identification of the compensatory errors and substitution errors. Once the underlying error is recognized, the therapy will be eminently

In patients with communication disorders due to cleft lip and palate, early intervention of communication disorders is a critical component. Occasionally, after surgery, a physiological or an anatomical deficit may remain. This will preclude clear speech. When compared to their normal peers, they may exhibit (i) articulation defects viz. substitutions, omissions and glottal stops, (ii) resonance defects viz. hyper nasality, nasal emissions and hypo nasality (iii) defects in their receptive and expressive language, limited vocabulary and limited socio-communicative interactions. The Speech Language Pathologist must be able to identify underlying specific articulatory, resonance and language defects and to provide early intervention. The speech therapist must make an effort to correct the errors and overcome any residual nasal leak (nasal emissions) by appropriate therapeutic techniques. The therapist must also intuitively decide and prognosticate success. The strategies used for restoring speech and language are 199 10th World Cleft Lip, Palate & Craniofacial Congress ●● the targeted phoneme, one phoneme at a time. This would be followed by Phoneme specific approach used for achieving the correct production of

●● words and sentences. Focused stimulation in which the child is presented with vocabulary ●● targeted at particular sound and stabilized with drills work.

monitor.Improving the Auditory discrimination of the child helps to become aware of his wrong speech production techniques and enables the child to self ●●

Modeling is another commonly used strategy for developing speech and language. In this, the Speech Language Pathologist (SLP) models the correct ●● speech and language behavior that the child has to learn. Stimulability is a strategy which uses multisensory stimulation (i.e.) a sound is taught using visual, auditory and tactile cue. This is also used in ●● reducing the nasal emissions and phoneme specific nasal emission. Oro-motor exercises like blowing, puffing the cheek, which even though not associated directly with speech movements, facilitate correct flow of breath stream for speech production and building up of intraoral breath pressure. For any of the therapy technique to be effective, it is very important program.to empower the parent or the significant other by teaching them few simple techniques or observing the therapy session and execute the home training

200 10th World Cleft Lip, Palate & Craniofacial Congress

Feeding Assessment - Prosthetic appliances for feeding and for speech correction

Ambethkar Suprent Asst. Prof. of Audiology & Speech Language Pathology, SRM Medical College, SRM University

Abstract

Right from birth, the nutrition of all the children must be taken care of. Prosthetic devices are useful in preventing nasal regurgitation in a child with wouldpalatal notdefect. be feasible. Another set of appliances have been designed for correction of speech defects, also. They can be used in children, for whom surgical correction

In some individuals, some degree of velo pharyngeal insufficiency may persist even after surgical closure, due to a short velum. In certain other post- operative individuals, due to scar contracture in the velum, a velo pharyngeal incompetence i.e. a dynamic inadequacy of closure may be present. In these individuals, nasal regurgitation especially on liquid intake viz. water, juice and milk may occur. Further, nasal emissions and alterations in resonance may also occur. In this presentation, comprehensive information about the available prosthetic devices like speech bulb and other obdurators will be given. Additionally, behavioral modification techniques will have to be employed. The direct and indirect approaches to improve the speech intelligibility and swallowing functions

201 10th World Cleft Lip, Palate & Craniofacial Congress

Stressed parent and the anxious child

Julie Sandra Abel BSc (Speech Hearing Language) Clinical supervisor (Audiology & Speech Language Pathology), SRM Medical College, SRM University

Abstract

Parents of children with Cleft lip and palate harbour specific doubts and anxiety. Most feel guilty and and tend to blame themselves for the problem of the child. Many times, the extended family also, tend to blame the parents for the child’s disability. Most parents worry over the aesthetics of the child. They wonder how much of a normal life the child will have. They want to get over the issues, quickly. Will he be able to attend a normal school ? Whether he will have associated problems? team. AsTheir a Speech understanding language pathologist,and emotional counseling well being and is psycho important education to a better plays a pivotal role in the child’s holistic care. Parents are a part of the cleft care prognosis of the child. She must also encourage the parents to get involved in the therapy techniques. Psychological education: The parents are overwhelmed with feelings of anxiety, anger and denial. Because of this, they may fail to grasp the information given by us. This is likely to impede their active and cheerful participation, in the cleft care team. Formation of parent groups, can pay rich dividends, in this particular sphere. Feeding issues: The moment the child is born, the problem of feeding and nutrition arises. Will he be getting the adequate nutrition for his development? In the child already undergone repair, residual feeding issues can be taken care of, by temporary prosthetic appliances. Uch smaller (but, palpable) differences will improve the morale of the child and the parents. Behaviour therapy: Parents are also concerned about the emotional impact cleft lip and palate will have on the child. Whether he will be able to cope 202 10th World Cleft Lip, Palate & Craniofacial Congress

with the social stigma of looking different? How will it affect his self- esteem? They also worry over peer ridicule. Reward techniques: Every time, the child attains a targeted goal, the apositive disabled. outcomes must be reinforced. This, in turn, will build up the morale of the child. The speech therapist should focus on the child as a person and not as

Parents must also be provided with sustenance, as an integral member of the cleft care team. The parents will then continue the therapy at home, and reach the ultimate goal faster.

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The Secondary Rhynoplasty in Cleft Lip and Palate Patients

Domenico Scopeliti Authors: Scopelliti D., Amodeo G. Affiliations: U.O.C. Maxillo-Facial Department, San Filippo Neri Hospital VOipae Praatniaomn aS m52il,e 0 I0ta1l9y8 F Rouonmdea –ti oItna l–y Smile House Milan – ASL Roma1 - Rome Corresponding author: Amodeo Giulia, Via Giuseppe Martinotti 20, 00135, RomenKoesyew doerfodrsm: citleyf t lip and palate nose deformity; unilateral cleft nose deformity; bilateral cleft Abstract

Cleft lip and/or palate malformation is the most common congenital ,occurring in 1 in 500 to 1,000 live births (1). The treatment concept for CLCP requiresmultidisciplinary care to correct the anatomical changes inherent in the disease and to avoid the deforming consequences. If the primary surgery was done respecting the anatomical landmark and if each aspect was appropriately corrected and repaired, the majority of the most challenging secondary deformities seen in adolescence can be avoided. After an inadequate primary repair, the next most common cause of severe nasal deformities is multiple (2). It is universally accepted that correction of cleft lip nose deformity remains a formidable challenge for any cleft surgeon. The nose is a prominent part of the face, and hence a masterly executed cleft lip repair directs the beholders’ eyes from the deformed lip to the deformed nose. We report our experience in cleft lipKeywords: and palate nose secondary deformities.

cleft lip and palate nose deformity; unilateral cleft nose deformity; bilateralIntroduction cleft nose deformity

Deforming secondary malformations, in patients with cleft lip and palate, obviously affect 204 10th World Cleft Lip, Palate & Craniofacial Congress

each component of the nasal structure. Each defect may occur singly or in combination with others. In literature many authors like Ortiz Monasterio, Millard, Aiach (3, 4, 5, 6), have analyzed the anatomy of the nose in patients with cleft lip and palate proposing various reconstructive techniques depending on the extent of the damage and the basic defect. Anatomical structures are distorted by the disease and by the many procedures, which the patient has undergone, often resulting in more complex anatomical changes in a scar tissue tough to be corrected. The anatomical components have to be restored individually and in mutual anatomical respect in order to achieve the best harmonious result. The main unilateral cleft nose deformities are: tip asymmetry, nasal dorsum deviation, alar cartilages malposition, alar hypoplasia in the cleft side and septal deviation contralateral to the cleft. The main bilateral cleft nose deformities are: hypoplasia of all the structure on the median line, prolabium and premaxilla hypoplasia, short columella and deformities of alar cartilages. Materials and methods From 2000 to 2016 we analyzed 59 patients with secondary deformities due to primary treatment of cleft lip and palate. All the patients showed nose deformities often in association with upper lip ones and maxillo-mandibular discrepancy. To establish proper criteria of evaluation we divided the patients into four groups, considering the patient request and the objective needs to achieve a better result (Table 1). In the first we included the younger or adolescence patients in which the nasal surgery could be an interceptive option. We decided to exclude from this study this group because interceptive surgery could require further refinement or orthognathic surgery at the end of growth or later. The second group included patients who required other surgical procedures. Among this group we decided to divide the patients in two other groups depending on the necessity of orthognathic surgery or lipofilling. The third was dedicated to patient with nose-lip secondary deformities. The fourth included patients with several previous treatments of nose deformities in CLCP. In each patient were performed different procedures dependingDiscussion on the secondary cleft defects.

205 10th World Cleft Lip, Palate & Craniofacial Congress the primary surgery or are treated not following the standard treatment The patients in our study are supposedly not adequately treated during protocol used by the major centers of excellence (orthodontic treatment, speech therapy, orthognathic surgery at the end of the growth). These could be the reasons why each patient has a secondary or tertiary nasal deformities as a result of incomplete or unsuitable treatments. In our revision, except for the first group that included patients growing, in which we preferred to perform an interceptive surgical procedure as expressed by McComb (7, 8, 9), all the patients were submitted to a complete rhinoplasty, generally through an open approach. In selected cases we decided to perform this procedure concurrently with other ones like the orthognathic surgery. In case of patients that were previously treated the defects and the reconstructive options would be several. The nasal base is characterized by hypoplasia and retrusion, in this case the skeletal augmentation of the piriform aperture could be a treatment option. The ala and lower lateral cartilages would be collapsed, weakened or displaced. In these situations it is possible to perform an alar contour graft or a lateral crural strut graft or lateral crural transection and repositioning. The soft tissue triangle would be notched or retracted. The nasal tip would be underprojecting, with an acute columellar-labial angle, or without definition. The solutions would be several; it is possible to use a columellar strut graft, a spreader graft, an infratip lobular graft, to perform a caudal septal resection, an interdomal or transdomal sutures, or a cephalic trim. The dorsum would be deficient or overprojecting or deviated. In these cases the possibilities medialwere the osteotomies. dorsal augmentation, the dorsal reduction with or without a spreader graft on the concave side and a dorsal strut in association with lateral and/or

The choice of the procedure has to analyze the defect, the anatomical components and the correlation between them and the whole face. Each aspect, in fact, has to be restored individually and in mutual anatomical respect, in order to achieve the best harmonious result. We would like to show our experience reporting five cases: the first one was a bilateral secondary deformity. She was previously treated in other hospital but none on the nasal region. We performed an open rhinoplasty and orthognathic surgery. The second patient is a unilateral lip and nose deformity. The third patient is a left cleft lip and palate outcome. While the fourth and the fifth patients are left cleft lip and palate outcome that were previously treated three and four time respectively in other structures with206 unsatisfactory aesthetic and functional results. 10th World Cleft Lip, Palate & Craniofacial Congress

The first patient is a bilateral cleft lip and palate outcome. She came to our observation for the unsightly aesthetic results and for breathing difficulties. The patient presented underprojecting columella, hypodefinition of the premaxilla, nasal base widening, lack of tip definition, right dorsum deviation, naso-labial angle alteration, alae cartilages deformity and horizontally oriented nares (Fig. 1, Fig. 2, Fig. 3). In this case we decided to perform a columellar graft to obtain tip rotation, a cephalic trim to define the tip and a butterfly graft to resolve the atbreathing the time difficulties of the rhinoplasty. and reinforce the alar valve zone (10, 11, 12). Considering the hypoplasia and the maxillary retrusion, we performed a Le Fort I osteotomy

The patient was satisfied from the surgery and refers an improvement in breathing (Fig. 4, Fig. 5, Fig. 6). The second one is a unilateral cleft lip and palate outcome. She came to our observation for the upper lip scar retraction and the nasal deformity (Fig. 7, Fig. 8, Fig. 9). We decided in this case to perform a left ala repositioning and upper lip lipofilling to fill the scar depression (Fig.10, Fig. 11, Fig. 12). The third patient is a unilateral left cleft lip and palate outcome. She was previous treated in other institute for the primary defect but the results were uncomfortable. She presented dorsum and tip nasal left deviation, upper lip scar retraction, nares asymmetry and a different nares orientation (Fig. 13, Fig. 14, Fig. 15). In this case we decided to perform an open rhinoplasty and the upper lip scar revision. The results were satisfactory (Fig. 16, Fig. 17, Fig. 18). The fourth patient was previously treated three times on the nasal region through a costal cartilage graft, a second one for the failure of the first and a sequent third skull graft. Furthermore he underwent orthognathic surgery. He came to our observation for breathing difficulties, recurrent maxillary infections and the unsightly results. The patient had characteristic features. In frontal view were evident nasal dorsum deviation, alar malpositioning, overdistanced ala and medial crus and the previous graft; in lateral view the patient presented a saddle nose deformity, naso-frontal angle alteration and nasal tip hypoprojection; in axial view the alterations were alar malpositioning, lateral crus displacement, nasal tip asymmetry, nasal dorsum deviation and infero-posterior alar displacement (Fig. 19, Fig. 20, Fig. 21). Considering the clinical and anamnestic features we decided to remove the previous graft and its fixation screw, to perform an alar batten graft to reinforce the nares and a spreader graft to reestablish the normal breathing (13). 207 10th World Cleft Lip, Palate & Craniofacial Congress

The results were satisfactory both from a respiratory point of view that from an aesthetic one (Fig. 22, Fig. 23, Fig. 24). The fifth patient is a left cleft lip and palate outcome. She was submitted to four previously surgical treatments to reconstruct the nasal region but none of them obtained the result. She came to our observation for the consequently problems of these procedures. She presented right dorsum deviation, nares asymmetry, scar retraction on the left labial side, saddle nose deformity, obtuse naso-frontal angle, maxillary hypoplasia, columellar retraction and tip deformity (Fig. 25, Fig. 26, Fig. 27). Causing the clinical features we than decided to reconstruct the entire nasal structure both the osseous part than the cartilaginous one with a costho- condral graft (14, 15, 16, 17), and to perform a bilateral alar batten graft to reinforce the weakened alar cartilages (Fig. 28, Fig. 29, Fig. 30). Conclusions The patient aesthetic and respiratory conditions improve after surgery.

The secondary rhinoplasty in cleft lip and palate patients is a challenging surgical procedure. In our experience, as expressed by McComb (7, 8, 9) and Salyer (18, 19, 20) the most important aspects that allow the best results are complete undermining of the alar cartilages, extensive dissection of them, andsubperiosteal them proper undermining repositioning. of the structure up to the pirifom aperture along the frontal process of the maxilla, dissection of the abnormal muscular insertions

When, however, these procedures are not performed through an appropriate approach, the structures anatomic restoration become of more complex execution. The tissues, already deformed by the defect, are displaced and fibrotic; the mucosa and skin are more adherent to the overlying and underlying structures with greater difficulty in isolation and the vascularization is reduced. In many cases, in which the cartilaginous skeleton change is not enough, a structural approach was necessary, as suggested by Toriumi (14, 15), to obtain the anatomic and functional restoration of the inferior third of the nose. In these cases we reconstructed the entire tip-columellar complex using autologous cartilage. Becomes than a priority to solve the problems that previous surgical procedures have made reducing the therapeutic options (tissues to be graft already used and than missing). In these cases the patients have to be followed by an experienced surgeon, able to understand in details the deformities and 208 able to understand what is obtainable. The experienced surgeon may have the 10th World Cleft Lip, Palate & Craniofacial Congress

knowledge of all the possible treatment options and the capacity to modulate these on the specific case to achieve the best possible results, both from an aesthetic point of view and functional. In these case it is not enough to be prepared on the basic techniques because the wide anatomic variability. It is fundamental to have a large wealth of experience that allows the possibility to adapt each single technique to the anatomical deformity that the singleReferences case presents. 1. Steven L. Goudy, Travis T. Tollefson Complete Cleft Care Cleft and Velopharyngeal Insufficiency Treatment in Children 2. Dallas Rhinoplasty - Nasal Surgery by the Masters, Third Edition 3. Ortiz Monasterio F, Ruas EJ. Cleft lip rhinoplasty: the role of bone and cartilage grafts. Clin Plast Surg. 1989 Jan;16(1):177-86. 4. J.-B. Duron , G. Aiach. Cartilaginous graft in rhinoplasty. Annales de chirurgie plastique esthétique (2014) 59, 447—460 5. P.S. Nguyen, J.-B. Duron, J. Bardot, Y. Levet, G. Aiach. Surgical approaches in rhinoplasty. Annales de chirurgie plastique esthétique (2014) 59, 406—417 6. J.-B. Duron, W. Noel, P.S. Nguyen, Y. Jallut, G. Aiach. Tip sutures. Annales de chirurgie plastique esthétique (2014) 59, 461—466 7. McComb HK, Coghlan BA. Primary repair of the unilateral cleft lip nose: completion of a longitudinal study. Cleft Palate Craniofac J. 1996 Jan;33(1):23-30 8. McComb HK. Primary reapir of the bilateral cleft lip nose: a long-term follow up. Plast Reconstr Surg. 2009 Nov;124(5):1610-5 9. McComb H. Primary repair of the bilateral cleft lip nose: a 4-years review. Plast Reconstr Surg. 1994 Jul;94(1):37-47 10. Clark JM, Cook TA. The butterfly graft in functional secondary rhinoplasty. Laryngoscope 2002 Nov;112(11):1917-25 11. Friedman O, Cook TA. Conchal cartilage butterfly graft in primary functional rhinoplasty. Laryngoscope. 2009 Feb;119(2):255-62 12. The Conchal Cartilage Butterfly Graft. Facial Plast Surg. 2016 Feb;32(1):42-8. doi: 10. 1055/s-0035-1570326. Epub 2016 Feb 10 13. Sheen JH. Spreader graft: a method of reconstructing the roof of the middle nasal vault following rhinoplasty. Plast Reconstr Surg. 1984 Feb. 73(2):230-9

209 10th World Cleft Lip, Palate & Craniofacial Congress 14. Dean M Toriumi, Daniel G Becker. Rhinoplasty Dissection Manual. 15. Dean M Toriumi. Structure approach in rhinoplasty. Facial Plast Surg Clin North Am. 2005 Feb; 13(1):93-113. 16. Daniel RK. Rhinopalsty and rib grafts: evolving a flexible operative technique. Plast Reconstr Surg 1992; 94:597-611 17. Cervelli V et al. Reconstruction of the Nasal Dorsum With Autologous Rib Cartilage 18. Haisong Xu, Kenneth E. Salyer, Edward R. Genecov. Primary Bilateral One-Stage Cleft Lip/Nose Repair- 40-Year Dallas Experience- Part I. J Craniofac Surg 2009;20: 1913- 1926 19. Salyer KE, Haisong Xu, Edward R. Genecov. Unilateral Cleft Lip and Nose Repair; Closed Approach Dallas Protocol Completed Patients. J Craniofac Surg 2009;20: 1939-1955 20. Haisong Xu, Kenneth E. Salyer, Edward R. Genecov. Primary Bilateral Two-Stage CleftLip/Nose Repair: Part II. (J Craniofac Surg 2009;20: 1927-1933

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Secondary correction of cleft lip nasal deformity

Jin-Young Choi Dept of Oral and Maxillofacial Surgery, School of Dentistry, Seoul National University, Korea

One of the most challenging procedures in cleft lip and palate related surgery is the correction of the cleft lip nasal deformity. Cases differ widely in terms of whether they are associated with unilateral or bilateral cleft lips, depending on the severity of the clefting. It is very important to analyse where and how much deformity in nasal anatomical structure of the patients exist to correct nasal deformity. With that in mind, an individualized approach approapriate to the patients deformity should be adopted in each case. The basic techniques for the correction of cleft lip nasal deformities are as follows 1. Symmetrical positioning of alar cartilage and dome 2. Cartilage graft for • Increasing projection and symmetry • Comouflage residual asymmetry 3. Septoplasty, nasal osteotomy for • Correction of septal deviation 4. Cartilage, bone graft for • Profile deficiency, depression, irregularity, Also the patients with cleft lip nasal deformities may have problems which patients without cleft lip nasal deformities have in Asian country such as dorsal augmentation or tip projection. Rhinoplasty should be done considering not only cleft lip nasal deformity but also nasal aesthetics. In this presentation, the anatomy of nose, basic rhinoplastic techniques, implant materials, its applications focusing on my favorite techniques will be introduced and discussed.

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Computer- assisted craniofacial surgery – experiences of the interdisciplinary Berlin Centre for Craniofacial Surgery

1Nicolai Adolphs, 2Matthias Schulz, 2Hannes Haberl, 1Bodo Hoffmeister 1Department for Craniomaxillofacial Surgery – Campus Virchow Klinikum, 2Pediatric Neurosurgery – Campus Virchow Klinikum, Interdisciplinary Centre for Craniofacial Surgery, Charité Universitätsmedizin Berlin

Objectives

Computer assisted technologies are well established in all fields of contemporary surgery. With respect to complex craniofacial corrections computer assisted surgery is also able to support planning, surgical transfer and documentation of operative results. Possibilities and limits of these technologies are demonstrated based on the experiences of the Berlin Centre forM&M Craniofacial Surgery.

Since 2006 computer assisted technologies have been used for the planning of different craniofacial corrections in selected patient cases (n=15). DICOM datasets were used for individual 3d-model fabrication and virtual simulation of different skeletal displacements in the first patient cases. Subsequently surgical cutting guides (DePuy Synthes Trumatch®) as well as patient specific implants (KLS Martin Uniqos®) were used in order to improve result.the transfer of the final surgical plan. Superimposition of pre- and postoperative skeletal situations was performed in order to evaluate the effective surgical Results

Preoperative workflows have been improved since the first application in 2006. Virtual simulation of different surgical options was helpful in determining the individual treatment plan especially in distraction procedures. Intraoperative transfer can be supported by cutting guides that are created according to the virtual surgical planning. A promising approach is the use of patient specific implants in challenging skelettal situations. Superimposition of pre- and postop skeletal situations offered additional options with respect to assessment,212 evaluation and documentation of skeletal changes with respect to 10th World Cleft Lip, Palate & Craniofacial Congress

qualityDiscussion/Conclusion control and surgical teaching.

Computer assisted technologies are usable for surgical corrections of the craniofacial framework. They support planning, transfer and evaluation of complex corrections. Although preoperative workflows have improved additional efforts of cost and time limit the application of these technologies to selected patient cases for the moment.

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Evaluation of prenatal and postnatal growth and associated congenital anomalies of cleft palate children in Sri Lanka

Prabodha LBL1, Nanayakkara BG1, Dias DK2, Ilayperuma I1. 1Molecular Genetics Laboratory, Department of Anatomy, Faculty of Medicine, University of Ruhuna, Galle, Sri Lanka. 2Regional Cleft Centre & Maxillo-Facial Department, Teaching Hospital, Karapitiya, Galle, Sri Lanka.

Introduction

Cleft palate is one of the commonest congenital malformations of the head and neck region which is associated with cleft lip and other congenital anomalies. It occurs in all races, both sexes and all socio-economic groups(1). Cleft lip and palate is associated with long term disability and psychological stress in children. Multidisciplinary approach with modified feeding techniques and regular respiratory assessments are very important with surgical repair of the cleft defect, speech therapy, orthodontic management, ENT assessment and regular pediatric follow up(1). Systematic review and meta-analysis of the birth prevalence of orofacial clefts in low- and middle-income countries, estimated that an incidence of 1 in 730 live births with cleft lip with or without cleft palate(2). In Sri Lanka, data is available from the Central province where Amaratunga and Chandrasekera (1989) reported the incidence of cleft lip and palate to be 0.83 per 1000 live births while the incidence of isolated cleft palate is 0.19 per 1000 live births(3). A study of congenital malformations among all live births at the General Hospital, Anuradhapura, over a one year period from February 2002 to January 2003 found that the prevalence of cleft lip with or without cleft palate was 2.19 per 1000 live births(4). There are several genetic and environmental factors involved in the process of morphogenesis of the primary and secondary palates(3,5). Stickler, Apert, Otopalatodigital, Pallister Hall, Kabuki, Cornelia de Lange, Oro facio digital, Campomelic dysplasia, DiGeorge, Treacher Collins are some common syndromes associated with cleft lip and palate(6). Patients with oral clefts may also exhibit other anomalies. However, the data is conflicting with widely different estimates for each associated anomaly varying between 2-65% in different countries(7-11). The associated anomalies include those affecting the214 cardiovascular, respiratory, musculoskeletal, genito-urinary nervous and 10th World Cleft Lip, Palate & Craniofacial Congress

gastrointestinal systems. Psychological problems are also seen more frequently among cleft palate subjects(12,13). An infant with cleft palate will require some modifications in feeding techniques and positioning for feeding(14,15). Infants with cleft palate demonstrate poor weight gain due to various reasons. Frequent nasal regurgitation, lengthy feeding time and increased respiratory effort of the infant as well as reduced lactation in mothers due to poor sucking reflex are few causes for poor weight gain of an infant. Specific nutritional deficiencies may also occur and need addressing. Other clinical syndromes associated with cleft palate also contributed to the poor weight gain(14,15). The impairment in weight and length was more severe in patients with cleft palate with or without cleft lip compared to the isolated cleft lip group(16). Objective of this study was to evaluate the demographic background, birth history, physical growth and associated congenital malformations of children less than 5 years of age with cleft palate in Southern Sri Lanka and compareMaterials that and with methods the national figures.

The subjects were recruited from the Regional Cleft Centre & Maxillo- Facial Department, Teaching Hospital, Karapitiya, Galle, Sri Lanka. Patients with cleft palate without cleft lip (CP) below five years of age were identified among patients registered from 1st of January 2000 to 31st of May 2016 in the cleft clinic. Detailed clinical data were recorded by the researcher. Weight, height, and occipitofrontal circumference (OFC) were recorded and weight for age, height for age, occipitofrontal circumference for age were calculated. Physical growth of these subjects was calculated according to the guidelines issued by World Health Organization (WHO) by using WHO anthro (version 3.2.2.) software. Data were compared with annual health statistics that was available at the Medical Statistics Unit, Department of Health Services, Ministry of Healthcare and Nutrition, Sri Lanka. Epi Info TM 7.1.0.6 software was used for statistical analysis. Proportions of the same groups such as growth parameters were compared using one way chi square test. Numerical data were compared using student T test. Ethical clearance for this study was obtained by University of Keleniya. Informed writtenResults consent was obtained from parents or guardian of study subjects. Gender distribution

Total of 262 cleft palate subjects less than five years of age were identified215 and requested to participate in the study. Out of the total 262, 153 subjects 10th World Cleft Lip, Palate & Craniofacial Congress participated. Majority (92%) of the subjects were from the Southern province of Sri Lanka. There were 90 (58.8%) female subjects and the male to female ratio (sex ratio) of the study sample was 0.7. According to the Department of census and statistics in Sri Lanka the sex ratio in the Southern province of Sri Lanka is 0.96(17). There was a statistically significant (p<0.05) difference Birth History between study sample and national figures.

Majority of the subjects were first born babies of the family (51.81%) and there was a statistically significantly higher incidence (p = 0.0001) of cleft palate among first rank births in the Southern part of Sri Lanka (Table 1). Table: 1 Comparison of birth rank of the study subjects with that of the general population(18).

There were 11 (7.2%) preterm births and 58 (37.9%) low birth weight subjects with cleft palate and there was a statistically significant (p < 0.0001) number of low birth weight among cleft palate subjects (Table 2). Table: 2 Comparison of low birth weight subjects among the general population and study sample(19).

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Mean birth weight of the study sample was 2.73kg and this was a statistically significant difference (p < 0.05) (Table 3) when compared with national figures(20). Table: 3 Comparison of mean birth weight among the general population and study sample(20).

Anthropometric measurements in less than five years old subjects Anthropometric assessment revealed that the weight of 124 (81%) subjects were below the 50th centile for age. Ninety (58.8%) and 71 (46.4%) of subjects were below the 50th centile for height and OFC for age respectively. There was a statistically significant (one way chi-square test, P < 0.0001, chi-square 35.14) proportion of reduced weight for age among cleft palate subjects (Table 4). Table: 4 Distribution of the weight for age among cleft palate subjects Parameter

There was a statistically significant (one way chi-square test, P < 0.05, chi-square 5.14) proportion reduced height for age among cleft palate subjects (Table 5). Table: 5 Distribution of height for age among cleft palate subjects

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There was no statistically significant proportion of reduced OFC for age among cleft palate subjects (Table 6). Table: 6 Distribution of OFC for age among cleft palate subjects

Other associated clinical features and syndromic diagnosis.

A total of 70 (45.7%) subjects had other clinical features associated with the cleft palate. There were 28 (18.3%) subjects with dysmorphic features, system22 (14.4%) abnormalities, with congenital epilepsy, heart genital, defects, gastro 20 intestinal (13.1%) and with renal developmental anomalies delay, speech delay, hearing impairment, visual abnormalities, central nervous were seen among study subjects. Clinical genetic evaluation identified 13 (8.5%) subjects with clinically diagnosed syndromes among cleft palate subjects. Suspected syndrome diagnoses included , Femoral hypoplasia and unusual face syndrome, Fetal valproate syndrome, Pierre Robin syndrome, Apert syndrome, DownDiscussion syndrome and Edward syndrome.

Cleft palate is one of the commonest malformations affecting the craniofacial region(1). It is associated with cleft lip and various other clinical manifestations. The London dysmorphology database includes more than four hundred syndromes associated with cleft palate(1). Regional Cleft Centre & Maxillo-Facial Department, Teaching Hospital referredKarapitiya, to isthis a major unit for cleft their palate surgery centre and situated follow inup the treatment. Southern province of Sri Lanka. Many cleft lip and palate patients from different parts of Sri Lanka were

This study shows that the cleft palate (without cleft lip) is more common in females than males in the Southern part of Sri Lanka. In surveys on the epidemiology of the cleft lip and palate in Thailand, China, Australia, Estonia and Jordan reported the female predominance of cleft palate without cleft lip(10,21,22,23,24). 218 Birth rank in the family of all the one hundred and fifty three (n=153) 10th World Cleft Lip, Palate & Craniofacial Congress

subjects were analyzed. Using data from the population and housing statistics, (2008), birth ranks of the Sri Lankan population were compared with the birth ranks of the current study. There was a statistically significant higher incidence (p = 0.0001) of cleft palate among first rank births in the Southern part of Sri Lanka(18). Different studies across the world have analyzed the birth rank and the incidence of cleft palate. Majority of studies identified that the increasing birth rank is associated with higher incidence of cleft palate (Table 7)(3,25, 26,27,28,29). Table: 7 Incidence of cleft palate with birth order

An optimal birth weight of a new born indicates the successful and optimal growth and the development of the fetus in the uterine cavity(30). According to the human development unit of the South Asian region, the normal birth weight of a new born is defined as the birth weight between 2.5kg to 3.5kg. Below 2.5kg indicates the low birth weight (LBW) and birth weight below 1.5kg indicates the very low birth weight of the new born. A preterm birth is defined as a birth of a new born less than 36 weeks of gestational age(30). Birth weight of a new born baby is an indicator of child’s survival and its ability to bear the risks in early childhood. There are several common reasons for low birth weight in a newborn including maternal malnutrition,219 10th World Cleft Lip, Palate & Craniofacial Congress preterm delivery, maternal age at birth and multiple gestations. The Sri Lanka demographic and health survey (SLDHS), 2006/07 reported that 17.3% of live births had birth weight less than 2.5kg which falls in the category of low birth weight(19,31). Incidences of low birth weight babies are higher in the estate sector than in rural and urban sectors in Sri Lanka(19,31). There was a higher proportion of LBW babies in the cleft palate population compared with the general population and the difference was statistically significant (p < 0.0001). Nanayakkara et al., (2011) conducted a prospective population based study involving four thousand one hundred and twenty (n=4120) births at the maternity unit, Teaching Hospital Kandy, in the years 2008 and 2009 to identify the birth weight of Sri Lankan newborns. The mean birth weight in the Central province of Sri Lanka was identified as 2.854kg(20). Association between birth weight and cleft palate in different populations are tabulated in the table 8(26,28,32,33). populations. Table: 8 Association between birth weight and cleft palate in different

Cleft palate is associated with a number of other major clinical anomalies(1). In this study seventy (45.7%) subjects had other clinical abnormalities. systemDevelopmental abnormalities, delay and epilepsy, dysmorphic genital, features gastro wereintestinal the commonest and renal anomalies identified problems. Cardiac anomalies, speech delay, hearing impairment, central nervous were the other associated problems found in subjects with cleft palate. There had been no formal and consistent methodology involved in identification of associated anomalies among cleft palate subjects in previously published 220research. Some studies had excluded syndrome diagnoses and chromosomal abnormalities(9,10,23) while other studies included associated malformations 10th World Cleft Lip, Palate & Craniofacial Congress

as a part of syndromic or other associated genetic condition(26,34,35,36,37). isolated Table: 9 Percentages of other associated anomalies among subjects with

Physical growth of the cleft palate subjects below five years of age were assessed using a protocol stipulated by the World Health Organization (WHO anthro software version 3.2.2. January 2011). There were a total of eighty three study subjects below five years of age. Majority of them were below the 50th percentile for their weight for age and height for age were statistically significant according to the WHO standards for the Asian population. There was no statistically significant association with occipito-frontal circumference design.(OFC) for age. It is not possible to comment on the catch up growth in the current group of Sri Lankan cleft palate cases as this would require a longitudinal study

There are more than four hundred syndromes associated with cleft palate(1,6). There were 12 patients who were clinically diagnosed as having suspected syndromes of which three patients with down syndrome and two patientConclusion with Edward syndrome were confirmed by conventional karyotyping.

Study subjects showed statistically significant female predominance and prenatal and postnatal growth retardation compared to the normal population. High incidence of associated congenital anomalies and syndromes indicated 221 a possible genetic etiology of cleft palate children. The results signify the 10th World Cleft Lip, Palate & Craniofacial Congress importance of concomitant clinical evaluation and genetic screening to identify the etiology and further management of cleft palate. Table: 10 Growth of cleft lip and palate subjects in different populations(32,38,39,40,41)

Acknowledgment

Authors wish to thank all the patients who participated in the study without any financial gaining. A sincere acknowledgment has to go to the staff of the Department of Anatomy, Faculty of Medicine, University of Ruhuna, Sri Lanka and regional cleft centre and maxilo-facial department. Furthermore, I wish to thank Professor Vajira H.W. Dissanayake, Director, Human Genetics Unit and Dr. Nirmala Sirisena, Senior Lecturer, Human Genetics Unit, Faculty of Medicine,References University of Colombo for their help and encouragement. 01. Hodgkinson PD, Brown S, Duncan D, Grant C, McNaughton A, Thomas P and Mattick, R. Management of children with cleft lip and palate: A review describing the application of multidisciplinary team working in this condition based upon the experiences of a regional cleft lip and palate centre in the United Kingdom. Fetal and Maternal Medicine Review 16(1): 1–27, 2005 02. Kadir A, Mossey PA, Blencowe H, Moorthie S, Lawn JE, Mastroiacovo P, Modell B. Cleft Palate Craniofac J. 2016 Jul 19. [Epub ahead of print]. 222 10th World Cleft Lip, Palate & Craniofacial Congress 03. Amaratunga, AN de S and Chandrasekera, A. Incidence of cleft lip and palate in Sri Lanka. J Oral Maxillofac Surg 47(6): 559-61, 1989. 04. De Alwis ACD, De Silva KNS, Bandara WDMSD and Gamage TGPM. Prevalence of talipes equinovarus, congenital dislocation of the hip, cleft lip/cleft palate, Down syndrome and neural tube defects among live newborns in Anuradhapura, Sri Lanka. Sri Lanka Journal of Child Health 36: 130-2, 2007. 05. Murray JC., Gene/environment causes of cleft lip and/or palate. Clinical Genetics 61(4): 248–256, 2002. 06. Bush JO and Jiang R. Palatogenesis: morphogenetic and molecular mechanisms of secondary palate development. Development.139(2): 231-43, 2012. 07. Womersley J and Stone DH. Epidemiology of facial clefts. Arch Dis Child 62: 717-720, 1987. 08. Jones MC. Etiology of facial clefts: Prospective evaluation of 428 patients. Cleft Palate Journal 25(1): 16-20, 1988. 09. Rajabian MH and Sherkat M. An epidemiologic study of oral clefts in Iran: analysis of 1,669 cases. Cleft Palate Craniofac J 37(2): 191-6, 2000. 10. Qiao-juan Z, Bing S, Zong-dao S, Qian Z and Yan W. Survey of the patients with cleft lip and palate in China who were funded for surgery by the Smile Train Program from 2000 to 2002. Chinese Medical Journal 119(20): 1695-1700, 2006. 11. Gowans LJ, Adeyemo WL, Eshete M, Mossey PA, Busch T, Aregbesola B, Donkor P, Arthur FK, Bello SA, Martinez A, Li M, Augustine-Akpan EA, Deressa W, Twumasi P, Olutayo J, Deribew M, Agbenorku P, Oti AA, Braimah R, Plange-Rhule G, Gesses M, Obiri-Yeboah S, Oseni GO, Olaitan PB, Abdur-Rahman L, Abate F, Hailu T, Gravem P, Ogunlewe MO, Buxó CJ, Marazita ML, Adeyemo AA, Murray JC, Butali A. Association Studies and Direct DNA Sequencing Implicate Genetic Susceptibility Loci in the Etiology of Nonsyndromic Orofacial Clefts in Sub-Saharan African Populations. J Dent Res.95(11):1245-56:2016. 12. Lu JH, Cung MY, Hwang B and Chein HP. Prevalence and Parental Origin in Tetralogy of Fallot Associated With Chromosome 22q11 Microdeletion. Official Journal of the American Academy of Pediatrics 104(1): 87-90, 1999. 13. Halder A, Fauzdar A, Kabra M and Saxena A. Detection of 22q11.2 Hemizygous Deletion by Interphase FISH in a Patient with Features of CATCH22 Syndrome. Indian Paediatrics 42: 1236-1239, 2005. 14. Tervo RC and Chudley AE. Management of Cleft Lip and Palate: A Team Approach. Can Fam Physician 27:1964-1970, 1981. 223 10th World Cleft Lip, Palate & Craniofacial Congress 15. Woolridge MW. The ‘anatomy’ of infant sucking. Midwifery 2(4): 164-71, 1986. 16. Luiz C. Montagnoli,1 Marco A. Barbieri,2 Heloisa Bettiol,3 Ilza Lazarini Marques,4 Luiz de Souza5. Growth impairment of children with different types of lip and palate clefts in the first 2 years of life: a cross-sectional study. J Pediatr (Rio J). 81(6):461-5, 2005. 17. Department of census and statistics-Sri Lanka. Population and housing censuses in Sri Lanka, 2001. Available at: (Accessed on 15th June 2016). 18. Department of census and statistics in Sri Lanka. Population and housing statistics. Live births registered by live birth order, sex and districts in Sri Lanka, 2008. Available at: (Accessed on 15th June 2016). 19. Medical Statistics Unit, Department of Health Services, Ministry of Healthcare and Nutrition, Sri Lanka. Annual Health Statistics. 2007. Available at: (Accessed on 15th June 2016). 20. Nanayakkara KK, Samarakoon AB, Perera BH, de Silva AW and Nanayakkara CD. Size of Sri Lankan newborns at birth. J Obstet Gynaecol. 31(4): 311-4, 2011. 21. Chuangsuwanich A, Aojanepong C, Muangsombut S and Tongpiew P. Epidemiology of cleft lip and palate in Thailand. Ann Plast Surg 41(1): 7-10, 1998. 22. Linda DVN, Riley MM and Halliday J. An Epidemiologic Study of Isolated Cleft Lip, Palate, or Both in Victoria, Australia From 1983 to 2000. Cleft Palate Craniofac J 41(2): 185-94, 2004. 23. Jagomagi T, Soots M and Saag M. Epidemiologic factors causing cleft lip and palate and their regularities of occurrence in Estonia. Stomatologija, Baltic Dental and Maxillofacial Journal 12: 105-8, 2010. 24. Al-Omari F and Al-Omari IK. Cleft Lip and Palate in Jordan: Birth Prevalence Rate. Cleft Palate–Craniofacial Journal 41(6): 609-612, 2004. 25. Ismail AR, Ismail NM and Naing L. A pilot study of risk factors in cleft lip/palate patients. Proceedings of the 9th NICMS conference, University of Sains Malaysia, Health Campus, Kubang Kerian, Kelantan. 2004. 26. Boo NY and Arshad A R. A study of cleft lip and palate in neonates born in a large Malaysian maternity hospital over a 2 year period. Singapore Med J 31: 59-62, 1990. 27. Bender PL. Genetics of cleft lip and palate. J Pediat Nurs 15: 242–49, 2000.

224 10th World Cleft Lip, Palate & Craniofacial Congress 28. Fraser GR and Calnan JS. Cleft Lip and Palate: Seasonal incidence, Birth weight, Birth rank, Sex, Associated malformations and parental age: A statistical survey. Arch Dis Child. 36(188): 420–423, 1961. 29. Martelli DRB, da Cruz KW, de Barros LM, Silveira MF, Swerts MSO and Martelli JH. Maternal and paternal age, birth order and interpregnancy interval evaluation for cleft lip and palate. Braz J. Otorhynolaryngol 76(1): 107-12, 2010. 30. Report No. 40906-LK. Health, Nutrition and Population, Human Development Network, Human South Asia Region Development Unit. Sri Lanka. Malnutrition in Sri Lanka: Scale, Scope, Causes, and Potential Response. 2007. Available at: (Accessed on 16th June 2016). 31. Medical Statistics Unit, Department of Health Services, Ministry of Healthcare and Nutrition, Sri Lanka Sri Lanka health at a glance. 1:1-10, 2008. Available at: (accessed on 16th June 2016). 32. Ranalli DN and Mazaheri M. Height-weight growth of cleft children, birth to six years. Cleft Palate J 12: 400-4, 1975. 33. Wyszynski DF, Sarkozi A, Vargha P and Czeizel AE. Birth weight and gestational age of newborns with cleft lip with or without cleft palate and with isolated cleft palate. Journal of Clinical Pediatric Dentistry 28(2): 185-190, 2003. 34. Krumova V. Clinical and Genetic Peculiarities of Isolated Cleft Palates. Journal of IMAB - Annual Proceeding (Scientific Papers) 14(2): 52-54, 2008. 35. FitzPatrick DR, Raine PAM and Boormanm, JG. Facial clefts in the west of Scotland in the period of 1980-1984: epidemiology and genetic diagnoses. J Med Genet 31:126-129, 1994. 36. Jensen BL, Kreiborg S, Dahl E and Fogh-Anderson P. Cleft lip and Palate in Denmark, 1976-1981: Epidemiology, Variability and early Somatic Development. Cleft Palate Journal 25(3): 258-269, 1988. 37. Shprintzen RJ, Sadewitz VLS, Amato J, Goldberg RB, Opitz JM and Reynolds JF. Anomalies associated with cleft lip, cleft palate, or both. American Journal of Medical Genetics. 20(4): 585–595, 1985. 38. Paradise JL, Mangubat, OV and Josefczyk P. Undernutrition in young infants with cleft palate: A prevailing but remediable problem. Presented at American Cleft Palate Association, Boston, Mass. 1974.

225 10th World Cleft Lip, Palate & Craniofacial Congress 39. Avedian, LV and Ruberg, RL. Impaired weight gain in cleft palate infants. Cleft Palate J 17(1): 24-6, 1980. 40. Pandya AN and Boorman JG. Failure to thrive in babies with cleft lip and palate. British Journal of Plastic Surgery 54(6): 471-475, 2001. 41. Lee J, Nunn J and Wright C. Height and weight achievement in cleft lip and palate. Arch Dis Child 76: 70-72, 1997.

226 10th World Cleft Lip, Palate & Craniofacial Congress

Aesthetic Camouflage for Correction of Cleft Deformities: Surgical Options and Outcomes

David P. Tauro National President, Association of Oral & Maxillofacial Surgeons of India, Facial Plastic & Reconstructive Surgeon, The Taulins Clinic, Center for Craniofacial & Aesthetic Facial Surgery, Bangalore, India

Abstract

A secondary facial deformity be it mild, moderate or severe following corrective surgery of the cleft lip & palate is an inevitable consequence of even the most diligent & meticulous surgery. Besides the soft tissue disabilities of the lip, most patients present with an obvi-ous element of maxillary regression consequent to maxillary hypoplasia resulting in a grotesque class III skeletal deformity. In addition, concomitant nasal deformities do occur which also need to be addressed. These deformities could also be a result of repetitive surgical interventions done in a manner that do not follow a chronological sequence of procedures. Given a choice most patients who present for the correction of the residual aesthetic de-formities do not subscribe to a prolonged and a cumbersome distraction osteogenesis schedule. Also many of these patients having gone through an array of surgical proce-dures are now looking for an aesthetic correction with the a minimal surgical morbidity and downtime. Careful and diligent planning of combining orthognathic procedures in conjunction with facial implants and rhinoplasty can produce predictable & excellent results much to the satisfaction of the patients. Cases illustrating this very concept are presented and dis-cussed.

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Management of secondary zygomatico- orbital deformities

Yogesh Bhardwaj Professor and Head, Dept. of Oral & Maxillo-Facial Surgery, HP Govt. Dental College & Hospital (IGMC), Shimla, India

Secondary deformities of orbito-malar region are psychologically devastating and sometimes functionally crippling aesthetic deformities which present as a formidable challenge to the surgeon for their adequate correction. The optimal management of such deformities is ideally grouped into two headings : 1. Osteotomy techniques and 2. Camouflage procedures. Obviously the osteotomy techniques provide more holistic functional and aesthetic alternatives to these often complex problems. In the current presentation I would try to highlight the management strategies involved in achieving these aesthetic and functional goals. The need for a learning curve from routine fresh trauma to these secondary deformities is emphasized for achieving good surgical results. A series of cases would be displayed to illustrate various surgical techniques in managing secondary deformities of this complex and often surgically challenging area.

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Presurgical orthodontic treatment of children with a bilateral cleft lip and palate

Mamedov AA, Zangieva OT, Blinder JA, McLennan AB, Mazurina LA, Kalinina AI, Magomedov RN I.M. Sechenov First Moscow State Medical University, Faculty of stomatology, Department of Pediatric dentistry and orthodontics. Offices 202, 203 2-4 Bolshaya Pirogovskaya str., Moscow 119991, Russia

Introduction

Cleft lip and palate (CLP) is one of the most common malformations of the maxillofacial area that has a tendency to increase. Lodging on the 3-4 place within the structure of congenital anomalies, CLP occupies one of the first places with respect to the severity among the anatomical and functional disorders. Among those, the most severe form is the bilateral CLP, which occurs less frequently (15-25%) than other forms. In recent years many clinics have adopted fixed orthodontic appliances with intramedullary fixation to reposition the intermaxillary bone and to expand of the fragments of the upper jaw in children with CLP (Egorova MI 2010; Katasonova ES 2011; Starikov NV 2006, 2014; Blinder JA 2016). At present time, it is becoming increasingly important to perform the primary operation at early stages of child growth. It requires an interdisciplinary approach. Moreover, with the progress of orthodontic techniques, it becomes possible to perform a successful surgery using orthodontic preparation at the early stages of child life. This study presents how to use microimplants in presurgical treatment as the fastest and easiest way for preparation before surgery. We developed simple mechanics using microimplants retracting intermaxillary bone to boneimprove of the lip older protrusion group ofbefore patients. operation of newbone patients. Moreover, the use of microimplants helps to correct the bite and position of intermaxillary

The aim of this study is to increase the efficiency of surgical treatment using pre-orthodontic correction of the intermaxillary bone position in children with bilateral CLP. 229 10th World Cleft Lip, Palate & Craniofacial Congress Material and methods.

During the period from 2011 to 2016 an orthodontist and maxillo oral tosurgeon one year. working in collaboration have developed an algorithm for treatment of children with bilateral CLP and have treated 24 patients aged from two months

During two weeks period, they had been performing intermaxillary bone traction towards an attached appliance, whilst at the unit itself the movement had been directed towards the side of the upper jaw fragments by unwinding the screw. After two weeks they had removed the orthodontic appliance and the microimplants from the intermaxillary bone. In this way the whole assembly has been removed. This had been followed by the primary-stage bilateral cheiloplasty.Results Orthodontic protocol

The orthodontic protocol includes obtaining of an impression, making a model (a control model is simultaneously made for further investigations and measurements), making an individual orthodontic appliance with expanding screw and a splint with toe loops to attach to the intermaxillary bone. This is shown in Figures 1, 2, 3, 4.

Fig.1. Patient K-va, 2 months old, bilateral cleft lip and palate.

Fig. 2. Patient K-va, 2 months old, computed tomography in two projections.

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Fig. 3. Obtaining of an impression and manufacturing of diagnostic and control models.

Fig. 4. Making a custom tray and an individual plate shaped orthodontic appliance with expanding screw and splint with toe loops to attach to the intermaxillary bone.

Surgical procedure

Before the start of the surgical treatment the orthodontic appliance is attached to the side of the upper jaw fragments using miniscrews, and microscrews are attached to the both sides of the intermaxillary bone. The main distinctive feature of this approach is that a partial vomer osteotomy is performed before installing the orthodontic appliance. This makes it possible to reduce the time of preparation for primary cheiloplasty. Microimplants are connected to the main structure using spring. Two weeks later, when the intermaxillary bone and the lateral fragments are in the optimal state, the primary cheiloplasty is performed under endotracheal anaesthesia (Figures 5, 6, 7).

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Fig. 5. Partial vomer osteotomy, fixing of the orthodontic appliance to the fragments of the upper jaw with microscrews. In the process, we abandoned the splint with intermaxillary bone toe loops and used microscrews instead. Those were fixed to the both sides of the intermaxillary bone. This proved to be much more effective than the splint with toe loops. Next, the orthodontic appliance and the mini screws were fixed by using springs.

Fig. 6 Fixation of the microscrews to both sides of the intermaxillary bone.

Fig. 7. Fixation of the microscrews and the orthodontic appliance containing springs and removal of the orthodontic appliance after two weeks.

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Fig. 8. Position of the intermaxillary bone before treatment and before surgery.

Fig. 9 Results of the primary cheiloplasty immediately after and two weeks after the surgery.

Orthodontic correction with cortical support on microimplants also increases the effectiveness of surgical treatment in older groups of patients. This approach does not require the use of massive orthodontic appliances, which improves the quality of treatment and reduces the time of treatment, improves hygiene and does not prevent speech development.

We also used orthodontic protocol for older withpatients. a Thisdiagnosis can be seenof in a 13 years old patient

thecomplete patient bilateral state cleftbefore lip and palate. Fig. 10 shows

orthodontic correction. Fig. 10.Patient 13 years old withbilateral cleft lip and palate.

For orthodontic correction Smart Clip braces were used. (Fig. 11)233 10th World Cleft Lip, Palate & Craniofacial Congress

Fig.11. Arch wire 0.14 NiTi

implantsAfter were 8 weeks, used atthe the arc side 14x25 fragments NiTi was of replaced. the maxilla At theand stage a minisrew of levelling, was to monitor the position of the posterior teeth, miniplates supported by micro fixed in the intermaxillary bone. From the miniscrew to miniplate we fixed elastic chain from the both sides. This allowed intrusion and rotation of the intermaxillary bone. A distractor was inserted and was used during two weeks. (Fig. 12, 13).

Fig 12. Patient K-v, 13 years old, 8 weeks, arc 14x25 NiTi.

Fig 13. Patient K-v, 13 years old. A distractor is fixed to the palate fragments.

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After 4 month the wire was replaced by TMA 17x25 with compensatory bends for further rotation of the intermaxillary bone. The wire was fixed in the side section to the micro implants to avoid extrusion of teeth in the lateral segments. This is shown in Fig. 14.

Fig. 14. The result of orthodontic treatment before and after 6 months.

On the upper jaw we have got +8 mm more between premolars and the flat occlusion plate. intermaxillaryThe strategy bone. of the further treatment is the normalization of the retrusive position of incisors on the low jaw, which makes the stable position of the

Then the final surgical correction, guided bone regeneration and the prosthodonticConclusions treatment are planned.

01. Clinical and biometric research was performed on jaw models from 24 children with bilateral CLP both at the preoperative and the postoperative periods. Normalization of the relations between the intermaxillary bone and the lateral fragments was observed in all patients. 02. The use of the modern appliances design with modifications and the use of micro implants allows reducing the time of the orthodontic preparation to the surgery for infants (under one year). The described approach allows introducing active elements into the orthodontic appliance, to normalize the position of the intermaxillary bone and the shape of the upper jaw, and to perform subsequent primary cheiloplasty. 03. For a successful early orthodontic preparation it is necessary to ensure a reliable fixation of the orthodontic appliance. This reduces the treatment time, creates favourable conditions for primary cheiloplasty, improves patient condition during postoperative period, prevents the development of secondary deformities of the maxillofacial region, and significantly reduces the time of rehabilitation of patients with bilateral CLP with the 235 good aesthetic results. 10th World Cleft Lip, Palate & Craniofacial Congress

04. In older patients, the use of microimplants allows successfully and in a short time to carry out orthodontic correction of anomalies of dental system without the use of additional massive orthodontic appliances, which impair References.speech and hygiene and bring general discomfort to the patients. 01. Egorova MV. Orthodontic treatment of young children with unilateral cleft lip and palate using an appliance made from metal with shape memory. PhD thesis, Moscow, 2010.-P.18. 02. Katasonova ES. Use of micro implants for treatment of children with congenital cleft lip and palate. PhD thesis, Almaty, 2011. 03. Supiev TK, Mamedov AA, Negametzyanov NG, Congenital cleft lip and palate, Almaty, KazNMU, 2013.-P.496. 04. Starikova NV Structural features of tongue dysfunction in patients with cleft lip and palate: clinical significance and choice of the orthodontic treatment strategy. PhD thesis, Moscow, 2014.-P.24

236 10th World Cleft Lip, Palate & Craniofacial Congress

Modified 3D NAM appliance for the correction of shifted pre maxilla

Midhun Naik Assistant professor, Department of Orthodontics, A.J institute of dental sciences., Mangalore, Karnataka, India

Cleft of the lip, alveolus and palate (CLAP) are the most common congenital mal-formations of the head and neck. India being the second most populated country in the world with a population of 1.21 billion, it is estimated that out of 24.5 million births per year - the birth prevalence of clefts is between 27,000 to 33,000 clefts.1 The incidence of CLP in India is 1 in 781 live births.2 The bilateral cleft lip deformity typically arises with a procumbent or rotated premaxilla. The alar base width is significantly increased, and the lip segments are widely separated.3 The flattened nasal tip is tethered directly to the prolabium by a severely deficient or absent columella. Lower lateral alar cartilages are flared and concave where they should be convex. The greatest challenge for esthetic reconstruction is the absent or deficient columella. Presurgical infant orthopedics achieve alignment of the maxillary segments, presenting a more symmetrical platform and width reduction of the alveolar ridge cleft. This enables elevation of the alar base on the cleft side and lip closure. The objectives of early active maxillary orthopaedics are two-fold.4 First, in cases of wide unilateral clefts or protrusive premaxilla in bilateral clefts initial cheiloplasty is difficult due to the distance the tissue must be mobilized to close the defect. This causes excessive tension on the surgical site, which may lead to wound dehiscence. Presurgical maxillary orthopaedics allow earlier, more ideal lip closure with minimum tissue tension since the soft tissues will overlie a more normal bony anatomy. If by using orthopedic therapy, the requirement of lip adhesion surgery prior to a definitive repair may be eliminated. Early maxillary orthopedics can move the maxillary segments to a more anatomically correct position and the soft tissues will be carried with the segments, leading to a decrease in the width of the defect, which will reduce surgeon time, hospital time, and the risks of additional general anesthesia. Eliminating lip adhesion surgery also eliminates the need to perform additional surgery in the presence of scar tissue from an earlier operation. Second, left untreated, the lateral alveolar segments usually "collapse", leading to malalignment. With early orthopedic intervention, a more normal arch form may be achieved, resulting in better237 10th World Cleft Lip, Palate & Craniofacial Congress leads to better soft tissue approximation alignment of the segments. The more normal or near normal alveolar alignment

Previous approach for the shifted premaxilla was more focused on the surgical correction and repositioning .The aim of this study is to reposition the shifted premaxilla without extruding it for better retraction. This presurgical infant orthopaedics reduces the tissue tension and scarring which have detrimental effects on maxillary growth. This innovative 3D screw type appliance is easy to fabricate, use and it works in all direction. A modified 3D NAM appliance for the correction of shifted pre maxilla was used in this study. CASE NO: 1

ConclusionCASE NO: 2

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239 10th World Cleft Lip, Palate & Craniofacial Congress

The modified Screw type appliance will allows the growth of the alveolar

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processes in the right direction, reshapes the flattened nose, helps in minimizing theREFERENCES extent of complex surgeries.

01. Banerjee M, Dhakar AS. Epidemiology-clinical profile of cleft lip and palate among children in India and its surgical consideration. J Surg 2013;2:45-51. 02. Sharma MP, Sandhu BH, Kumar MA. Presurgical nasoalveolar molding in unilateral cleft lip and palate patient. Indian J Dent Adv 2012;4:1024-9. 03. Latham R. Development and structure of the premaxillary deformity in bilateral cleft lip and palate.Br J Plast Surg. 1973;26:1–11 04. Sierra, Frank J.; Turner, Clara, 1995: Maxillary orthopedics in the presurgical management of infants with cleft lip and palate. Pediatric Dentistry 17(7): 419-423

241 10th World Cleft Lip, Palate & Craniofacial Congress

Median Cleft Lip with Congenital Midline Upper Lip Sinus: A rare case report

Lam Chin Yan, Vimahl Dass Oral & Maxillofacial Surgery Department, Hospital Tawau, Sabah, Malaysia

Abstract

Median cleft lip is defined as midline vertical cleft through the upper lip. It is a rare congenital craniofacial anomaly, which arises from incomplete fusion of medial nasal prominence. The incidence is reported to be about 1: 10,00,000 births, or accounting to 0.43%-0.73% among all patients of cleft lips A median cleft lip associated with upper lip sinus, on the other hand, is extremely rare, with only 5 cases reported thus far. The authors present a rare case of median incomplete cleft lip together with midline upper lip sinus. Following surgical intervention, a satisfactory aesthetic result was achieved. The embryology, presentation, surgical technique and a brief discussion on midline upper lip sinus are discussed.

242 10th World Cleft Lip, Palate & Craniofacial Congress

Bilateral cleft lip management by bilateral millard procedure; outcome of 50 cases

Mahesh KumarMugria Departmant of Plastic and reconstructive surgery,L.U.M.H.S, Jamshoro, Pakistan

Purpose of presentation / study

A brief statement of the purpose of the study and the current state of research in the field. The purpose of this study was to s manage this congenital anomaly with bilateralStudy Period millard techniques.

The study at the Department of Plastic &L.U.M.H.S and other hospitals atMethods Hyderabad /Procedure from 2013 details to 2015.

In this observational study 50 patients of different age groups of both genders presenting with bilateral cleft lip and palate were included. Bilateral millard surgical techniques was done in all cases whether they were complete orResults incomplete /outcome or premaxilla was protruded or not.

Out of 50 cases,nasal deformity persist in10 patients. ConclusionsAesthetic appearance of lip seems to be good in most of thecases

Bilateral cleft lip is common congenital anomaly which can be managed with Billateral Millard procedure with acceptable aesthetic outcomes.

243 10th World Cleft Lip, Palate & Craniofacial Congress

Palatal Ruguoscopy in Patients with Cleft Palate – A Pilot Study

ASWATH G, V.B. KrishnaKumar Raja, Sneha SRM DENTAL COLLEGE & HOSPITAL, RAMAPURAM, CHENNAI.

ABSTRACT

The study and analysis of lip prints and dermatoglyphics has been one of the corner stone in the field of Forensic odontology. In the recent past the topattern establish of palatal the different rugae and patterns their distributionof the palatal is rugae gaining in different importance populations in the field & of forensic odontology and Oro facial sciences. Studies have been done in the past different age groups. The development of the palatal rugae has been attributed to the differences in the growth rates on the oral and the nasal surfaces of the maxilla. Studies in orthodontic literature have stated that irrespective of the active forces on the maxilla for orthodontic tooth movement, the pattern of the maxillary rugae remain the same pre and post orthodontic treatment. The aim of our study is to study the patterns of palatal rugae in patients with cleft palate and correlate them with the parent/ off springs to assess if these findings can predict in the pattern of hereditary transmission of the Cleft palate.

244 10th World Cleft Lip, Palate & Craniofacial Congress

A comparative evaluation of complications of single and multiple stage procedures in unilateral cleft lip, alveolus and palate patients

Satish Prathigudupu1, Somuri Anand Vijay1, L. Krishna Prasad2, Srinivas Chakravarthy3, M. Sridhar3, Rahul Tiwari4 Reader1, Principal2, Professor3, Post Graduate Student4, Sibar Institute Of Dental Sciences, Takkellapadu, Guntur, India

ABSTRACT:

Aim: Cleft lip and palate are the most common congenital deformities of the maxillofacial region. It is estimated to be 1 in 700 live births. There are various surgical techniques proposed in the literature. So the study aims to compare single stage and multi stage closure in cleft lip and palate patient. Patients and Methods:20 subjects presented to department of oral and maxillofacial surgery in Sibar Institute of Dental sciences requiring surgical treatment for cleft lip alveolus and palate were indulged in the study.Subjects are divided into two groups. Group 1 consists of 10 subjects for single stage procedure (case) and group 2 involves 10subjects for multi stage procedure (control). The complications occurred in a follow up of 1 year were evaluated. Results: There were 5 male and 5 female in the group 1 with a mean age of 10.8 months and 5 male and 5 female in group 2 with a mean age of 10.9 months. Only unilateral cleft patients were included in the study.In first group 5 had scarring and 3 had palatal fistula compare to group 2 which had 3 scarring and 1 palatal fistula. We observed 9 complications in group 1 and 4 complications in group 2. Conclusion: Single stage procedure produces less psychological stress and is cost effective but post operative scar and alveolar bone grafting has a high failure rate with more amounts of complications as compared to the multi stage procedure. 245 10th World Cleft Lip, Palate & Craniofacial Congress

Keywords: unilateral cleft, single stage surgery, multiple stage surgery, complications Introduction:

Congenital deformities are the abnormalities in the appearance or function that occur due to unusual occurrences in the embryological development sequence during the intrauterine period. Cleft lip is a congenital deformity that presents as an abnormal lack of continuity of the lip musculature, lip skin and the mucous membrane. The unusual breach can extend from the base of nose to the free margin of lip. The deformity may range from a simple notch at the free margin to a complete disjointed lip that also results in a nasal deformity. This most commonly occurs in the upper lip. Cleft palate is an abnormal discontinuity in the palatal musculature, mucous membrane with or without involvement of the hard palate, which may extend from the uvula to the alveolus. These deformities are present since birthand affect the esthetics function and stability of the individual. It will have various trajectories involved including the psychological stress to the velopharyngeal incompetence. The growth of facial form should be accounted inherent with the craniofacial development. The growth of face depends on various factors like the technique, timing and sequence of the procedures1.Thesurgeon’s primary objective is always to restore natural and attractive contours, highlights landmarks and in effect recreate the relationship of form that define regional units and establish the normalcy of face. In the modern age there is alarming increase in the births of children’s with clefts and other congenital malformations. One of the trends in the management of cleft lip and palate is correction of the entire anatomical deficit at a very young age, even before the functional pattern hasdeveloped. This is in contrast to the older concepts where the correction was done at different stages.So this study aims at comparing the effectiveness of surgical management of cleft lip alveolus and palate using two different approaches i.e. singlePatients stage and and methods: multiple stage closure.

The present study was carried out at the department of oral and maxillofacial surgery, Sibar institute of dental sciences Guntur, during the period of October 2013 to March 2015. 20 patients, with unilateral cleft lip alveolus and palate deformities were selected for this study, at random. It is a prospective design of study. In 20 patients, 10 were treated by single stage closure of cleft lip, alveolus and palate at the earliest possible time. The second group of 10 patients was treated by multi stage closure of cleft lip, alveolus and palate246 defect using different techniques. 10th World Cleft Lip, Palate & Craniofacial Congress

Two different approaches in the management of cleft lip and palate patients were compared. The evaluation was done on the post-operative complicationsResults: after a follow up of one year.

There is no significance difference between the mean age of the patients undergoing the procedure between the two groups. The earliest stage at which a single stage procedure was carried out was at 5 months of age. The wide variation in the age is due to the fact that patients present themselves to the hospital at different stages. The ages in single stage group varied from 5 months to 30 months with a mean age of 10.8 months(Fig. 1A) and in the multiple stage group the age varied from 3 months to 30 months with a mean age of 10.9 months (Fig.1B). In single stage group 5 males and 5 females were present with a ratio of 1:1. (Fig. 2A).Even in multiple stage groupalso the males were 5 and females were 5 with a ratio of 1:1 (Fig. 2B). Only unilateral clefts were included in the study and there was almost equal distribution of right and left area was noticed. (Fig 3) This is in contrast to the usual finding that the unilateral clefts are more common on the left side. However it is not significant because of the small sample size. Of the 10 patients who belong to the multi stage group, underwent a mean of 2.4 procedures per patient, whereas only one patient among the 10 of the single stage group had to have a subsequent procedure. (Fig. 4) Therefore two of the claimed advantages of the single stage procedure are born out in this study i.e. reduced psychological stress due to the less number of procedures and reduced cost of treatment. On assessing the lip in the single stage procedure out of 10 patients 6 had scarring. (Fig.5A). In multi stage procedure out of 10 patients 3 had scarring.(Fig. 5B).However problems in the form of scarringwere seen to a great extent with those who underwent single stage procedure. This is probably due to the technique of repair rather than timing because even in the multistage procedure lip repair is carried out the same stage. Complete closure of the palatal defect was obtained in only about 60% of cases in both the study groups. On assessing the palate in the single stage procedure out of 10 patients 3 had palatal fistula in which 2 had anterior fistula and 1 had posterior fistula. On assessing the palate in the multi stage procedure out of 10 patients only 1 had palatal fistula which palate.was present on the anterior side. (Fig.6). This illustrates the problem of getting good anatomic continuity, leave along function in a patient with complete cleft

There was a great number of dehiscence of the palatal defect a total of 1patient in a single stage group. However it did not matter in the long run because the percentage of palatal fistula was the same eventually. However247 10th World Cleft Lip, Palate & Craniofacial Congress in about 33 % of patients who underwent single stage procedure the alveolar bone graft was exposed and subsequently lost. This finding seems to question the wisdom of using the alveolar bone graft at the same time as the closure of the cleft lip and palate.The immediate complications encountered in single stage group are 1 with anterior palatal dehiscence. Late complications included 1 with graft exposure. In multi stage group 1 had wound dehiscence in the lip as an immediate complication. The late complications include 1patient with palatal fistula. The descriptive analysis of the study was performed withMann Whitney and Spearman correlation coefficient test. The data was analyzed via SPSS ver.20.Discussion:

The cleft abnormality is the cause of underdevelopment and subsequent loss of function. The primary cleft surgery to correct the secondary deformities of previous nonfunctional defects should aim to restore the function, normal anatomy and physiology with an emphasis on muscular reconstruction of lip and soft palate if normal development is to be encouraged.2The functional abnormalities are due to the lack of attachment of nasolabial muscles which are the primary cause of underlying skeletal malformation. The closure of lip and soft palate should be carried out at the age of 6 months and little later in case of bilateral clefts. The residual defects between the premaxilla and maxilla proper in wider clefts can be closed with gingivoperiosteal flaps at a later stage before the functional patterns are developed3.Tight lip in repaired cleft lip and palate patient is one of the casual factors of the maxillary growth retardation. This trend was more common in bilateral cleft lip and palate than unilateral cases4.Immediate reconstruction and reorientation of the musculoperiosteal apparatus enable the interaction between the primary and secondary growth centers which activates the physiological growth stimuli and improve the development of mid facial skeleton. If the primary and secondary growth centers are not joined at the first operation and mid facial muscles are not constructed even orthopedic stimulation of growth will not compensate the defect5. The periosteal musculoaponuerotic reconstruction is a key objective in primary repair in cleft lip as it influences the growth and development of the nose.6 The importance of identifying and preserving intravelar muscle during dissection that enables the velar muscles to lie in the midline and prevents traction during velopharyngeal closure is important as this muscle is invariably divided and reoriented in non-anatomic position in the Furlow double opposing Z248 plasty.7Klaus Honigmannevaluated the functional development of the palate 10th World Cleft Lip, Palate & Craniofacial Congress

and mid face and emphasizes on the speech when compared to two stages of closure and found that the closure of the cleft lip, alveolus and palate in a single stage within a year, the maturation of the hearing tract is not affected as the tracts develop one year after birth.8 This procedure has another advantages like easy dissection, less psychological trauma to parents and patients and is cost effective. The need for reconstruction of the anatomy of the cleft of the hard and soft palate is not only to recreate the levatorvelli palatine muscle sling but also palatal aponeurosis which serve as transmitter of forces of the glossovelopharyngeal muscle.9Patient treated at later stage had minimal retrusion of maxilla than who had been treated at an early age.10A differential and individual approach should be used for determining the most convenient age for correction according to the extent of the defect. Use of orthopedic appliance immediately after lip surgery prevents the collapse of palate post operatively.Simultaneously closure of the alveolar cleft at the nasal side results in continued reduction of anterior cleft width. Cleft lip and palate deformities pose major challenge to the surgeon, venturing its correction. There are innumerable techniques and lots of controversies surrounding the timing of these techniques. Majority of the cleft lip and palate surgeons, prefer to tackle the complete defect in multiple operative stages. However there has also been a trend at single stage procedure for closure of cleft lip and palate. The proponents for these consider it important for the various reasons8 i.e. functional reason is for construction of normal anatomy as early as possible leading to base for the normalization of muscle function and also for psychological reasons. All the procedure being done in single stage there is a reduction in psychological stress for the child and the family. Dissection of an unscarred operating area and is less expensive. On other hand the proponents of the multi stage closure also advocate multi stages for the functional reasons. It is considered important to close the palate in two stages11 achieving a functional veloplasty without disturbing the maxillary fibro mucosa during the closure of the hard palate and without compromising the length of the soft palate. In the present study a group of 20 subjects were divided into two equal groups of 10 each. On group undergoing a single stage closure and second group undergoing multistage operations. The resultsConclusion: of the two groups were compared for the post-operative complications.

The study concluded that complications with the single stage procedure were higher than that of the multiple procedures. Alveolar bone grafting at the same time as repairing the lipand palate defect has high failure rate.249 10th World Cleft Lip, Palate & Craniofacial Congress

Advantages of the single stage procedure lies in fact that it is very cost effective and produces less psychological stress. The superiority of the one approach to the other can only be truly establish by a long term study of how they affect the growthReferences: pattern as well as speech in a large series of patient. 1. Semb G, Shaw WC. Facial growth after different methods of surgical intervention in patients with cleft lip and palate. ActaOdontol. Scand. 1988;56:352-355. 2. AF Markus, J Delaire, WP Smith. Facial balance in cleft lip and palate and secondary deformities. British Journal of Oral and Maxillofacial Surgery. 1992;30:296-304. 3. AF Markus, J Delaire. Functional primary closure of lip. . British Journal of Oral and Maxillofacial Surgery. 1993;31:281-291. 4. Susuami T, Kamiyama H et al. Quantitative evaluation of the shape ans elasticity of the repaired cleft lip. Cleft Palate CFJ. 1993;30:309-312. 5. U Joos. Skeletal growth after muscular reconstruction for cleft lip alveolus and palate. British Journal of Oral and Maxillofacial Surgery. 1995;33:139-144. 6. Bruse B Horswell, Oldrich A Pospisil. Nasal symmetry after primary cleft lip repair: Comparison between DelaireCheilorhinoplasty and Modified Rotation advancement. Journal of Oral and Maxillofacial surgery. 1995;53:1025-1030. 7. Martin HS, Huang. Structure of Musculus Uvulae: Functional and surgical implications of an anatomic study. Cleft Palate Craniofacial Journal. 1997;34(6):466-474. 8. K Honigmann. One stage closure of uni and bilateral cleft lip and palate. British Journal of Oral and Maxillofacial Surgery. 1996;34:214-219. 9. KH Hubertus Koch, MagdalenaGrzonka, Josef Koch. Pathology of the palatal aponeurosis in cleft palate. Cleft Palate Craniofacial Journal. 1998;35(6):530-534. 10. ZybnekSmahel, ZivaMullerova. Facial growth and development in unilateral cleft lip and palate during period of puberty: Comparision of the development after periosteoplasty and after primary bone grafting. Cleft Palate Craniofacial Journal. 1994;31(2):106-115.

250 10th World Cleft Lip, Palate & Craniofacial Congress 11. AF Markus, WP Smith, J Delaire. Primary closure of cleft palate- A functional approach. British Journal of Oral and Maxillofacial Surgery. 1993;31:71-77. FIGURES:

Figure 1 A: Distribution of patients according to age at first surgery – Single Stage

Figure 1 B: Distribution of patients according to age at first surgery – Multiple Stage 251 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 2 A: Distribution of patient according to sex – Single Stage Figure2 B: Distribution of patient according to sex – Multiple Stage

Figure 3: Distribution of patient according to site involved- Single stage and Multiple Stage

252 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 4: Number of surgeries – Multi stage Figure 5 A: Complications occurred in lip - Single stage.

Figure 5 B: Complications occurred in lip - Multiple stage. Figure 6: Distribution of complications on palate according to site– Single stage and Multiple stage

253 10th World Cleft Lip, Palate & Craniofacial Congress

D-Cleft: an innovative method for feeding neonates born with Cleft palate

1Anubhav Jannu, 2Ruchi Gupta, 1Assistant Professor, Department of Oral & Maxillofacial Surgery, KLE Society’s Dental College & Hospital, Yeshwantpur, Bengaluru, Karnataka, India, 2PG student, Department of Pediatric and Preventive Dentistry, Shree Bankey Bihari Dental College, U.P., India

Introduction:

Babies born with cleft palate anomaly suffer from feeding problems which interfere with their nutrition for growth and development; thereby, hampering the criteria for subsequent corrective surgery. The palatal cleft interferes with nursing and causes insufficient suckling or aspiration/nasal regurgitation of food. Coughing, choking, vomiting, infection of the nasopharynx and chronic catarrh are frequent. Because of altered anatomy and presence of oro-nasal fistula, baby tends to present .suction difficulties due to lack of negative intraoral pressure. The nursing time1 is longer. This can lead to anxiety and concern of parents and/or caregivers Presented here is an innovative solution. A patented device for attaching on commercially-available feeding bottles to facilitate effective nutrition method. The presentation is not only case series report but also about this device’s journey from concept to patient. It is to show that any Invention with Materialsright amount and of Methods: care grows into a fruitful tree benefitting masses.

Innovation is the back-bone for providing solutions. Any innovative idea has to follow a series of steps to convert into viable effective solution. These steps include Idea, Patent, Prototype, Trials and Mass manufacture. The journey of the present idea started with attachment of commonly available plastic spoons to the rim area of feeding bottle cap. This provided a seal on cleft-palate defect during feeding procedure. It also provided a scaffold against which the nipple could rest on and be pressurized upon during suckling motions. Further, it could be customized by addition of soft-relining material to seal254 the defect completely while stabilizing the attachment. 10th World Cleft Lip, Palate & Craniofacial Congress

In a series of otherwise successful case trials, main drawbacks found were improper fit of spoon to the plastic of bottle and greater distance between concave surface of spoon to the nipple during suckling movement. Further development of the device was entrusted to a team of professionals at Vaatsalya Inventures who tirelessly worked on the design. The device was hence further modified to fit to the rim of bottle around the whole circumference and adapted by wax in close proximity to the nipple for better suckling without need for greater pressure. This was followed by filing of patent which included complete specification and all possible modifications of device. Several iterations later, final 3D design of the device was formed using Autodesk Alias and 123D. D-cleft was thus printed in 3D printing machines using PVA material in Flashforge 3-d machine. Prototypes were finished using waterproof sand-paper abrasives. The prototypes are currently being tried on patients for final modifications before theResult: next steps of mould-formation for mass production.

The device was tried and tested successfully in many neonates born with Cleft palate. It has been successfully used to feeding neonates by their mothers/ care-takers with almost no training required. D-Cleft provided good seal of palatal defect from nasal regurgitation and was found to act as a scaffold against pressing of feeding-bottle’s nipple during suckling movements. It has overcome several disadvantages presented by in- dwelling tubes or long-term intra-oral attachment methods most importantly constant irritability for child. Other drawbacks such as impression procedure, sterilizationDiscussion and hygiene issues are almost non-existent. as follows.Previous literature mentions several methods of feeding for babies born with cleft palate. Some of the most common citations and their drawbacks are

Reid et al. reviewed and stated that feeding treatment modalities should be preceded by altering the positioning of the baby. In PRS babies with cleft palate, breast-feeding is not possible, but one can always attempt it. In all feeding practices, baby should be kept in an upright position, with head in one hand and bottle in another, and fed at an angle of 450 with frequent burping passageof the child.. This allows the milk to flow down and helps to prevent choking. If incorrect2 position is employed for feeding, milk may enter the respiratory255 10th World Cleft Lip, Palate & Craniofacial Congress

Devi et al discussed different feeding methods mentioned in various literatures and suggested that breast, bottle, spoon or cup feeding or a combination may be chosen with lactation instructions pertaining to correct positioning of the child. Speciality feeding products like supplement nursing system or special feeding bottles like Mead Johnson, Haberman. feeder, soft squeeze baby bottle. But these special products have drawbacks3 like high-cost, leakage around the nipple and difficulty in sterilization Prodoehl DC and Shattuck KE stated that as a feeding modality, the positioning.placement of The an use in-dwelling of feeding nasogastric tubes are said tube to shouldbe responsible be considered for most in of case the upper airway obstruction and feeding difficulties are not resolved by prone recorded perforations as they are soft at first and become hard and stiff after several hours of use. Reports of rare complications such as urinary bladder feedingperforation,. pericardial sac perforation and enterobacteriaceae colonization on prolonged4 feeding expose the risks associated with using intubation for

According to Savion I et al. there are two schools of thoughts. The first one advocates its advantages as ease in feeding, facilitation of cleft repair, enhancement of speech and facial growth. The other group claims its disadvantages such as cumbersome impression taking procedure, difficulties associated with sterilization. and hygiene maintenance, expensive manufacture and effort involved in 5 training the caretaker about effective intra-oral attachmentConclusion of prosthesis

Very few clinicians have knowledge of fabricating prosthesis for feeding such patients which otherwise adds to the distress of caretakers. The presented device extremely easy to use, easy to maintain and highly economical. In cases where modifying the feeding position alone cannot make much difference, these attachments can be advocated. It is a versatile device in that it can be attached to feeding bottle of any measurement and it can be brought to fit the cleft defect without any hassle. Further scope of using face-scanner, table-top scanner and 3D printing machines toReferences: customize such feeding prostheses is also possible. 01. Dalben GS, Costa B, Gomide MR, Neves LT. Breast-feeding and sugar intake in babies with cleft lip and palate. Cleft Palate Craniofac J. 40(1):84-7, 2003

256 10th World Cleft Lip, Palate & Craniofacial Congress 02. Reid J. A review of feeding interventions for infants with cleft palate. Cleft Craniofac J 41(3):268-278, 2004. 03. Devi ES, Sankar AJS, Sujatha B. Maiden morsel - feeding in cleft lip and palate infants. J Int Soc Prev Community Dent 2(2): 31–3, 2012. 04. Prodoehl DC and Shattuck KE. Nasogastric Intubation for nutrition and airway protection in Infants with Robin sequence. J Perinatol 15:395-7, 1995. 05. Savion I and Huband M. A feeding obturator for a preterm baby with Pierre Robin sequence. J Prosthet Dent 93:197-200, 2005.

257 10th World Cleft Lip, Palate & Craniofacial Congress

Influence of NAM & GPP on UCLP patients in mixed dentition: A 3D Model analysis

Arvind Sivakumar, Yuki Sato Yamamoto Dept of Craniofacial Orthodontics, Saveetha Dental College, Chennai.

ABSTRACT Introduction

Combination of Nasoalveolar molding [NAM] followed by Gingivoperiosteoplasty [GPP] has reduced the need for secondary alveolar bone grafting by 60 to 70 %. The aim of the present study is to evaluate the effects of NAM and GPP, on the development of maxillary dentition and arch form, at mixed dentition, in subjects with UCLP using 3 Dimensional model analysis. Materials & methodology: 65 UCLP patients (38 females & 27 males) were selected for the study. Group I (n=24) subjects who had no NAM or GPP, Group II (n=24) subjects who had only NAM without GPP. Group III (n=17) subjects who had both NAM & GPP. Study models were obtained at mixed dentition & were digitized to 3D models by laser scanning using DIPRO XY scanner & analyzed by Zengaku3D software. ANOVA with Bonferroni post-hoc analysis was performed. Results: The Group III had significantly higher inter 1st deciduous molar arch width than Group I [p=0.009] & II [p=0.017]. The anterior cleft segment showed significant difference between group I & group II (p=0.021). Both group II & group III had Ip-I-Tm angle closer to 0 which was significantly different from group I [group I Vs II p=0.002, group I Vs III p=0.008]. Conclusion: Early closure of the cleft gap by NAM helps in increase in arch width. Anterior segment on the non-cleft side was found to be greatest in the NAM-GPP group, showing that GPP promotes alveolar growth on the non-cleft side. Both NAM & NAM-GPP groups have symmetrical arch forms. Thus to conclude NAM does influence the alveolar morphology & occlusion with growth.

258 10th World Cleft Lip, Palate & Craniofacial Congress

FEEDING CONSIDERATIONS OF CLEFT LIP AND CLEFT PALATE PATIENTS

SAUVIK SINGHA PG Resident, Oral & Maxillofacial Surgery, RDVV Jabalpur, Madhya Pradesh, India

Cleft lip and palate is a common congenital maxillofacial defect. One of the most important problems with it is the interference with feeding. When a baby is born with a cleft palate, he faces major feeding difficulties, whether fed by breast, bottle, or both. An affected infant cannot produce negative pressure in the oral cavity and therefore cannot move the bolus backward to the pharynx. The degree of difficulty depends upon the severity and location of the cleft, as well as other factors. Inability of the baby to be fed and receive nutrition causes malnourishment and delayed growth. This presentation reviews the different feeding interventions such as breast feeding, modified bottles and/or teats, obturating plates and maternal counselling and support.

259 10th World Cleft Lip, Palate & Craniofacial Congress

Comparison of Early Onset Nasoalveolar Molding With Patients Who Presented for Molding Up to 1 Year of Age

VIKRAM SHETTY1, ANKUR THAKRAL2, CHANDNI SREEKUMAR3, B. RAJENDRA PRASAD4, SATADRU ROY5 1Director, Nitte Meenakshi Institute of Craniofacial Surgery, K.S. Hegde Charitable Hospital, Nitte University, 2Dental Officer and Graded Specialist in Oral and Maxillofacial Surgery, Corps Dental Unit, P-8 Khulna Marg, Sultania Infantry Lines, Bhopal, 3Assistant Professor, A.B. Shetty Memorial Institute of Dental Sciences, Nitte University, 4Director (PG Studies) and Professor & Head of the Department, Department of Oral and Maxillofacial Surgery, A.B.Shetty Memorial Institute of Dental Sciences, 5Junior Resident, Department of Oral and Maxillofacial Surgery, A.B. Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, Karnataka, India

ABSTRACT INTRODUCTION:

The goal of primary closure of the lip for unilateral cleft lip is to ensure a normal and symmetrical lip and nose. It is well established that surgical repair alone cannot solve the multiple problems encountered with the deformities that arise from clefts of the lip and palate. Grayson et al were accredited with the development of the technique of nasoalveolar molding (NAM). This study was done to compare the effectiveness of nasoalveolar presentingmolding (NAM) for treatment. in infants with complete unilateral cleft lip and palate presenting before and after 6 months of age and justify its use in older infants MATERIALS AND METHODS:

The university NAM protocol was followed for 100 patients who were included in the study. NAM was performed below 6 months of age (group I, n = 50), and at 6 months to 1 year of age (group II, n = 50). Seven linear anthropometricRESULTS: measurements were compared using dentofacial models.

Statistical analysis before and after NAM showed that group I had 83, 176, 69, and 142% improvement in intersegment distance, nasal height, nasal 260 10th World Cleft Lip, Palate & Craniofacial Congress

dome height, and columella height respectively. Group II had 45, 38, 28, and 62%CONCLUSION: improvement.

Patients in both the groups showed improvement with the NAM protocol. Although patients who presented for treatment before 6 months of age benefited the most, those who presented at 6 months to 1 year of age benefited as much from NAM as those who presented below 6 months, thus validating its use in these patients.

261 10th World Cleft Lip, Palate & Craniofacial Congress

Nasoalveolar molding - The first step towards rehabilitation

SHAILAJA. R, G. VIMALA PG STUDENT, Tamil Nadu Govt. Dental College, Chennai,Orthodontics And Dentofacial Orthopedics, F-27 golden jubilee apartments, Anna main road, Kk nagar, Chennai-78, TamilNadu, INDIA,

ABSTRACT

Cleft lip and palate is the most common congenital malformation of head and neck. Patients present with deficient hard and soft tissue and have compromised function in esthetics. Incidence of cleft lip and palate in Indian population is 1 in every 781 live births with unilateral cleft more common than bilateral. Pre-surgical orthopedics has been employed since 1950 for rehabilitation of cleft lip and palate. Nasoalveolar molding (NAM) represents a paradigm shift from traditional method of pre surgical orthopedics. NAM technique takes the advantage of malleability of immature nasal cartilage and its ability to maintain permanent correction of its form. Emphasis is laid on molding prior to lip surgery which reduces the number and cost of revision surgeries performed later. This paper will highlight case report of early intervention of cleft lip and palate by NAM technique.REFERENCES: 01. Grayson BH, Shetye PR. Presurgical nasoalveolar moulding treatment in cleft lip and palate patients. Indian J Plast Surg. 2009 Oct;42 Suppl:S56-61 02. Kirbschus A, Gesch D, Heinrich A, Gedrange T. Presurgical nasoalveolar molding in patients with unilateral clefts of lip, alveolus and palate. Case study and review of the literature. J Craniomaxillofac Surg. 2006 Sep;34 Suppl 2:45-8.

262 10th World Cleft Lip, Palate & Craniofacial Congress Thermographic Evaluation of the Effect of Pre-surgical Nasoalveolar Molding on Lip Tension in Unilateral Cleft Lip and Palate Infants Pre and Post Cheilorhinoplasty

Navdeep Bhusria; Dennis C. Limb; Paolo M.B. Manzanoc; Janet M. Pandand; Ann L.T. Querijeroc; Ronaldo G. Vergarae aCurrently Private Practice, New Delhi, India; Former Postgraduate Resident, Department of Orthodontics, Graduate School, University of the East, Manila, Philippines and, Trainee, Noordhoff Craniofacial Foundation Philippines Inc., Our Lady of Peace Hospital, Coastal Road, San Dionisio, Paranaque City, Philippines.b Associate Professor, Department of Orthodontics, Graduate School, University of the East, Manila, Philippines.c Assistant Professor, Department of Orthodontics, Graduate School, University of the East, Manila, Philippines.d Orthodontics Program Chair and Board of Trustees Member, Noordhoff Craniofacial Foundation Philippines Inc., Our Lady of Peace Hospital, Coastal Road, San Dionisio, Paranaque City, Philippines.e Professor and Chair, Department of Orthodontics, Graduate School, University of the East, Manila, Philippines.

Navdeep Dental Orthodontic and Implant Clinic (NOIC), A-25/26 Indira Nagar, Opp. Panchwati Colony, Delhi-110033, India

ABSTRACT Introduction

The aim of this research was to evaluate effect of pre-surgical nasoalveolar molding therapy on the subjective tension and tissue healing in upper lip pre and post cheilorhinoplasty in non-syndromic complete unilateral cleft lip and palate infants. Infrared Thermography was used in this study which is a non- contact,Materials non-invasive and Methods: diagnostic tool, uses no radiation and is completely safe.

Four groups were studied; PNAM with cheilorhinoplasty group (PNAM) had 6 sujects, lip tape with cheilorhinoplasty (LPTP) consisted 6 infants, cheilorhinoplasty only group (NoPreTx) had 7 samples and a control group (CTRL) of 7 healthy infants without cleft. Fluke Ti9 infrared thermal imager was employed to capture thermograms at three time intervals; first being before surgery (i1), second 1 week after cheilorhinoplasty (i2) and third at follow up 1 month after surgery. The temperature differentials (dt) between cleft (c) and non-cleft (nc) sides were studied for all groups at the three time intervals. The thermal data underwent paired ‘T’ test and analysis of variance at confidence interval of 95% and significance level of 0.05. 263 10th World Cleft Lip, Palate & Craniofacial Congress Results:

Thermographic evaluation showed significant tissue tension still existing 1 month post-surgery in LPTP group (p=0.014) and NoPreTx group (p=0.000), whereas the PNAM group showed non-significant results (p=0.603) which were more consistent with the control group (p=0.220). Also PNAM group showed significant difference to the LPTP (p=0.013) and NoPreTx group (p=0.000) whereasDiscussion it was more closer to CTRL group (p=0.171).

Infants who underwent PNAM therapy before cheilorhinoplasty showed a drastic reduction in cleft to non-cleft side thermal gradients and thus treatmentapparent diminution modalities. in subjective tissue tension at one month follow up period as compared to infants who underwent lip tape or only cheilorhinoplasty ARTICLE FILE INTRODUCTION

Clefts of lip and palate are the fourth most common craniofacial birth defect. The incidence varies in different races, and prevalence of cleft type varies by gender.1 Incidence of cleft lip-palate in Philippines has been reported to be 1.94/1000 live births which supports higher incidences2 of clefting in Asian populations when compared to Caucasian populations. The wider, more extensive clefts are associated with more significant nasolabial deformity and most surgeons would agree that chances of achieving finer surgical scar, good nasal tip projection, and more symmetrical and precisely repair,defined whether nasolabial3 for complex the unilateral would beor better bilateral in an anomaly, infant presenting is to restore with normal minor cleft deformity. The basic goal of any approach of cleft lip, alveolus, and palate anatomy. Ideally, deficient tissues should be expanded, and mal-positioned structures repositioned prior to the surgical correction4 which would provide a foundation for a less invasive surgical repair. The standard treatment protocol for cleft lip- palate patients remains a subject of debate. Two main school of thoughts are, one involving surgical repair alone and other5 involving pre-surgical molding of cleft segments followed by surgical repair. Pre-surgical infant orthopedics has been in the scene since 1950s as an adjunctive neonatal therapy for correction of cleft lip-palate 6when McNeil first introduced the concept of modern pre-surgical orthopedics. Pre- surgical264 nasoalveolar molding (PNAM) treatment is based on study by Matsuo 10th World Cleft Lip, Palate & Craniofacial Congress

inferring that nasal cartilage is developing, lacks elasticity and still tenable to molding within neonatal period7 (first 6 weeks) and which he used to mold nostril using silicon tubes. Pre-surgical Nasoalveolar Molding8 (Figure 1) was first described by Dr. Grayson from New York University.

The effectiveness of PNAM in patients with unilateral9-16 cleft lip and palate (UCLP) has been reported by a few number of studies. Use of PNAM in UCLP patients is a subject of controversy in developing countries regarding therapeutic benefits vs. time and costs involved. Only one13 study till date has been done on outcome analysis of cost in UCLP treatment . There is no scientific proof that passive orthopedics provides improved results, it is an empirical17 approach based on clinical team members’ interaction and influence. Based on clinical observation it is believed that the appliance improves horizontal and vertical skeletal deficiency by stimulating bone production18 before and after lip closure; however this hypothesis remains to be proved. No valid research exists to demonstrate that PNAM reduces tension pre or/and post cheilorhinoplasty. Various modalities have been used to measure muscle/tissue tension namely Electromyography (EMG), Ultrasonography (USG) and Magnetic Resonance Elastography (MRE), all of them being direct contact techniques, whereas researcher explored the non-contact means of measuring tension subjectively in perioral orbicularis group of muscles which are always involved in unilateral cleft lip deformity. Medical Digital Infrared Thermal Imaging (MDITI) has been used since long time for scanning fever at airports, in automotive manufacturing, in construction and architecture; as an adjunctive diagnostic tool for breast cancer screening, for monitoring healing after surgery and, for diagnosing pathologies like varicose veins. Increasing the temperature of a liquid decreases its surface tension, eventually reaching critical temperature at which surface19 tension goes to zero, general formula for this relationship being empirical. Surface Tension (γ) is inversely proportional to Temperature (T) and vice versa, γ ∝ 1/T. It has long been known that muscle tension is temperature-dependent and a negative temperature coefficient, where the tension produced in response to a given stimulus decreases with increasing temperature,20 is shown by some whole muscle preparations and isolated fast twitch fibers. The converse would hold true as well and thus tension may be quantified in a group of muscles like orbicularis oris with means of non-contact, non-invasive infrared thermography diagnostic infraredtechnique. thermography. Face muscle contractions with increased blood flow result in higher temperature of overlying21 skin. This effect can be visualized and quantified by 265 10th World Cleft Lip, Palate & Craniofacial Congress

No studies exist till date applying infrared thermography to subjectively measure upper lip tension and track healing post cheilorhinoplasty procedure in unilateral cleft lip and palate patients. The aim of this research was to evaluate efficacy of pre-surgical nasoalveolar molding therapy in unilateral complete cleft lip and palate patients by subjectively quantifying tension and healing of upper lip using Medical Digital Infrared Thermal Imaging (MDITI) pre and post cheilorhinoplasty.MATERIALS AND METHODS- Research design and Locale

The research design of this study was prospective, exploratory and quasi- experimental. The major part of research; Pre-surgical nasoalveolar molding treatment, infrared thermography, cheilorhinoplasty and data collection was conducted in Noordhoff Craniofacial Foundation Philippines Inc. (NCFPI) partner Our Lady of Peace Craniofacial Center (OLPCC) located in Our Lady of Peace Hospital (OLPH), Coastal Road, Barangay San Dionisio, Paranaque City, 1700, Philippines. The suitability of site for conduct of research was accounted byEthical availability Considerations of all required medical and emergency facilities at OLPH.

The study protocol was reviewed and approved by panel of the Graduate School, University of the East. Approval for conducting research was obtained from the Medical Director, Our Lady of Peace Hospital, Paranaque, Manila, Philippines. The Ethics Review Committee (ERC), Research Institute for Health Sciences, University of the East Ramon Magsaysay Memorial Medical Center (UERMMMC), Aurora Blvd., Quezon City, Manila, Philippines; reviewed and granted approval for implementation of research protocol vide study protocol code 0201/E/O/14/064. The procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation and with the Helsinki Declaration of 1975, as revised in 2000. Patient privacy and confidentiality was protected by assigning each participant a unique patient code.Sampling design and Sample size computation

Non-probability purposive sampling method was used suitable for in-depth qualitative research in which focus is often to understand complex phenomena.22 Applying formula for two sample computation of proportions, keeping Confidence level (Za/2) at 95%(1.96); Desired Power (Zb) at 80%(0.84); Size of difference of clinical importance of PNAM treatment (∩1- 266 ∩2), at 80%(∩1)-15%(∩2) ie. 0.65 (reducing cleft gap by at least 65% from 10th World Cleft Lip, Palate & Craniofacial Congress

6mm to 2mm); N = { (Za/2+Zb)2 x [∩1(1-∩1)+∩2(1-∩2)] } / (∩1-∩2)2, we obtainGrouping, sample Inclusion size, N and = 5.3349 Exclusion (≈6) criterionper group.

Twenty six infants were enrolled in four groups, 6 subjects in PNAM group who came to the craniofacial center at earliest age after birth underwent PNAM therapy and cheilorhinoplasty; 6 subjects in group LPTP who got lip tape therapy and cheilorhinoplasty; 7 subjects in group NoPreTx who underwent no pre-treatment but only cheilorhinoplasty only, and 7 healthy infants without any craniofacial and/or systemic condition constituting control group CTRL. Inclusion criteria for groups PNAM, LPTP and NoPreTx were complete unilateral cleft lip, alveolus and palate; maximum 6 weeks of age at start of PNAM therapy; and at least 3 months age and 5kg weight for cheilorhinoplasty. Exclusion criteria followed for cleft patients with syndromes or other systemic disorders, patients above 6 weeks at the start of PNAM (PNAM), patients above from6 months the study. for group NoPreTx. Non-compliant parents of infants who missed more than 1 appointment or did not use PNAM or lip tape had to be eliminated Instrument and Methodology

lens with The thermal Fluke sTi9 handheld thermal imager used is based on a 160 X 120 focal plane array uncooled microbolometer detector, 20mm EFL F/0.8 infrared ensitivity of ≤ 0.2 ̊C at 30 ̊C (200mK), covering temperature range -20 ̊C to +100 ̊C and infrared spectral band 7.5μm to 14μm (Figure 2). Effect of PNAM on postsurgical descriptive tissue tension and vascular healing was studied using MDITI equipment for 1cm X 1cm area of interest (Figure 3b) on both cleft (c) and non-cleft (nc) sides of upper lip in the four groups PNAM, LPTP, NOPRETX and CTRL at three time intervals, i1- before cheilorhinoplasty to establish baseline skin surface temperature, i2 - one week after cheilorhinoplastyIn order to meet the(on internationalday of suture standards removal), for and thermography i3 - one month parents after cheilorhinoplasty when wound attains adequate strength.29 usingwere instructedinfant head not stabilizer to feed infantspi four hours before test and to acclimate for 30 minutes in craniofacial center.21 Infants’ head movements were controlled llow and the thermograms were captured with camera kept at 1 foot distance (Figure 3a) from area of interest with emissivity ofInterpretation thermal imager and set Data at 0.98extraction for human from skin. Thermograms The thermograms were interpreted for temperature readings using

Smartview Software version 3.5.31.0 (Fluke Corp 2014). The temperature267 10th World Cleft Lip, Palate & Craniofacial Congress gradient is color coded from red to yellow to green to blue, being hot to cold signifying more vascular (less tension) to less vascular (more tension) tissue respectively (Figure 3b). The temperature readings were recorded in Fahrenheit scale. Since temperature can vary from one time interval to another even at the thansame studying anatomic temperature landmark, a readings more conclusive per se. system of studying temperature differentials (dt) between cleft (c) and non-cleft(nc) sides was chosen rather Statistical Analysis

The normality distribution of infrared thermal data was numerically checked with Shapiro Wilk’s test and graphically through Normal Q-Q plots. The temperature differentials (dt) of the region of interest at cleft and non-cleft areas were compared with two-tailed Paired ‘T’ test dependant means in all four groups (PNAM, LPTP, NoPreTx, CTRL). The temperature differential dt1, dt2 and dt3 at different time intervals ‘i1’, ‘i2’ and ‘i3’ respectively were quantfied with one way ANOVA repeated measures and further compared using Tukey HSD Multiple Comparisons test for all groups. All statistical analysis were done at confidence level of 95% and 0.05 as level of significance. Statistical software SPSSRESULTS- 17.0 (IBM Corp.) was used for all analysis.

The descriptive characteristics of research subjects and mean ages (weeks) for each group are tabulated in Table1 and Table 2 respectively. The mean age at start of Pre-surgical Nasoalveolar Molding Therapy (2.47±1.77 weeks) was well within first month of birth. Infrared thermal data for all groups is given in Table 3. Thermal gradients over three given intervals were plotted in Figure 4. For paired T-test (Table 4), in PNAM group significant differences were found between temperature differentials at intervals i1-i2 (p=0.027) and i2-i3 (p=0.009), whereas no significant change was observed for dt1-dt3. For LPTP group.and NoPreTx groups, thermal differentials were significant for all three time intervals. No statistical significance was found in any of time intervals for CTRL

The results of one way ANOVA (Table 5) for thermography data of groups PNAM to CTRL state there were significant differences among the four groups. Moreover, Post Hoc Tukey HSD Multiple Comparisons test (Table 6) show statistical significance between PNAM-LPTP (0.013) and PNAM-NoPreTx (0.000), whereas the thermal differential (dt3) for PNAM group when compared to268 CTRL (0.171) was found insignificant. 10th World Cleft Lip, Palate & Craniofacial Congress DISCUSSION-

The representative infrared thermal patterns of the four groups (Figure 5) at 1 week post-surgery interval (i2) depicts a lower temperature in the cleft side compared to the non-cleft side. In the thermal pictures taken 1 month post- cheilorhinoplasty (i3) the PNAM group shows equalization of temperature, whereas the temperature still remains low in cleft side as compared to non-cleft side of LPTP and NoPreTx groups. Patients with PNAM had higher normalization of thermal gradient (Figure 6) between the cleft and non-cleft sides. Both the groups LPTP and NoPreTx showed insignificant change in thermal patterns even at one month post-surgical follow up. Thermal gradient for the control group (CTRL) was insignificant till the last interval. Tension being inversely proportional to temperature, the researcher subjectively established that reduction in tension occurred one month post cheilorhinoplasty between cleft and non-cleft sides of the infants who received PNAM therapy compared to the infants who presented later than 6 weeks after birth and received lip tape before surgery or only cheilorhinoplasty as treatment (Figure 4). The reason behind tension reduction in PNAM group is based on the fact molding therapy started early within 6 weeks of birth with maternal estrogen still triggering hyaluronic acid activity6, enabling interstitial tissue expansion23 and, clefts approximated within controlled period of appointments. Since, tissues are expanded and cleft lip-alveolus approximated, post-surgical healing would occur with less inherent tension in the sutured muscle plane. As the infants presenting late to the craniofacial center could not be benefited by PNAM therapy and tissue expansion phenomena never occurred, significant tension was seen even 1 month post-surgery. Treatment outcomes may differ even in children born with same kind of cleft and treated with identical procedures because of great genetic variability expressed by CLP patients.24 However, in this study many parameters were controlled by the researcher to reduce variation in results taking into account limited number of sample per group. Two subjects were excluded from the thermographystudy being diagnosed data. with pneumonitis and allergy to penicillin respectively, both the situations might have interfered accurate measurement of infrared

A classical paper on measurement of neonatal skin temperature using infrared thermography explains that head being a stable site is less responsive to environmental change than mean skin temperature.25 This study correlated with the method of using 1cm x 1cm square areas, as used in a previous research which thermographically visualized trumpet players’ warm up patterns over269 10th World Cleft Lip, Palate & Craniofacial Congress time.21 A new ΔT system was devised in a previous study and considered to be an important diagnostic parameter in staging a disorder and measuring outcomes.26 In the current study the researcher also applied the differential in temperature (dt), it being a better comparing tool than temperature per se, to measure thermal gradient between cleft and non-cleft sides over three intervals of time. The temperature differential (dt) of virtual cleft and non-cleft sides in the control group of this study had no significant differences (p>0.1, Table 4) at all three intervals of time and were consistent with the findings of the study in which the author studied temperature changes in healthy subjects over time and found no significant differences (p=0.7) between left and right sides.27 The researcher found the temperature (Table 3) in the repaired cleft side being lower than the non-cleft noted 1 week after surgery (i2 interval) because of obstructed microvasculature post-surgery. These findings were supported by previous study which concluded that the temperature in the affected regions of patients with peripheral vascular disorders was low in extremities due to obstructedCONCLUSIONS- arteries.28

Though a lot of authors condemn using PNAM therapy because of the issue of burden of care, use of pre-surgical molding for pre-surgical approximation of cleft, it was highly suggested by the researcher of this study on the basis of the benefits established by this research with the means of infrared thermography, majorly being reduction in pre and post-surgical tissue tension and better healing of scar post-surgically under less tension. Medical Digital Infrared Thermal Imaging (MDITI) is a successful means of assessing subjective tissue tension and/or tissue healing post-surgery. Further efforts to convert the subjective thermal readings to a more objective finite element thermo-elastic stress analysis should be taken in future. The researcher recommends use of infrared thermography in exploring and answering other research questions in craniofacial science for it being a non-contactREFERENCES- non-radiation diagnostic technique which has a promising future. 1 Vig, K. W., & Mercado, A. M. (2005). The orthodontist's role in a cleft palate-craniofacial team, Chapter 26. In T. M. Graber, R. L. Vanarsdall, & K. W. Vig, Orthodontics: Current principles and techniques, 4th Edition (pp. 1097-1121). Elsevier. 2 Murray, J. C., Daack-Hirsch, S., Buetow, K. H., Munger, R., Espina, L., Paglinawan, N., et al. (1997). Clinical and eidemiologic studies of cleft lip and palate in the philippines. Cleft Palate-Craniofacial Journal, 7-10. 270 10th World Cleft Lip, Palate & Craniofacial Congress 3 Grayson, B. H., & Maull, D. (2005). Nasoalveolar molding for infants born with clefts of the lip, alveolus, and the palate. Seminars in Plastic Surgery, 294-301. 4 Patil, P. G., Patil, S. P., & Soumil, S. (2011). Nasoalveolar molding and long-term postsurgical esthetics for unilateral cleft lip/palate: 5-year follow-up. Journal of Prosthodontics, 577-582. 5 Ezzat, C. F., Chavarria, C., Teichgraeber, J. F., Chen, J.-W., Stratmann, R. G., Gateno, J., et al. (2007). Presurical nasoalveolar molding therapy for the treatment of unilateral cleft lip and palate: A preliminary study. Cleft Palate Craniofacial Jounal , 8-12. 6 McNeil CK. Orthodontic procedures in the treatment of congenital cleft palate. Dent Rec (London). 1950 May; 70(5):126-32. 7 Matsuo, K., & Hirose, T. (1991). Preoperative non-surgical over-correction of cleft lip nasal deformity. . British Journal of Plastic Surgery, 5-11. 8 Grayson, B. H., Santiago, P. E., Brecht, L. E., & Cutting, C. B. (1999). Presurgical nasoalveolar molding in infants with cleft lip and palate. Cleft Palate Craniofacaial Journal, 486-498. 9 Bennun, R., Perandones, C., Sepliarsky, V., Chantiri, S., Aguirre, M., & Degliotti, P. (1999). Nonsurgical correction of nasal deformity in unilateralcomplete cleft lip: A 6-year follow-up. Journal of Plastic and Recontructive Surgery, 616-630. 10 Grayson, B. H., Santiago, P. E., Brecht, L. E., & Cutting, C. B. (1999). Presurgical nasoalveolar molding in infants with cleft lip and palate. Cleft Palate Craniofacaial Journal, 486-498. 11 Maull, D. J., Grayson, B. H., Cutting, C., Brecht, L., Bookstein, F., Khorrambadi, D., et al. (1999). Long-term effects of nasoalveolar molding on three dimensional nasal shape in unilateral clefts. Cleft Palate Craniofacial Journal, 391-397. 12 Cho, B. (2001). Unilateral complete cleft lip and palate repair using lip adhesion and passive alveolar molding appliance. Journal of Craniofacial Surgery, 148-156. 13 Pfeifer, T., Grayson, B., & Cutting, C. (2002). Nasoalveolar molding and gingivoperiosteoplasty versus alveolar bone graft: An outcome analysis of costs in the tretment of unilateral cleft alveolus. Cleft Palate Craniofacial Journal, 26-29. 14 Da Silveira, A., Oliviera, N., Gonzales, S., Shahani, M., Reisberg, D., Daw, J., et al. (2003). Modified nasal alveolar molding appliance for management of cleft lip defect. Journal of Craniofacial Surgery, 700-703. 15 Yang, S., Stelnicki, E., & Lee, M. (2003). Use of nasoalveolar molding appliance to direct growth in newborn patient with complete unilateral cleft lip and palate. Journal of Pediatric Dentistry, 253-256. 271 10th World Cleft Lip, Palate & Craniofacial Congress 16 Liou, E. J.-W., Subramanian, M., Chen, P. K., & Huang, C. S. (2004). The progressive changes of nasal symmetry and growth after nasoalveolar molding: A three-year follow-up study. Journal of Plastic and Reconstructive Surgery, 858-864. 17 Salyer, K. E., Rozen, S. M., Genecov, E. R., & Genecov, D. G. (2005). Unilateral Cleft Lip-Approach and Technique. Seminars in Plastic Surgery, 313-328. 18 Salyer, K. E., & Genecov, E. (1995). Surgical-orthodontic management of cleft patient from infancy to adulthood: 25 years experience. 49th American Cleft Palate Craniofacial Association Annual Meeting. Toronto: ACPCA, Pittsburgh, PA. 19 Vowell, S. (2009, March 19). Microfluidics: The Effects of Surface Tension. Retrieved from http://www.phys.washington.edu/~sharpe/486/vowell_f.pdf 20 Linehan, C. M. (1982). The effect of temperature on the tension responses of the anterior byssal retractor muscle (ABRM) of Mytilus Edulis. J. exp. Biol., 375-384. 21 Bertsch, M. (1998). Visualization of trumpet players' warm up by infrared thermography. Dissertation at the University of Vienna. 22 Marshal, M. N. (1996). Sampling for qualitative research. Family Practice, Vol.13, No.6, 522-525. 23 Hamrick, M. (1999). Chondral modelling theory revisited. J Theor Biol., 201-208. 24 Mølsted, K. (1999). Treatment outcome in cleft lip and palate: issues and perspectives. Crit Rev Oral Biol Med., 225-239. 25 Clark, R., & Stothers, J (1980). Neonatal skin temperature distribution using infra-red colour thermography. Journal of Physiology, 323-333. 26 Gratt B, Graff-Radford S, Shetty V, Solberg W, & Sickles E. A 6-year clinical assessment of electronic facial thermography. Dentomaxillofac Radiol. 1996, 247-255. 27 Christensen J, Vaeth M, Wenzel A. (2010). Thermographic imaging of facial skin--gender differences and temperature changes over time in healthy subjects. Dentomaxillofacial Radiology, 662-667. 28 Bhagavathiappan S. (2009). Infrared thermal imaging for detection of peripheral vascular disorders. Journal of Medical Physiology, 43-47. 29 Pickett BP, Burgess LP, Livermore GH, Tzikas TL, Vossoughi J. Wound healing. Tensile strength vs healing time for wounds closed under tension. Arch Otolaryngol Head Neck Surg. 1996 May; 122(5):565-8. FIGURE LEGEND-

Figure 1 a. UCLP Defect, b. PNAM Biomechanics, c. PNAM treatment result 272 Figure 2 Fluke Ti9 Infrared Thermal Imager 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 3 a. An infrared thermogram being taken at 1 foot distance from the target area, b. A thermogram generated by SmartView software ver 3.5.31.0 (c=cleft side, nc= non-cleft side)

Figure 4 Thermal Gradients dt1, dt2 dt3 over

three time intervals i1, i2, i3 respectively for all groups Figure 5 Repesentative thermograms at intervals i1, i2, i3 for all groups

Figure 6 Level of temperature normalization between cleft (c) and non- 273 10th World Cleft Lip, Palate & Craniofacial Congress

cleft (nc) sides 1 month post cheilorhinoplasty. Averaged difference between

dt2 & dt3 for all groups

274 10th World Cleft Lip, Palate & Craniofacial Congress

TABLE LEGENDS

Table 1 Descriptive Characteristics of Research Subjects

Table 2 Mean Ages in weeks

275 10th World Cleft Lip, Palate & Craniofacial Congress

Table 3 Infrared Thermography Data at i1, i2 and i3 intervals

Table 4 Paired T test for Thermal Differentials dt1, dt2 and dt3 at three time intervals of all groups

276 10th World Cleft Lip, Palate & Craniofacial Congress

groups Table 5 One way ANOVA of thermal differentials dt1, dt2 & dt3 for all the

Differentials Table 6 Post Hoc Tukey HSD Multiple Comparisons of Thermal

277 10th World Cleft Lip, Palate & Craniofacial Congress

Combo flap interposition in recurrent TMJ ankylosis

A. Thangavelu , K.Janarthanan, K. Bharathi Annamalai University, Chidambaram, India

Abstract:

Aims and objectives: Various interpositional materials have been used to prevent the recurrence after gap arthroplasty in temperomandibular joint ankylosis cases; most commonly used being the temporalis muscle and/ or fascia and free fat dermis graft. In this study, a combination of temporalis muscle and free fat dermis interposition was used and its efficacy in preventing reankylosisMaterials and was methods: evaluated.

Two cases of unilateral temperomandibular joint reankylosis were evaluated with a follow up of 3months. Results: Regular clinical and radiological follow up was done for 3 months during which the average mouth opening of 32 mm was maintained, with good occlusion and proper function. Post op Orthopantomogram showed a well maintained gap between the newly created articular surfaces indicating no evidence of reankylosis in both cases. Conclusion: In this short term prospective study, we found that this combo interposition of temporalis muscle flap and free fat dermis graft in recurrent ankylosis along with adjuvant indomethacin therapy produced satisfactory resultsKeywords in achieving adequate mouth opening and jaw function.

Introductioncombo interposition, reankylosis , free fat graft, temporalis muscle flap

Temperomandibular joint (TMJ) ankylosis is a pathologic condition where 278the mandible is fused to Glenoid fossa by bony or fibrous tissues. This interferes 10th World Cleft Lip, Palate & Craniofacial Congress

with mastication, speech, oral hygiene, and normal life activities. Trauma is the most common cause of TMJ ankylosis, followed by infection. Ankylosis of the TMJ usually develops before the age of 10 years, but can be found at any age; the incidence peaks in patients aged 20–30 years.(5) Ankylosis of the TMJ can be classified based on a combination of the site (ie, intra-articular or extra articular), the type of tissue involved (ie, bony, fibrous, or fibro-osseous), and the extent of fusion (ie, complete or incomplete)(6). Surgery is the protocol for management. If insufficient mandibular bone is removed , a recurrence of the ankylosis can be expected. Interpositioning is recommended after gap arthroplasty as a means of preventing re-ankylosis. For establishment of a ‘functional pseudoarthrosis’, it is essential to consider an autogenous interpositional graft(2). Different interposition grafts have been used, such as the temporomandibular meniscus, temporalis muscle/fascia, fascia lata, skin, auricular cartilage, fat, alloplastic materials and xenografts (4). Dimitroulis interposed dermis fat graft, taken from the groin in the TMJ. The presence of dermis in the graft keeps the fat intact by preventing its disintegration. The free fat graft has more chances of its resorption (3). The temporalis muscle is the most widely used among interpositional material due to its ease of usage, dependable blood supply, proximity to temporal joint, good functional results, minimal risk of facial paralysis, successful clinical results and minimal complications. While the versatility of temporalis as interpositional material is undoubtful, reports of persistence of postoperative pain, re-ankylosis and necessity for aggressive postoperative physiotherapy (7). A dual interposition was tried in our study by placing both fat and muscle inMaterials the joint and followed methods by aggressive physiotherapy.

Two cases of unilateral temperomandibular joint reankylosis were evaluated. First case being a 14 year old female and the second being a 7 year old male. Both patients had undergone a surgery for ankylosis at our centre before 2years. At presentation the maximum incisal opening was almost 0 mm. Patients were assessed both clinically and radiologically (fig 1,2). The protocol consisted of the following steps: 1) the creation of a minimal gap of 15 to 20 mm by gap arthroplasty; 2)free fat dermis graft on the medial aspect; 3) temporalis muscle interposition on lateral aspect;4) postoperative indomethacin for 1 week 5) immediate aggressive physiotherapy. Details of the previous surgery regarding the cause, extent, duration, maximal incisal opening were recorded. Gap arthroplasty with interposition279 10th World Cleft Lip, Palate & Craniofacial Congress of free fat dermis harvested from abdomen was done in the first procedure. Following this, reankylosis had developed in a span of two years likely due to inadequate physiotherapy and infrequent check up.

fig.1 pre op mouth opening fig.2 pre op OPG Surgical procedure:

A second surgery to correct reankylosis was planned under nasoendotracheal blind intubation. An Alkayat Bramley incision was placed and adequate exposure of zygomatic arch, sigmoid notch and callus was made and a gap arthroplasty of 1.5 to 2cm was done by resection of callus. Immediate intraoperative mouth opening was measured as 28mm and 32mm respectively. Free fat dermis was harvested from lower abdomen (fig3). The graft was placed in saline immediately after its removal from donor site. Now, anterior two thirds of temporalis muscle attached to the coronoid process was raised, rotated and tunelled beneath zygomatic arch (fig4). First, the free fat dermis graft was tucked anteromedially into the arthoplasty site and followed by interpositioning the temporalis muscle flap posterolaterally. Anchoring of interpositional material was done periarticularly using 3-0 vicryl sutures. A mastoid bandage to reduce postoperative edema was placed in both cases and removed at discharge. Indomethacin therapy as an adjuvant was started in the immediate postoperative period in divided doses of 25mg twice daily with regular monitoring of renal function tests (RFT). Indomethacin was withdrawn on 2nd post op day in the first case due to rise in the RFT values. It was however continued upto one week in the second case. Physiotherapy was started from first280 post operative day in both the cases. 10th World Cleft Lip, Palate & Craniofacial Congress

Results:Fig.3 free fat dermis graft from abdomen fig.4 temporalis muscle flap interpositioning

Patients were assessed for maximal interincisal opening, protrusive and lateral movements, involvement of facial nerve, pain, swelling and infection. Both of them had aetiology of trauma in their early childhood. Inability to open mouth led to poor oral hygiene, problems in speech and feeding due to which the subjects were underdeveloped for their age. Severe crowding and a midline shift towards the ankylosed side was seen in both cases. Previous attempts of gap arthroplasty and free fat interposition was a failure in both cases. Recurrent ankylosis was mainly attributed to the inadequate interpositional material in the medial aspect of joint. Temporalis muscle flap was sandwiched more the lateral aspect leaving the medial portion, which is more prone to reankylosis, devoid of any interposition material. To overcome this, we have tried combo flap interposition where in the free fat was tucked into arthroplasty site on the medial aspect and temporalis muscle flap was interpositioned on the lateral aspect. Indomethacin therapy was given as an adjuvant. A post operative mouth opening was measured to be average of 32mm in both cases. Follow up of after 3 months showed no evidence of recurrence till date (fig 5,6)

fig.5 post op mouth opening fig.6 post op OPG 281 10th World Cleft Lip, Palate & Craniofacial Congress Discussion:

Temperomandibular joint (TMJ) ankylosis is a devastating problem that results in limitation of mandibular movements leading from mild to complete inability in opening mouth and associated difficulties in speech, feeding, poor oral hygiene and underdeveloped growth(7) which impairs the normal social life of the individual. Trauma is the leading cause for TMJ ankylosis and the treatment of this condition poses a considerable challenge because of the high incidence of recurrence. A combination of surgery and aggressive physiotherapy is required to maintain mandibular function and prevent recurrence (12). Various methods can be used. Arthroplasty without interposition requires a gap of 10 to 20 mm and often results in mouth deviation and has a high recurrence rate of about 53%. For this reason it seems better to create a minimal gap and then place interposition to prevent recurrence caused by osteoblastic growth between the abraded bone surfaces(4). At present, there is no ideal interpositional graft. The problems encountered with the present autogenous grafts are: muscle shrinks and fibroses, fascia lacks bulk, cartilage tends to fibrose. Dimitroulis interposed dermis-fat grafts in 11 patients for the management of temperomandibular ankylosis, and identified only one case of reankylosis.(1). On the other hand, the temporalis muscle flap was seen to be an ideal interpositional material due to its close proximity to the site, good vascular supply, ease of access to the condyle area , minimal risk of nerve damage and good functional results(11) However, due to higher incidence of recurrences we, in this study, have evaluated the surgical outcome of a combined interposition of temporalis muscle and free fat dermis graft from abdomen in two cases of recurrent ankylosis of temperomandibular joint. To overcome the problem of recurrence in these cases indomethacin was given in the immediate postoperative period as an adjuvant . It should be started within 24 hours after surgery, because heterotropic ossification starts around 16 hours and reaches peak at 48 hours. Although indomethacin is a known prophylaxis for prevention of heterotropic bone formation after hip arthroplasty (12) , its use in treating recurrent temperomandibularConclusion: joint ankylosis is relatively new.

Inadequate interpositioning material in the medial aspect of joint space is the main culprit of reankylosis. The dual interposition of free fat dermis in anteromedial282 portion and temporalis muscle in posterolateral joint space along 10th World Cleft Lip, Palate & Craniofacial Congress

with indomethacin therapy as an adjuvant will go a long way in successful managementReferences: of reankylosis. 01. G. Dimitroulis:The interpositional dermis-fat graft in the management of temporomandibular joint ankylosis. Int. J. Oral Maxillofac. Surg. 2004; 33: 755–760 02. A. Thangavelu, K. Santhosh Kumar, A. Vaidhyanathan, M. Balaji, R. Narendar: Versatility of full thickness skin-subcutaneous fat grafts as interpositional material in the management of temporomandibular joint ankylosis. Int. J. Oral Maxillofac. Surg. 2011; 40: 50–56. 03. Sunil Gaba a, Ramesh Kumar Sharma a,*, Vidya Rattan b, N. Khandelwal ca The long-term fate of pedicled buccal pad fat used for interpositional arthroplasty in TMJ ankylosis*Journal of Plastic, Reconstructive & Aesthetic Surgery (2012) 65, 1468e1473 04. Bulgannawar et al. Use of Temporalis Fascia as an Interpositional Arthroplasty in Temporomandibular Joint Ankylosis: Analysis of 8 Cases J Oral Maxillofac Surg 69:1031- 1035, 2011 05. E.A. Al-Moraissi, T.M. El-Sharkawy, R.M. Mounair, T.I. El-Ghareeb: A systematic review and meta-analysis of the clinical outcomes for various surgical modalities in the management of temporomandibular joint ankylosis. Int. J. Oral Maxillofac. Surg. 2014 06. Ayman F. Hegab, PhD : Outcome of Surgical Protocol for Treatment of Temporomandibular Joint Ankylosis based on the Pathogenesis of Ankylosis and Re-Ankylosis. A Prospective Clinical Study of 14 Patients: J Oral Maxillofac Surg -:1-12, 2015 07. S. M. Balaji: Modified temporalis anchorage in craniomandibular reankylosis. Int. J.Oral Maxillofac. Surg. 2003; 32: 480-485. 08. Behcet EROL, Rezzan TANRIKULU, Belgin GORGUN : A clinical study on ankylosis of the temporomandibular joints; Journal of Cranio-Maxillofacial Surgery (2006) 34, 100– 106 09. Dimitroulis G, Slavin J. Histological evaluation of full thickness skin as an interpositional graft in the rabbit craniomandibular joint. J Oral Maxillofac Surg 2006: 64: 1075–1080. 10. Mehrotra D, Pradhan R, Mohammad S, Jaiswara C. Random control trial of dermis fat graft and interposition of temporalis fascia in the management of temperomandibular ankylosis in children. Br J Oral Maxillofac Surg 2008: 46: 521–526. 11. Chossegros C, Guyot L, Cheynet E, Blanc JL, Cannoni P. Full-thickness skin graft interposition after temperomandibular joint ankylosis surgery. A study of 31 cases. Int J Oral Maxillofac Surg 1999: 28: 330–334.

283 10th World Cleft Lip, Palate & Craniofacial Congress 12. Bhatt K1, Pandey S1, Bhutia O1, Roychoudhury A1. Use of indomethacin as an adjuvant to surgery for recurrent temporomandibular joint ankylosis in adults. Natl J Maxillofac Surg. 2014 Jul-Dec;5(2):198-201.

284 10th World Cleft Lip, Palate & Craniofacial Congress

Modification of Ryle's nasogastric tube

Manay Roshini Srinivas Department of Oral and Maxillofacial Surgery, Saveetha Dental College, Chennai.

Abstract:

A nasogastric tube is a narrow bore tube passed into the stomach via the nose. It is used for short- or medium-term nutritional support in patients, young or old, who are not able to feed on their own either due to congenital defect in the oro-pharynx region or during the post-operative healing period. Although the current design of Ryle’s tube has been used over decades. One-third of the tube, which is seen externally can not only be a source of infection but also have a psychological impact on the patient’s well-being. Hence to overcome this, a modification of the current design is done where the external segment of the tube can be detached and attached only when feeding is required. Keywords: Enteral Feeding catheter, Nutritional Support in Primary Care, NutritionalI. FIELD OF SupportINVENTION in Hospital, Nasogastric tube, Ryle’s nasogastric tube

The invention is based on a modification of the design of eternal feeding tubeII. DESCRIPTION used in the field OF THEof medicine PRIOR ART

A nasogastric tube is a temporary tube placed through the nose into the stomach. It is be used to remove fluid from the stomach, or used for nutritional support in patients who are not able to feed on their own either due to congenital defect in the oro-pharynx region or during the post-operative healing period after III. DRAWBACK a surgery or OFduring THE radiationEXISITING therapy STATE in OF patients ART diagnosed with cancer.

The current design of Ryle’s nasogastric tube has been used over decades all over the world, especially in patients undergoing going oncology (cancer) treatment. One-third of the tube is left hanging from the patient’s nostrils thethrough patient. which liquid food/supplement is passed. This can harbour infection if not maintained well. And also have a psychological impact of being diseased on 285 10th World Cleft Lip, Palate & Craniofacial Congress IV. NOVELTY

at the Thetime proposed of extubation design by is anaesthetist such that the The external internal tube tube can is positionedbe detached at andthe easily snapped on to the internal tube which is inserted into the patient nostrils level of the nostrils with semi-rigid support with the help of the two external allowsnose clips the whichpatient adhere to be free to the of analar external and collumela tube hanging respectively. outside Externaltheir nostrils. tube can be detached and attached only when required to the internal tube. This

( Fig.1) Fig 1. A schematic representation of nasogastric tube in a human aero- digestive tract view.

V. A DETAILED DESCRIPTION

The proposed design is a user friendly, hygienic and aesthetically appealing nasogastric tube for feeding nutritional solution to patients who are unable to feed on their own. The tube is devoid of fixed external extension of the tube from the nostrils unlike the current design, thereby eliminating the problemsIt comprises of infection of the following. and psychological stigma.

A. A hollow flexible internal tube of predetermined length comprises of two ends, closed top end and opened bottom end wherein plurality of tiny holes disposed on circumferential surface at predetermined length above the bottom end of the internal tube for easy discharge of nutritional solution into stomach and the closed top end is closed by means of thin layer with a slit. B. A hollow cylindrical rigid support of predetermined length disposed on outer circumferential surface on the top end side of the internal tube. (Fig 2)

Fig 2. Internal tube. 286 C. An aesthetic nose clip (fig 3.) comprises of a ring with pair of clips 10th World Cleft Lip, Palate & Craniofacial Congress

the internal tube in front of the rigid support. wherein the ring is disposed on outer circumferential surface on the top end of

Fig 3. Internal tube inserted and positioned at the nostrils

insertedD. intoA hollow the top flexible end of detachablethe internal external tube through tube the(fig. slit 4).of during predetermined feeding length adapted to be

Fig 4. External tube inserted Fig. 5. Storage box with 10 ml syringe E. A storage container (fig 5.) for storing the external tube when feeding

is complete is provided along with the set 287 10th World Cleft Lip, Palate & Craniofacial Congress The internal tube is to be inserted into the patient nostrils at the time of extubation. The bottom end of the internal tube is positioned inside

the stomach and the top end of the internal tube is positioned at the level of the nostrils with the rigid ensuresupport the with support the help of ofthe the internal two clips tube that to nostrilsadhere to the alar and collumela region. The rigid support

and comfortabilityThe slit on the to patient top end of the said internal tube opens when the said external tube being

inserted during feeding and the slit closes when the said external tube is removed after feeding thereby preventing the infection to enter into the internal tube The nasogastric tube - internal tube and external tube is made of materials comprising of polyvinylchloride (PVC). The diameter of the external tube is lower than the diameter of the top end of the internal tube which aids in the easy-snapVI. SUMMARY. fit insertion of the tube.

With the change in design, it is not only user friendly and hygienic, it also helps those who are on it for long term to re-integrate themselves into the society and carry forward their routine activities by demolishing the stigma the patients carry on themselves. (Fig 6) The simplicity of the proposed design will definitely bring about a change in the quality of lives of our patients. Fig 6. A comparison of the external apperance with the current (left) design and proposed (right) design

288 10th World Cleft Lip, Palate & Craniofacial Congress

Observational prospective study to correlate incidence of cleft palate fistula with anatomical variation of cleft palate

Umang Kothari 3rd Year DNB, Dept. of Plastic Surgery, Medanta-The Medicity, Gurgaon, Haryana, India

1.1 Introduction:

Cleft lip and palate (CLP) is the most common congenital deformity of the craniofacial region with an average world-wide incidence of 1 in 700[1].Its incidence in Asian population is reported to be around 2.0/1000 live births or higher.[2] In India, though national epidemiological data is not available, many studies from different parts have reported a variation in the incidence of cleft anomaly. At a rough estimate there are over a million cleft patients in India. Every year over 35,000 new such patients are added to the community.[3] Cleft and Craniofacial Anomalies are unique congenital anomalies. The management requires a large team of specialists working in tandem to create a normal individual – physically, psychologically and socially. High fistula rate and inferior speech results are common after primary repair of substantially wide cleft palates. Incidence of palatal fistula ranges from 0 to 63 % and recurrence is common.[4-9]. Cleft palate fistula is defined as failure of healing or breakdown of primary surgical repair .[6]various factors have been implicated in the development of cleft palate fistulas including the type of repair,Anatomy of cleft palate,Surgeon performing the operation. Respiratory1.2 Review ofinfection,Sex Literature: Age of repair.[4-9]

Studying the history of cleft palate repair is an important step in the prevention of complications. Johann Friedrich Dieffenbach in 1816[10] first to elevate soft-tissue flaps instead of paring the edges only. However, he used lateral palatine osteotomy to move the palate medially which was a rather radical surgical procedure. 289 10th World Cleft Lip, Palate & Craniofacial Congress

von Langenbeck was the first to raise pedicled mucoperiosteal flaps based on the sound knowledge of anatomy . Veau in 1927 described the importance of nasal layer repair, which is being practiced until date. He also described breaking of the hamulus and push back procedure, which probably has lost its importance in the present day.[11] Independently, Wardill and Kilner[12] developed the more radical push back procedure in 1937, which was becoming popular in UK. Of course, with time this technique has proved to be disastrous due to the extensive scarring and the raw bony surface that it leaves after surgery. With these disastrous results and knowledge of early surgery and scarring causing growth problems. Schweckendiek and Doz[13] technique of two-stage repair where in the hard palate is closed later than 5-7 years of age. A study by Bardach et al.[14] presented his two flap technique suitable for wide cleft in the presence of alveolar cleft and with less incidence of fistula. Later, superiorly based vomer flap was used to close hard palate as a single layer with the expectation of better growth outcome.[15] The latest addition to the cleft palate operation is “Radical Muscle Mobilization” developed simultaneously by, Sommerlad and Cutting et al. However, while Sommerlad uses the operating microscope for the muscle dissection and sling formation, Cutting et al. uses the magnifying loupes. The authors claim better speech outcomes following radical muscle dissection and veloplasty.[16,17] Interpreting and comparing fistula incidences has been difficult in previous years. Many articles discussing fistula incidence did not mention a definition of fistula, thereby making comparison between incidence numbers unreliable.Cohen et al. (1991) were the first to describe the type of fistula that they included in their study.[5] Muzaffar et al. (2001) defined a fistula as ‘‘a failure of healing or a breakdown in the primary surgical repair of the palate,excluding intentionally unrepaired fistulas.’’[6] Factors that have been proposed to influence the fistula rate are Veau class, surgeon experience, age at repair, gender, ethnicity, and whether the cleft is part of a syndrome or a range of other malformations. Only Veau class [5,6], surgeon’s experience [4,5] and certain syndromes [9] have been found to be significant predictors of fistula incidence. Although numerous studies have suggested that cleft width can be associated with the rate of fistula formation.[5,6] by showing a relation between290 Veau class and fistula occurrence, others could not demonstrate this association[4]. 10th World Cleft Lip, Palate & Craniofacial Congress

Recently an article was published in which palate and cleft measurements were related to fistula rate . The main conclusions of that article were that a preoperative cleft width of >/= 15 mm and a ratio of cleft width to the sum of the palatal shelves’ width of >/= 0.48 have a statistically significant risk of fistulization.[7]1.3 Aims & Objectives: Aim:

PrimaryTo Objective: correlate incidence of Cleft Palate fistula with width of cleft palate. palate. To correlate incidence of Cleft palate fistula anatomical variation of cleft Secondary objective:

To correlate incidence of Cleft Palate fistula with Subjective assessment of1.4 quality Material of mucosa, & Methods: tension over stitch lines and quality of muscle. Type of Study:

SampleObesrvational Size: Prospective study.

Study 45Place and duration: is earlier.Primary cleft palate repair done in Plastic Surgery Department of Medanta-The Medicity till 31/12/2015 or sample size is achieved whichever Inclusion Criteria: palate repair. All patients with Cleft lip and palate or cleft palate undergoing primary Exclusion Criteria:

Redo Palate reapir, Intensionally unrepaired Palate Nasoalveolar Fistula Syndromic Cleft palte patients. 291 10th World Cleft Lip, Palate & Craniofacial Congress Methodology:

After including patients in study intraoperative assessment done of anatomy of cleft palate as per Standard of care. In this study surgeon And Operative methods are kept constant. In Cleft palate intraoperative measurements were taken with Stainless steel wire and Plastic ruler scale . shape of cleft,cleft width,Palatal shelf width,distance between posterior pharyngeal wall and uvula.length of hard palate.length of cleft palate.Total length of palate were noted. operatingIntraoperative surgeon. assessment of Quality of Repair (Oral Mucosa,Quality of Nasal Mucosa,Tension over Stitches,Quality of muscles) assessed as per

Length of total palate after repair and distance between posterior pharyngeal wall and uvula after repair is noted. days. Follow up done within first 15 days.next follow up visit on 45 +/- 15

If follow up visit suggestive of fistula ,Patients were assessed for Location, Size ,clinical significance of fistula. Intervention for fistula if required any was planned and documented.

292 10th World Cleft Lip, Palate & Craniofacial Congress

External Rhinoplasty for cleft nasal deformities

M.R.MUTHUSEKAR , Vinod Narayan, M.F. Baig, BALA JAGANNATH GUPTA.B Saveetha dental college and hospital, No:162, poonamalle high road,chennai-77, Tamil nadu, India

INTRODUCTION:

Nasal deformity in cleft lip type presents one of the most complex surgical challenges. Based on a common theme with numerous variations of degree and additions it is the ultimate three-dimensional soft and hard tissue puzzle with an extra feature of relentless change from infancy to adulthood. This nasal deformity is congenital malformation manifested by anatomic arrangement in the nose and its underlying facial skeletal platform always changing until maturity .The extent of the nasal deformity is related to the severity of the cleft lip .All tissue layers including external skin, cartilage, vertebral lining and skeletal platform are involved. Repair of the cleft type nasal deformities requires attention to all tissue layers. Correction of cleft lip nasal deformities can be divided into primary and secondary repairs. Primary repairs are classified are those performed at the time of initial cleft lip repair and secondary are those done when the nose is normal or near normal in size later in life. Cleft lip nasal deformity can occur either in unilateral or bilateral cleft lip type and each abnormality having distinctly different characteristics. The severity of the primary nasal deformity is related to the severity of the cleft lip. The severity of the secondary nasal deformity however depends on the type of primary deformity, the type of lip repair done and any prior attempts at surgical correction of primary nasal deformity. Secondary nasal deformity correction can be best performed through grafts.an open rhinoplasty approach that allows better diagnosis of specific cleft lip deformity. This approach also allows accurate placement of structural cartilage

This case series enumerates the specific secondary deformities with both unilateral and bilateral cleft lips and outlines precise techniques used to correct cleft lip nasal deformities. 293 10th World Cleft Lip, Palate & Craniofacial Congress Aims and objectives: ●●

To reposition the lower left cartilage superiorly and medially to a more ●● normal anatomical position. ●● To create the tip of the nose. ●● To achieve a symmetric projection of both alar domes. ●● To elongate the columella when necessary. ●● To add structural support by means of cartilage grafts. MaterialsImproves and themethods: patient’s confidence by improving esthetics.

This study was pertaining to the secondary correction of cleft lip type nasal deformity was conducted in Saveetha dental college and hospital, Chennai. This study done on 5 cases all who presented with cleft lip type deformity. All of them were treated by open rhinoplasty approach. Among the 5 cases 4 were males and 1 female patient .The age range of the case was from 13-18 years with an average age group of 15 years .Out of the 5 cases, 4 were unilateral cleft lip type nasal deformity and 1 was a bilateral cleft lip type deformity. A detailed history was taken with much emphasis on the chief complaints, where were unpleasant appearance of the nose, functional defect and sometimes both. Past history of cleft lip and cleft palate correction was noted down. The external internal examination included assessing the symmetry of the face, symmetry of the nose in relation to eyes, lips and upper and lower jaws, estimation of nasolabial angle, examination of nasal dorsum, assessment of the tip projection and support, columellar length and deviation, alar flare, the activity of the muscle structure and the amount of scar present due to primary correctionThe internalof the lip. examination was done under illumination using tudigums retractor to observe the nasal deviation, hypertrophied turbinates, and assessments of internal nasal valve for obstruction and mucous membrane. In all the cases routine orthopentamograph, para nasal sinus view and occlusal radiographs taken to see the underlying bony support and conditions ofProcedure: the teeth. Photographs were taken in 3 views and filed.

All the cases were done under hypotension general anesthesia and aseptic technique294 were followed. 10th World Cleft Lip, Palate & Craniofacial Congress

●●

●● An open rhinoplasty procedure was carried out in all the cases. Surface marking of the important anatomical landmarks like bony dorsum, ●● upper lateral cartilages and alar cartilage are done using marking ink. Infiltration anesthesia with 30 gauze needle using 1:80,000 adrenaline is ●● injected. Through a transcolumellar incision skelitinization of the cartilage done, then the medial crus were brought near, graft were placed where never necessary. These graft were stabilized with 4-0 vicryl sutures. Then the ●● mucosal incision closed 4-0 vicryl. ●● Columella length was increased when necessary by “V-Y” closure. Alar base width on the cleft side reduced when necessary by “Y-V “ closure and skin incision at alar base margin and columellar base were sutured ●● using 6-0 proline sutures. Intranasal packing done using sofra tulle, which was retained for 24-48 ●● hours. Streristrip dressing was given over the nose for a week. ●● In all the cases antibiotics ampliclox-500mg TID for 7 days ●● Decran BID gradually tapered. Inj voveran 75 mg S.O.S post operatively. Postoperatively after 2 days otrivin nasal drop were prescribed to relieve ●● nasal congestion, healing in all patient was uneventful. ●● The Protocol followed in our institute as follows: ●● SelectionDiagnosis of and all casestreatment with cleft planning lip type after nasal examination deformity. of the nose and analysis. ●● under general anesthesia. General medical examination to assess the fitness to undergo surgery ●●

●● Surface marking of the important anatomical landmarks. ●● Selection of the incision. ●● Retraction of the dorsal skin from underlying bones and cartilage. ●● Repositioning of the depressed alar cartilage. ●● Straightening of nasal septum. Increasing the length of the columella when needed. 295 10th World Cleft Lip, Palate & Craniofacial Congress

●●

●● Placement and fixation the graft. ●● Redraping the skin over newly constructed framework. ●● Suturing of incision. DiscussionNasal packing.

Functional and esthetic correction of the cleft lip type nasal deformity is one of the most challenging rhino surgical procedures. Since this varies widely in its severity, it requires a great deal of individualization and special considerations as to the timing surgery and in the selection of an appropriate surgical method, especially with concern for the lower lateral cartilage and the nasal base (Mc Comb 1985) (1). Despite the application of rhinoplastics techniques in primary cleft lip repair ,the need for secondary nasal correction remains (Rodolphe meyer) (2). The rational of this study was based on the desire to reposition the structure as accurately as possible,therby satisfying both the patient and the surgeon. All the 5 cases which are included in this study ,are adult ,in whom the development of the nose was complete .The reason for not operating on children is for the fear of interfering with the continued development of the nose and also their inability to co operate in the postoperative period. The approach used in all these 5 cases is open or external rhinoplasty technique .The external rhinoplasty enabled to achieve good exposure, visualization of structures, evaluation of complete deformity, directly removal of scar and fibro fatty tissue, accurate trimming and suturing of the nasal graft. Additionally, the external incision can provide a source of tissue, which is important in increasing columellar length. Patient who demonstrate mild to moderate mid face retrusion yet have a normal dental occlusion as a result of previous orthodontics treatment, require pyriform fossa cartilage graft to improve their midface deficiency and to support the cleft alar base. Among the 4 unilateral cleft lip, nose all 4 approached with inverted “V” transcolumellar approach. The scar was hidden and unnoticeable in all cases. In 2 cases where additional columellar skin was required we used modified V-Y incision of bardach, where we could satisfactorily achieved coloumellar lengthening .To substantiate our point view, GARY FRIEDMAN et al (1983-84) (3) 296 10th World Cleft Lip, Palate & Craniofacial Congress

Used external approach in 103 patient, 83%needing primary rhinoplasty and 17%needing secondary rhinoplasty . At one year follow up, he reported only one patient had depressed scar. His study shows that, transcolumellar scar and external rhinoplasty approach can be used widely. The anataomic charcteristics of the unilateral cleft lip nasal deformity are associated with gross nasal asymmetry of the lower third of the nose. The cleft ala is usually asymmetrical and displaced laterally, inferiorly and posteriorly. It is this asymmetry in three planes that makes precise surgical correction difficult. We in our approach to the unilateral cleft lip nasal deformity used SALYERS●● (1986) (4) 4 steps procedure. ●● Dissection of the lower lateral cartilage from the skin and the lining. Shifting and repositioning of the lower lateral cartilage and redrapping ●● skin and lining. ●● Creation of a symmetric alar domes and nasal tip. Creation of a symmetric alar base. After dissecting the lower lateral cartilage on the cleft side completely, we repositioned it to a more symmetrical position .A similar medial and superior advancement vestibular skin into a more elevated position has been proposed by RESS (1966) (5). The flattened dome is their symmetric position is more likely to be assured post operatively. HOLT sutures the mobilized lower lateral cartilage to the ipsilateral upper lateral cartilage. We prefer this to maintain the position of the lower lateral cartilage in its new position. Some of the noses, which needed more tip projection, were obtained by septal grafts. In three cases, we used septal cartilage. This is because of its inherent properities such as shape, elasticity and easy in harvesting. Through the same site, perichondrium is exposed, a window septal graft is taken and perichondrium is sutured back. Advantage of window graft is postoperative nasal septal deviation is preserved as the shape of the septum is not disturbed. For the correction of the bilateral cleft lip we must take into account the fact that there are numerous patho-anatomic deformities present. We in our study have taken into consideration the analysis offered by HOFFMAN and LIERIE(1949,1957) (6) and by DENECKE (7) and MEYER (2) (1964,1967).

297 10th World Cleft Lip, Palate & Craniofacial Congress

●● They are: ●● Excessive shortness of the columella. Abnormally laterally displaced angles of the lower lateral cartilage, creating ●● a sharp and producing an oblique oval position of both the naries. Broadness of the lobule with tendency of bifid of the tip (2). In this study, only one case of bilateral cleft lip deformity has been treated. Since there was excessive scar, a modified POTTER’s (8) incision can be used; instead we used a inverted V trans columellar approach and a modified V-Y incision to lengthen the columella. An excellent columellar lengthening was achieved by doing this. MAEDE (9) considers free grafting of segment from the auricle to the columella particularly suitable for secondary correction in adults. Instead we have used only the septal grafts. Alar base was reduced by using modified swellingY-V incision and also on the replase cleft from side. repositioning. In all five cases after rhinoplasty procedure, nasal packing was done with sofra tulle. This has helped in reducing bleeding, SUMMARY AND CONCLUSION:

Secondary cleft nasal deformity involves repositioning of the lower lateral cartilage on the cleft side to raise the dome, lengthening the columella and bringing it towards the midline and correcting any asymmetries of the nasal floor,like reducing the width of the alar base on the cleft side .Additional structural support in the form of cartilage grafts is necessary in order to achieve the desired projection and angulation .This often requires open approach ,Which provides maximal exposure for suture fixation of columella struts, vertical division and reconstitution of the lower lateral cartilage and accurate placement and suturing of nasal tip graft. Hence we have used open rhinoplasty procedure in all our cases. In all the cases dealt with satisfactory both in surgeon and patients point of view. The immediate postoperative complication was incidence of nasal congestion, which lasted for 1 week. Prolonged edema of nasal tip following open rhinoplasty procedure gradually subdued with time usually 3 months. To conclude there is no single correction of cleft lip nose, which is satisfactory for the surgeon or the patient .It is important therefore that a combination of methods should be available in every case to minimize if not completely correct the deformity. 298 10th World Cleft Lip, Palate & Craniofacial Congress BIBLIOGRAPHY: 01. Mc COMB H . Primary correction of unilateral cleft lip nasal deformity. A 10- years review . Plast.Reconstr.Surg.1985;6:792-797. 02. RUDOLPHE MEYER. The difficult nose. A Textbook on secondary and functional rhinoplasty.1992;335-356. 03. GRAY P.FRIEDMAN. A fresh look at open rhinoplasty and technique .Plast.Reconstr. Surg.1973.Ronald P Gruber.1986;680-82. 04. SALYER K. Primary correction of the unilateral cleft lip nose. A 15 year experience. Plast. Reconstr.Surg.1986;77:558-566. 05. REES T.D ,GUYEL G,CONVERSE J.M Repair of the cleft lip nose, addendum to the synchronous technique with full thickness skin grafting of the nasal vestibule. Plast Reconstr.Surg 1977;37-47. 06. HUFFMAN W.C. LIERLE D M. The repair of the bilateral cleft lip. Plast.Reconstr. Surg.1949;4:489. 07. DENECKE H .J Arch.Ohr.Nas-U-Kehlk-Heilk.1962;180:466. 08. POTTER .J Some nasal tip deformities due to alar cartilage abnormalities. Plast Reconstr. Surg. 1955;15:502. 09. MAEDE R .J Composite ear graft for correction of columella. Composite ear grafts for the correction of nasal deformities associated with cleft lip& other congenital &post traumatic deficiencies of the columella. Plast .Reconstr.Surg.1959;23:134.

PICTURES:

TRANSCOLUMELLAR INCISION

299 10th World Cleft Lip, Palate & Craniofacial Congress

TRANSCOLUMELLAR INCISION

SKELETINISATION OF CARTILAGE

HARVESTING THE GRAFT

300 10th World Cleft Lip, Palate & Craniofacial Congress

STABILIZATION OF THE GRAFT

PRE OPERATIVE:

POST OPERATIVE

301 10th World Cleft Lip, Palate & Craniofacial Congress

Follicular-Unit Hair Transplantation to correct Cleft Lip Moustache Alopecia using a new Atraumatic Technique for Hair Implantation

S. A. K. Uroof Rahamatullah Senior Reader, C. K. S Dental College and Research institute, Tirupathi, Andhra Pradesh, India

Abstract Background

We strive to achieve a functional and aesthetic repair in cleft lip patients bestowing them with all natural landmarks and making the evidence of the repair undetectable for better quality of life and complete social acceptance. The last to offer is a moustache for an adolescent male cleft lip patient. Aim: The current study is a review of moustache restoration carried out for patients afterMaterials cleft lipand repair. Methods It includes 2 cases with a follow-up of 6 months to 2 years.

Follicular unit extraction using 0.9 mm motorized punches is the preferred technique. Alternative method is follicular unit transplant strip technique, where individual hair follicles are dissected for a 0.5-0.6 cm × 5-6 cm strip of scalp.Results Each follicle serves as a micro graft. of grafts.Hair growth along the scar is delayed, it begins 4-5 months after the transplant, and complete growth is seen by 6 months. There can be 7-10% loss Conclusion

Follicular unit micro grafting can be used for restoration of moustache after a complete cleft lip repair. Moustache provides animation of the face, restoration of aesthetic landmark, taking away the typical cleft lip look and buildingKeywords up confidence of the patient.

302 10th World Cleft Lip, Palate & Craniofacial Congress

Cleft lip, cleft lip scar, follicular unit extraction, follicular unit transplant, hairIntroduction transplant, moustache

Cleft lip scar is still an unavoidable and afflictive problem for cleft lip patients, al-though many operative procedures have been developed and established for either primary or secondary repair. To improve these conspicuous cleft lip scars in male adult patients, follicular-unit hair transplantation done with a new atraumatic technique for hair implantation Male patients with cleft lip should be offered complete reconstruction of facial aesthetics at the appropriate age, to enjoy good social life [1, 2]. The scar of a unilateral cleft lip repair is a hair less, and the prolabium of a bilateral cleft lip repair is always devoid of hair follicles.[3] Moustache restoration is the last procedure offered, after all the functional problems have been corrected and the patient has reached adulthood. The current study reviews 2 male patients who had cleft lip repairs carried out in childhood and approached for moustache restoration in their adulthood. The shortest follow- upMaterials is 6 months and methodsand longest 2 years.

The number of grafts, the technique used, the number of sittings required and the method of implantation in unilateral and bilateral cleft lip cases are listed in [Table 1].

FUT: Follicular Unit Transplant, FUE: Follicular Unit Extraction Strip and follicular unit extraction are two popular techniques of hair transplantation. Follicular unit extraction is a hair transplant technique followed303 10th World Cleft Lip, Palate & Craniofacial Congress throughout the world to cover small bald regions of the scalp. Follicular unit extraction, popularly known as FUE, involves harvesting of live hair follicular unit grafts from the occipital region of the scalp and transplanting them into bald region. This technique involves quick recovery of the patient without the usage of sutures in the donor region compared to strip hair transplantation technique. Follicular units may contain from one to six hairs per unit. Preoperative laboratory studies to be performed include hb%, blood counts including bleeding and clotting time and blood chemistry profile including sugar, hbs ag screening and hiv 1 & 2 screening. restoration Preferred technique for hair restoration for post cleft lip moustache

Our method of choice is follicular unit extraction (FUE), in which selected single hair follicles containing only one robust hair are individually extracted with a 0.9 mm motorized punch and implanted as micro grafts[4]. The second method available is follicular unit transplant (FUT) [5]. FUT is carried out by excising a strip of the scalp 0.5-0.6 mm wide × 5-6 long, from the level of the occipital protuberance. The depth of the strip is restricted to the sub-dermal level. Scalp defect is closed in two layers. The strip is then slivered along the width, to create single rows of hair containing 4-5 follicles each. Each of otherthese methods slivers is of dissected hair implantation. to yield single hair follicular units. Method of hair implantation used in our cases is new, innovative and atraumatic compared to Observation and results

In our review of 2 cases of post cleft lip repair moustache restoration [table-1], we had 1 case of unilateral and 1 case of bilateral cleft lip. For two cases, moustache restorations were done with FUE technique in single sitting. Graft Implantation by stick and place was used in both patients (100%). In unilateral cleft lip patient we transplanted 100 grafts in single sitting, whereas in bilateral cleft lip patient we transplant 120 grafts in single sitting.

The result, 6 months after moustache restoration shown in fig 1 and 2 Fig1: post unilateral cleft lip repair moustache restoration after 6 months Fig2: Post Bilateral cleft lip repair moustache 304 restoration after 6 months 10th World Cleft Lip, Palate & Craniofacial Congress Discussion

The aim is to minimize the patient recovery time and conceal the scar as best as possible. Follicular Unit Extraction (FUE) involves quicker recovery time. Moustache reconstruction by hair transplantation is especially useful in patients who have had a cleft lip or a scar following trauma. 2% lignocaine with adrenaline is generally used for anesthesia; tumescent technique is preferred. After trimming the hair in the back of the scalp harvesting of the grafts is initiated. Approximately, 100 to 120 grafts are harvested and transplanted from the back of the scalp to the cleft region. The patient is discharged within few hours after careful bandaging. Patient is prescribed antibiotics, oral steroid to minimize any oedema and pain relievers. the shedding phase. After 3 weeks of the procedure the grafts show slight growth and enter

The patient is satisfied with the result and quick recovery associated with the procedure. The FUE individual extraction technique avoids linear scar and suturing over the donor area [fig 3]. FUE does not require a team of assistants to sliver and dissect the grafts. With FUE, you can select single robust hair units for mustache restoration fig 4[4]. However, FUE requires training and development of skills.

of single hair grafts Figure 3: Follicular unit extraction donor area after selective harvesting 305 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 4: different types of hair grafts.

We will select robust single hair grafts only for moustache restoration. Robust single hair graft is present starting in these grafts. For an occasional requirement, the hair follicles can be best harvested as a strip from the scalp. Restricting the depth of strip harvesting to sub-dermal level preserves lymphatics, veins and nerve endings avoiding neuromas and postoperative numbness and ensure faster healing. Harvesting from the level of occipital protuberance offers long lasting permanent hair follicles that, in later life, are not affected by the process of hair miniaturization like the rest of the scalp The method we prefer for implanting the grafts is "stick and place."[6]. In this method a 20G or 19G hypodermic needle is used for hair implantation [fig 5and fig 6]. Simultaneously a graft is slide along the slope of the bevel of the needle with a forceps in the right hand [fig5], and the needle withdrawn while holding the graft in, this is called "stick and place." Sliding the graft along the bevel facilitates placement. The epidermis of the graft must be kept 0.5-1 mm above the skin. Avoid placing deep for the possibility of epidermal cysts. Grafts are naturally pulled into the skin during healing as the tissue swelling subsides. Acquiring skills for implantation can today be replaced with the simpler use of implanters [7]. Single hair grafts can be loaded into the needle tip of an implanter device and pushed in place with a built-in sliding plunger. Potential Hair regenerating stem cells are present in Sebaceous glands and dermal papilla of hair graft. Our innovative method of Hair Implantation is completely atraumatic because we hold the epidermis and hair of graft with forceps [fig5] which prevents trauma to sebaceous glands and dermal papilla. Our technique enhances the survival rate of these stem cells. 306 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 5

Figure 6 Care of the grafts after implantation

Patients are instructed to avoid very expressive talking, vigorous shampoolaughing andwithout take rubbing food in smallon the bites. grafts Do and not dry touch it by or pressing rub the with upper a folded lip for 2 days. After two days they can splash water and clean the area lightly with 307 10th World Cleft Lip, Palate & Craniofacial Congress towel. The dry scabs which are holding the grafts in place loosen up around 8-10 days and will fall off during the cleaning by 8-10th postoperative day. After 10th postoperative day, patients begin application of 5 drops of minoxidil 2% lotion and use antioxidants, iron, calcium, amino acids, biotin supplements in a planned program to improve regrowth of the grafted hair roots [8, 9]. The hair growth from grafts implanted after cleft lip repair is delayed. All the hair grafts do not commence growth together, 30-40% grafts grow at 4-5 months, but all the grafts start growth by 6 months. Failure in 7-10% growth of grafts can be attributed to poor technique, scarring, and excessive handling of the graft. This can happen during graft dissection, repetitive attempts at graft insertion during implantation, which squeeze the follicle making it nonviable. The loss of grafts and failure to grow improvesConclusion with experience.

Follicular unit micro grafting with a single hair, imparts natural appearance, layout and replacement for the restoration of the moustache in unilateral and bilateral cleft lip male patients in their adulthood. Implanting with hypodermic needles ensures minimal injury with faster healing. The technique involves careful planning and acquiring surgical skills. The rewards for the labor are encouraging. Potential Hair regenerating stem cells are present in Sebaceous glands and dermal papilla of hair graft. Our innovative method of Hair Implantation is completely atraumatic because we hold the epidermis and hair of graft with forceps which prevents trauma to sebaceous glands and dermal papilla. Our techniqueReferences enhances the survival rate of these stem cells. 01. Marcusson A, Paulin G, Ostrup L. Facial appearance in adults who had cleft lip and palate treated in childhood. Scand J Plast Reconstr Surg Hand Surg 2002;36:16-23. 02. Millar K, Bell A, Bowman A, Brown D, Lo TW, Siebert P, et al. Psychological status as a function of residual scarring and facial asymmetry after surgical repair of cleft lip and palate. Cleft Palate Craniofac J 2013;50:150-7. [pubmed] 03. Duskova M, Sosna B, Sukop A. Moustache reconstruction in patients with cleft lip: (final aesthetic touches in clefts-part ii). J Craniofac Surg 2006;17:833-6. 04. Rassman WR, Bernstein RM, McClellan R, Jones R, Worton E, Uyttendaele H. Follicular unit extraction: Minimally invasive surgery for hair transplantation. Dermatol Surg 2002;28:720-8. 308 10th World Cleft Lip, Palate & Craniofacial Congress 05. Bernstein RM, Rassman WR. Follicular transplantation. Patient evaluation and surgical planning. Dermatol Surg 1997;23:771-84. 06. Ubel C. The punctiform technique in hair transplantation. Hair restoration: State of the art. Semin Plast Surg 2005;19:109-27. 07. Gandelman M. Technique for reconstruction of eyebrows and eyelashes. Semin Plast Surg 2005;19:153-8. 08. Rajput R. Cyclical medicine for hair loss management and improved results in hair transplantation. Hair Transplant Forum Int 2008;18:208-10. 09. Rajput R. Controversy: Is there a role for adjuvants in the management of male pattern hair loss? J Cutan Aesthet Surg 2010;3:82-6.

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Colombian Air Force Cleft Lip And Palate (Happy Faces) Program. 10 Years Changinglifes From The Colombian Skies

LT. PILAR MARTINEZB.N M.N. , Colombian Air Force Reserve Cleft Lip And Palate Program, Meissen Hospital E.S.E, Colombia

Professionals of reserve of the Colombian Air Force Reserve, are a group of volunteer Officers who work not profitwith the Civilian populationat faraway places in Colombiancountryside by different social programs. One of this efforts is Happy Faces who treat cleft lip and palate children on a basisof interdisciplinaryteam, high standards and no surgical safaris. Every month this group of Military and civilianvolunteers,travel across de Country to follow upmore than 300 patients and perform all the spectrum of treatment and also create air bridge to bring difficult cases to Bogota D.C. for highcomplexity surgery from primaryrepair, distraction osteogenesis and orthognathicsurgery. These proceduresare performing at Roosevelt Children Orthopedic Institute and Meissen Hospital. Accommodation, feeding, transport, and post operatory recovery are also supply by them. We present our experience after 7 years of labor methods and statistics.

310 10th World Cleft Lip, Palate & Craniofacial Congress

USM-Indonesia Cleft Charity Project

Roselinda Abdul Rahman Oral & Maxillofacial Surgeon, Universiti Sains Malaysia, Malaysia

The USM-Indonesia cleft lip and palate charity project was initiated by Professor Abdul Rani Samsudin, the founder Dean of School of Dental Sciences, Universiti Sains Malaysia (USM) with Universitas Gajah Mada in 1996 in Bali, Indonesia. This marked the first international USM collaboration on cleft lip and palate operation for the bottom billions in Indonesia. This annual charity project continues on till today at various communities in Indonesia. In 2004, MoA between USM and DMDI-MGM(NGO) was signed to further facilitate the charity project through the NGO platform. The main objective of this project is to provide free surgical treatment for the low economic status communities and transfer of knowledge to the local surgeons. A total of 13 communities were visited. The treatment ranged from lip repair to palatoplasty. In conclusion, herethrough to stay. this charity projects, beside having international linkages, the spirit of volunteerism especially in helping the bottom billions is strengthened and is

311 10th World Cleft Lip, Palate & Craniofacial Congress

International cooperation for improving in- country mission for children with cleft lip & palate in Uzbekistan

Murod M. Jafarov Republican Center of Pediatric Plastic Surgery, Tashkent pediatric medical institute, Tashkent, Uzbekistan

Introduction:

Since 1999, we started to cooperate with the Korean plastic surgeons in organizing a humanitarian mission in the region. Since 2010 this activity became wider and it was attended by many international organizations, which had a great influence on the development of pediatric plastic surgery in the region,Objective: in particular for the treatment of cleft lip and palate.

We want to show the role of cooperation with foreign medical organizations in the development of surgical rehabilitation of children with cleftMaterial lip and and palate. research methods:

During the period from 2010 to 2015 have been organized in 14 foreign and 8 local humanitarian actions in different regions of the country. Foreign partners were surgeons from Korea (Yonsei University), the international organization «Operation Smile - Singapore», «Smile Asia", «TurkPlast» and «SmileTrain-India». During this period, were examined more than 3,000 children and operated from them more than 900. Beginning in 2012, the staff of our center began to organize local events, in-country mission using the experience of working with foreign partners. And in this we are very good at helpingResults: the organization «Smile Train».

Over the past 6 years, our staff has been inspected more than 1,000 children312 and about 100 of them were operated. Our actions have been organized 10th World Cleft Lip, Palate & Craniofacial Congress

in conjunction with other specialists such as ENT, ophthalmology, orthopedics and cardiologist. It was widely used grant «Smile Train» organization in the procurement of supplies. Operations are organized in local clinics, and often there is a lack of special surgical instruments and equipment for plastic surgery. To address these shortcomings by using the Ministry of Health and the German fund in 2016-2017, the planned purchase of a large number of surgical tools and equipment for plastic surgery in the regions. For more efficient operation, we are planning to organize a mobile outreach teams for surgery cleft lip and palate in long-distance areas of Uzbekistan. For this grant will be used «SmileTrain» organization in the procurement of mobile anesthetic machine, minivan and consumables.Conclusion:

With the support of international organizations such as «SmileTrain» can organize more in-country mission with their own resources.

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3D computer-assisted elastic models for cleft lip repair

Koichi Ueda, Yuka Shigemura, Yuki Otsuki, Misato Katayama Department of Plastic and Reconstructive Surgery, Osaka Medical College, Japan

Introduction

Usefulness of computer-assisted three-dimensional (3D) models is frequently reported in cranio-maxillo-facial operations. These models are used for simulation surgery, planning operations, and explaining the operative methods and are covered by insurance in Japan. They are solid models made from the CT data of patients’ facial bones. However, we recognized the necessity of elastic models as targets for flaps or soft tissue in plastic surgery or microsurgery. As well, soft and elastic models like skin or soft tissue are necessary for simulation surgery or the training of cleft lip repairs. However, it is difficult to make them by conventional methods. In this project, we tried to makeMethods 3D computer-assisted elastic models for cleft lip repair.

We sought many elastic materials which have texture like skin or soft tissue and made models by pouring several types of solvents into the outer molds calculated from CT or MRI data of cleft lip patients. The goals of the model are to be able to write incision lines on it by pen, to cut the model by surgical knife,Results to suture it, and to keep the stiches in place for a long time. obtained. Three-dimensional elastic models which reached these goals could be Therefore, we were able to shape ideal models made of polyurethane and silicone(Fig.1).

314 10th World Cleft Lip, Palate & Craniofacial Congress Discussion

It was found that the models must have two different layers. We must continue to improve the elastic models further.

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Zygomatic Hypoplasia in Cleft Lip and Palate Patients– A Pilot study

S.Rengasamy Iniyan , V.B.Krishnakumar Raja, Sneha Pendem. SRM Dental college,Ramapuram, Chennai, India

Abstract Aim and palate. To study the incidence of zygomatic hypoplasia in patients with cleft lip Subjects and Methods

15 adult patients with in an age group of 18-25 years who reported to us for the correction of cleft nasal deformity were included in the study. 4 patients were cases of bilateral cleft lip and palate, one patient had isolated cleft palate and the rest of the 10 patients had unilateral cleft lip and palate. All the patients washad undergoneperformed repairto assess of cleft the lip, position palate ofand the alveolus zygoma (Cleft on frontal,palate repair lateral alone and in case of isolated cleft palate) in their childhood. Photogrammetric evaluation submentoResults vertex views.

3 out of 4 patients with bilateral CLAP presented with unilateral hypoplasia of the zygoma while 8 out of 10 patients with unilateral cleft lip presented with zygomatic hypoplasia of the affected side. While patients with isolatedConclusion: cleft palate presented with hypoplasia of the right side

Zygomatic hypoplasia is one of the additional features that is seen in patients operated for cleft lip and palate that may need to be addressed to enhance facial esthetics.

316 10th World Cleft Lip, Palate & Craniofacial Congress

Unilateral Cleft Lip correction - Millard Vs. Delaire

E. NIRMAL TONY Post Graduate, OMFS, Ragas Dental College and Hospital, M. Veerabahu, J. A. Nathan

Abstract

Unilateral cleft lip repair, over the last century has seen both revolutionary changes and subtle technical refinements. Many techniques have evolved through these years. Delaire’s and Millard’s rotational flap were the most commonly used technique for repair of unilateral cleft lip. This paper discusses the advantages and disadvantages of both Millard and Delaire’s technique for unilateral cleft repair.

317 10th World Cleft Lip, Palate & Craniofacial Congress

Unoperated Clefts Teach Us A Lesson A Cephalometric Study

Abdul Latif1, A.M. Kuijpers-Jagtman2, C.Lekkas3, B.S. Latief4 1,4Department of Oral and Maxillofacial Surgery, Faculty Dentistry, Universitas Indonesia, Jakarta, 2Radboud University Medical Centre, Nijmegen, The Netherlands, 3Leiden University Medical Centre, Leiden,The Netherlands

Objective

The purpose of this study was to compare cephalometric facial morphology among adult, unoperated cleft groups: UCLA, UCLP. BCLA and BCLPMethods with a control (non-cleft) group of the same ethnic background.

In this retrospective observational study we analyzed the cephalograms of 160 subjects with UCLA, 59 subjects with UCLP, 21 subjects with BCLA, 13 subjects with BCLP and we compared them with 52 non-cleft controls. The cephalograms were digitized by one observer with commercially available software (Viewbox 3 Dhal Software, Kiffisia, Greece). The lateral cephalograms wereResults analyzed according to a modified Eurocleft protocol.

All cleft groups had more maxillary (SNA) and mandibular prominence (SNPg) than the control group. Sagittal jaw relationship (ANB) differed significantly between the clefts and the control group, being larger in the cleft groups. Upper incisor inclination and the interincisal angle did not differ significantlyConclusions between the controls and all cleft groups.

Facial morphology in adult, unoperated cleft groups showed potentially normal development in anteroposterior direction. Each type of cleft has its own characteristic facial morphology.

318 10th World Cleft Lip, Palate & Craniofacial Congress

A Multidisciplinary Approach in the Management of Patients with Cleft Lip and/ or Palate

Anand Sekhar, B. H Sripathi Rao Yenepoya Dental College, Mangalore

The needs of patients with a cleft lip and/or palate (CL/P) extend beyond surgical repair. A multidisciplinary approach to the care of patients with CL/P is the widely accepted standard. Treatment of the patient with cleft lip and palate represents a real problem from both functional and esthetic points of view. In the present era a functional result is not enough. An esthetic result is necessary to improve the quality of life. Affected patients have a number of medical issues and potential complications, and therefore require a wide variety of healthcare specialists beyond plastic surgeons and dental specialists. For this reason, the best environment in which to deliver this care is a multidisciplinary approach that features a team of healthcare providers, including audiology, paediatric otolaryngology, speech pathology, occupational/feeding therapy, and genetics. In the new-born period, making sure the infant can feed adequately is the primary concern but later, orthodontic and cosmetic issues become more prominent. Other potential complications include hearing loss, speech, dental, and orthodontic complications. These require consistent monitoring through adolescence and into adulthood. To address the variety of primary and secondary problems associated with cleft lip and/or palate, these individuals will see many ordifferent palate. specialists. In this paper we will provide such an overview, discussing a multidisciplinary approach in the management for patients with cleft lip and/

319 10th World Cleft Lip, Palate & Craniofacial Congress

The tongue flap for closure of oronasal fistulas

Karavanov D, Korotchenko G Dept. of Reconstructive Surgery Oleksandrovsky Hospital, Kiev, Ukraine

BACKGROUND

An oronasal fistulas (ONF) of hard palate in cleft patients is still not an uncommon situation. They can be present as a residual state after an incomplete primary cleft palate repair or be a result of previous unsuccessful closure attempts. Small, single fistulas usually are almost asymptomatic. On the other hand, single large or even multiple small fistulas can seriously affect quality of life due feeding difficulties, speech and other problems. Therefore all fistulas, that lead to functional impairments, need to be closed. Various techniques of ONF closing were described. One of them is using dorsal tongue flap.

METHODS

During 2008-2016 dorsal tongue flap was applied to 18 our patients (6 males and 12 females) who had ONF. Age of the patients ranged from 14 to 26 years. 13 patients had already had previous unsuccessful attempt of grafting.fistula repair. 5 patients have unilateral cleft and 13 – bilateral. In 11 cases we performed tongue flap procedure with simultaneous alveolar cleft bone

In all of our cases we used anterior based dorsal tongue flap with two- layer closure. The surgical treatment was done in two stages. We performed fistula closure with tongue flap transfer under general anesthesia. Then, after 12-14 days the pedicle of the flap was removed under local anesthesia.

320 10th World Cleft Lip, Palate & Craniofacial Congress RESULTS

All flaps healed well and no cases of flap necrosis were noted. In 15 cases abnormal communication between oral and nasal cavities was successfully eliminated. In 3 bilateral cases, where we couldn’t achieve complete integrity of nasal floor due to severe tissue scarring or it’s deficiency, small secondary fistulas occurred. In 1 cases fistula was closed within 1 year, and in another 2 cases patients were fully satisfied with obtained results and didn’t require any additional revisions. In all of the cases good functional improvement was achieved. Despite the fact that between first and second stage of procedure patients had restrictions in feeding and inability to speak they noted postoperative period as relatively easy for them. At follow up the shape and function of the donor tongue wasn’t affected inCONCLUSION all of our cases.

Tongue flap is a safe and effective method of ONF treatment. The main advantage of the tongue flap is possibilities to obtain a highly vascularized tissue for repair of even very big palate defects without any functional impairment of the donor site. The prerequisite to achieve good result is two-layer closure technique of the defects with reliable nasal floor reconstructing.

321 10th World Cleft Lip, Palate & Craniofacial Congress

ALVEOLAR BONE GRAFTING IN UNILATERAL AND BILATERAL CLEFT- HOW WE DO IT

YASMIN MARY JOSE IIIRD YEAR PG RAGAS DENTAL COLLEGE

ABSTRACT

Management of patients with cleft has developed significantly over the recent years. One of the major advances has been the development of a successful method to reconstruct the cleft alveolar (bony) defect with bone (secondary bone grafting) with few complications and no restrictions in facial growth. This paper discusses alveolar bone grafting procedure in unilateral and bilateral cleft patients with case reports.

322 10th World Cleft Lip, Palate & Craniofacial Congress

Controversies In Alveolar Cleft Reconstruction In Cleft lip and Palate Management

1D.KAVITHA, 2RAJASIGAMANI 12nd yr.Orthodontic PG, 2Head Of The Department Of Orthodontics, Raja Muthiah Dental College And Hospital, Annamalai University.

ABSTRACT

Cleft lip and cleft palate are birth defects that occur when a baby’s lip or mouth do not form properly during pregnancy. They are also known as “orofacial clefts”. They have various combination of clinical manifestations. It is essential to close the cleft site in order to maintain the arch integrity ,to guide the erupting permanent teeth,to support the alar base.The alveolar bone graft plays a vital role in the cleft management. However, controversies exist in the context of timing of graft placement , type of graft ,whether maxillary expansion indicated before or after surgery, eruption of teeth through the graft,methods of evaluating the reconstructed graft. On review of literatures, studies indicate that the prognosis for canine grafteruption material through surpass the graft the siteallogenous is more graftfavourable material ,if the in managingcleft reconstruction the osseous is carried out when ¼ to ½ of canine root completion has occurred. Autogenous bony defect. Expansion of the maxillary arch should be carried out before placing the graft ,as there should be enough space to receive the graft material. This prevent the early fusion of thesuturalbone.early surgical and orthodontic boneassistance graft. is more appropriate rather to wait for delayed eruption.CBCT appears to more efficient in evaluating volumetric measurement of the reconstructed

To achieve an optimal result, a complete co-operation between the orthodontist and surgeons is mandatory. The road doesn’t end here. Many research studies is needed in the progress of cleft management. This review paper highlights the controversies pertaining to the alveolar cleft reconstruction. 323 10th World Cleft Lip, Palate & Craniofacial Congress

INTRODUCTION

Alveolus Means Trough (Latin Word); Trough Containing Tooth Buds. Alveolarcleftisacommon developmental anomaly which affects approximately 75% of cleft lip or cleft lip and palate patients. The aetiology of this cleft is still poorly understood, but it is most likely considered to be multifactorial involvin ggeneticandenvironmental factors.Failure of fusion of Median Nasal Process& maxillaryprocess, when the ossification centres in the premaxilla& maxilla cannot migrate & fuse ,lead toclefting alveolus.The alveolar cleft may affect the developing dentition and contribute to the collapse of the alveolar segments. Failure to reconstruct the alveolar cleft may give rise to problems including oronasal fistula, fluid reflux, speech pathology, anteroposterior and transverse deficiency of the maxilla, lack of bone support for the anterior teeth, dental crowding, and facial asymmetry.Patients with a bony defect in the alveolar process with a symptomatic oronasal fistula and/or a lack of bone impairing tooth eruption ororthodontic treatment or prosthodontic rehabilitation in this area, RATIONALE should be considered for alveolar bone grafting.

Provide bone for the eruption and/or orthodontic repositioning of teeth. Closure of oro-nasal fistulas.Support and elevation of the alar base.Stabilization of the pre-maxilla in bilateral case.Provide continuity of the alveolar ridge.The restoration of jaw function and morphology in cleft lip and palate patients is critically important and requires reconstructioin of the maxillary alveolar clefts dependTIMING on OF osteogenic BONE GRAFT potential and bone regeneration in osseous bone defect.

Bone grafting of the alveolus is now generally acknowledged to be as integral to the management of the cleft patient as that of the primary lip or palate repair . Since the report describing secondary alveolar bone grafting by Boyne and Sands , this procedure has become the common method of choice. Th eoptimaltimingofbonegraftplacementremainscontroversial. 1) primary bone grafting: when bone grafting is performed in children younger than 2 years of age; 2) early secondary bone grafting: to be applied in patients between 2 and 5 years old; and 3) secondary bone grafting: when procedures are undertaken in patientsPRIMARY greater BONE than GRAFT 5 years of age.

It waspopular in the 1950’s -60’s.Usually done in conjunction with maxillary324 orthopedics.This concept gained popularity as therib grafts was placed 10th World Cleft Lip, Palate & Craniofacial Congress

either simultaneously with lip repair or shortly afterand created possibility for harmonious facial growth and development.Before the 1970s, primary alveolar bone grafting was commonly carried out until its adverse developmental effects on maxillary growth, severe crossbite, and poor alveolar morphology .After this it was abandoned.Earlysecondary bone grafting resulted in improved orofacial development SECONDARY but BONE this GRAFT was not always ideal .

Bone grafting is called as secondary when performed later at the end of the mixed dentition. It is the most accepted procedure and is performed preferably before eruption of the permanent canine in order to provide adequate periodontal support for eruption and preservation of the teeth adjacent to the cleft.Secondary alveolar bone grafting may bring cleft patients several benefits. 1. It gives bony support to the teeth proximal to the cleft and greatly enhances the follow-up orthodontic treatment . 2. It gives a bony base for eruption of teeth in the line of the cleft and this prevents drifting of proximal teeth into the cleft and their premature loss. 3. It provides union to the maxillary arch and re-establishes alveolar bone contour . 4. It provides support to arch width and minimizes collapse of the maxillary arc. 5. It stabilizes the maxillary segments for the maintenance of the dentition and mastication. 6. It reduces notching of the alveolar ridge . 7. It eliminates oronasal fistulae to improve the oral hygiene . 8. It may improve facial appearance through improving facial symmetry, providing alar base support, and improving nasolabial contour. Conversely, secondary alveolar bone grafting may cause: 1. increased incidence of canine impaction; 2. donor site morbidity; 3. reduced anterior-posterior and/or vertical maxillary growth The general steps for secondary alveolar bone grafting are as follows. graftFirstly, materials mucoperiosteal eitherautogenous flaps onthepalate bone andvestibularor bone substitute surfaces are of theprepared maxillary or segments to widely expose the alveolar bony defect are made. Meanwhile, the harvested. The nasal mucosa is firstly closed to repair the nasal floor. It is then pushed upwards and the bony wall is exposed. Bone or bone substitute is then packed into the defect, filling the alveolar defect completely. The palatal flaps are then released for primary closure of the oral layer. Often a finger flap is utilized from the vestibule and rotated over the alveolus. Lastly, all the flaps are sutured together to close the oral layer over the graft in the cleft site .

325 10th World Cleft Lip, Palate & Craniofacial Congress TYPE OF GRAFT MATERIALS

Autogenous –Cancellous- iliac crest Block Particulate – Cortical- calvarium, mandible Bone dust Blocks – Cortico-cancellous- iliac, rib, tibia, mandible (tibia and mandible only in late secondary grafting) An autogenous graft from the anterior or the posterior iliac crest is considered the optimum source of autogenous bone for alveolar cleft reconstruction and hence it is termed the “gold standard bone graft”.It provides a large quantity of cancellous bone and easy surgical access.the important disadvantages of autogenous graft are donor site morbidity,bleeding ,hematom Allogeneic(allograft)a,pain,discomfort,prolonged hospitalization ,etc. graft. -Bone Morphogenic Protein (BMPs) provide osteoinduction.theyare released in response to osteoclastic activity to stimulate bioincorporation of

-calcified collagen structure presents oseo-conductive properties forming a scaffold that facilitates bone deposition. Graft resorbs, remodels, may contribute to osteoinduction and osteoconduction. It has been suggested for alveolar cleft reconstruction because itAlloplast:- overcomes the disadvantage of autogenous graft.

-hydroxylapatite adult patientsBone grows who into do not and need around any alloplast.No future insertion active of osteoinduction endosteal implants. but some osteoconduction.Teeth do not erupt through alloplast.it is recommended in TIMING OF GRAFT PLACEMENT

326 The ideal time for performing alveolar bone graft to patients with cleft 10th World Cleft Lip, Palate & Craniofacial Congress

alveolus varies from patients to patients. Grafting is highly successful in the age group from 9 to 12 years when the canine root is ¼ to ½ formed .Canine teeth can migrate and erupt spontaneously through autogenous iliac crest bone graftandERUPTION the OF canine TEETH will THROUGH have a normal THE GRAFT root development.

Eruption of teeth through the graft has been a controversial. Most authors discuss eruption without details. Some report spontaneous eruption in 95% of patientswhereas others indicate a remarkably low rate of eruption and emphasize the need for surgical uncovering. Deeb et al reported 27% spontaneous eruption. They admit, however, that “this low rate may be an under estimate of unassisted eruption gives early surgical and orthodontic assistance was readily available to accomplish tooth position when it appeared appropriate to do so rather to wait for delayed eruption.”The bone graft rapidly transformed into functional alveolar bone which responded to the eruption and produced bony volume sufficient for the vertical height.Hence surgical and orthodonticPRE-VERSUS intervention POST-SURGICAL is often EXPANSION required for complete eruption.

Most authors prefer presurgical expansion .This is because there is less resistance ,improved access the cleft for closing the nasal floor ,better postoperative hygiene and less chance of reopening of the oronasal fistula. Orthodontic treatment should be started atleast 6 months before bone grafting. expansion.Development of favourable arch form is the ideal requisite. Correction of malalignment and crossbite are the corner stone of orthodontic treatment by EVALUATION OF ALVEOLAR CLEFT BONE GRAFTING

For many years ,bone graft success and measurement relied on the Bergland grading system.It was considered as the golden standard method.The major drawback of this system is that it compared only at the interdental level using the normal bone level as the reference.To overcome this Chelsea scale was developed. Conventional periapical ,occlusal and OPG radiographs are not reliable due to distortion of the images in the cleft area.studies show that CT scans can evaluate the amount of newly formed bone in the defect and provide adequate results. However the cone –beam system offers several advantage over CT scanners including a limited radiation,minimal scanning time ,high quality ,high resolution diagnostic imaging.

327 10th World Cleft Lip, Palate & Craniofacial Congress SUMMARY

Reviewing the literatures, it is found that the secondary bone grafts of the alveolar cleft are most widely accepted timing for surgical reconstruction. Autogenous bone graft are still considered the gold standard for alveolar cleft reconstruction with most reliable and best outcome despite the emergence of composite grafting materials.prognosis for canine eruption through the graft site is more favourable ,if the cleft reconstruction is carried out when ¼ to ½ of canine root completion has occurred.It is better to do surgical and orthodontic assistance to accomplish tooth position rather than delayed spontaneous eruption through the graft. Many authors prefer pre -surgical expansion and ConeREFERENCE Beam system is more advantageous in evaluating the bone graft. 01. Is alveolar cleft reconstruction still controversial? (review of literature) Sameh A. Seifeldin. The Saudi Journal for Dental Research (2016) 7,57-63. 02. Canine eruption into grafted bone in maxillary alveolar cleft defects.Mohamed El Deeb,LouiseB.Messer,MichaelW.lehnert,ThomasW.Hebda,DanielE.Waite.presented at the 37th Annual Meeeting of the American Cleft palate Association. 03. Secondary alveolar bone grafting in cleft of the lip and palate patients.AbhilashaaWalia. Contemporary Clinical Dent .2011 Jul-sep;2(3):146-154. 04. Secondary bone grafting for alveolar cleft in children with cleft lip or cleft lip andpalate. GangWu,Scott A Deacon. Research Gate. 05. Nique T, Fonseca RJ, et al: Particulate allogeneic bone go maxillary alveolar clefts in humans- A preliminary report. J Oral MaxillofacSurg 45: 386-392, 1987 06. Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate model. J Oral MaxiilofacSurg 47:946-952, 1989 07. Friede H, Johanson B: Adolescent facial morphology of early bone grafted cleft lip and palate patients. Scand J PlastReconstrSurg 16:41-53, 1982 08. Trotman CA, etal: Comparison of facial form in primary alveolar bone-grafted and nongrafted unilateral cleft lip and palate patients. Cleft Palate Craniofac J 33:91, 1996 09. Dado DV. Early Primary Bone Grafting. In: Kernahan DA, Rosenstein SW, eds. Cleft Lip and Palate. ASystem of Management. Williams and Wilkins, Baltimore, 1990. pp 182-188. Nelson CL: Primary Alveolar Cleft Bone Grafting. Oral MaxillofacSurgClin NA 3:599, 1991.

328 10th World Cleft Lip, Palate & Craniofacial Congress 10. Veau V. Paris: Masson; 1931. Division Palatine. 11. Bjork A, Skiller V. Growth in width of the maxilla studied by the implant method.Scand J PlastReconstr Surg. 1974 [PubMed] 12. Waitede, Kersten R.B. Residual Alveolar & Palatal Clefts. In: Bell WH, Proffit WR, White RP, editors. Surgical Correction of dentofacial deformities. Philadelphia: WB saunders; 1980. pp. 1329–1367. 13. Boyne P.J., Sands N.R. Secondary Bone Grafting of Residual Alveolar and Palatal Clefts. J Oral Surg. 1972;30:87–92. [ 14. Abyholm FE, Bergland O, Semb G. Secondary bone grafting of alveolar clefts.A surgical/ orthodontic treatment enabling a non- prosthodontic rehabilitation in cleft lip and palate patients. Scand J PlastReconstr Surg. 1981;15:127–40. [PubMed] 15. Goudy S, Lott D, Burton R, Wheeler J, Canady J. Secondary alveolar bone grafting: outcomes, revisions, and new applications. Cleft Palate Craniofac J.2009;46:610–12. [PubMed] 16. Newlands LC. Secondary alveolar bone grafting in cleft of the lip and palate patients. Br J Oral Maxillofac Surg. 2000;38:488–91. [PubMed] 17. Ozawa T, Omura S, Fukuyama E, Matsui Y, Torikai K, Fujita K. Factors influencing secondary alveolar bone grafting in cleft lip and palate patients: prospective analysis using CT image analyzer. Cleft Palate Croniofac J. 2007;44:286–91. [PubMed] 18. McIntyre GT, Devlin MF. Secondary Alveolar Bone Grafting (CLEFTSiS) 2000-2004. Cleft Palate Craniofac J. 2010;47:66–72. [PubMed] 19. Bergland O, Semb G, Abyholm FE. Elimination of residual alveolar cleft by secondary bone grafting and subsequent orthodontic treatment. Cleft Palate J.1986;23:175–205. [PubMed] 20. Troxell JB, Fonseca RJ, Osbon DB. A retrospective study of alveolar cleft grafting. J Oral Maxillofac Surg. 1982;40:721–5. [PubMed] 21. El Deeb M, Messer LB, Lehnert MW, Hebda TW, Waite DE. Canine eruption into grafted bone in maxillary alveolar cleft defects. Cleft Palate J. 1982;19:9–16.[PubMed]

329 10th World Cleft Lip, Palate & Craniofacial Congress

Facial Reconstruction after Pre Maxillary Resection in a Bilateral Cleft Case Report-Lip and Palate Patient

E. Silberstein1, N. Givol2, E. Droma3 1Oral and Maxillofacial Surgery Unit and 2Cranio-Facial Surgery Unit, 3Plastic Surgery Department, Soroka University Medical Center, Beer Sheba, Israel.

Abstract

Loss of the premaxilla is a rare event while treating patients with bilateral tocleft treatment lip and palate. and manipulation Its resection isof no the longer premaxilla, an acceptable and not method due to of intentional treatment in modern practice. Nowadays, most cases of premaxillary loss are secondary resection. In the following case presentation we present the treatment of a seven years old boy, presented to our institution after being treated for bilateral cleft lip and palate by resection of the premaxilla. Through a series of operations including bone grafting, distraction of the maxilla, and cartilaginous graft we managed to reconstructIntroduction a proper facial habitus, a convex facial profile and nasal projection.

"There is no danger in cutting too much of that which serves no purpose" was the accepted treatment method when handling bilateral cleft lip and palate during the 16th century, as it was described by Pierre Franco1. Facing the consequences of discarding the pre-maxilla, including maxillary retrusion, it took more than 300 years until a technique was devised to handle the protruding pre-maxilla in the late 19th century by vomerine resection and premaxillary setback.2 Today most surgeons prefer to change the position of the pre-maxilla using facial orthopedics and orthodontic treatment2, 3- i.e.by non surgical means. The protruding maxilla among cleft patients is always challenging. The goal of treatment is to incorporate the pre-maxilla in a harmonious manner into the maxillary alveolar process thus reconstructing the horse-shoe shape of the patient's alveolar crest. When lost or sacrificed for the benefit of lip closure,330 the treatment becomes a tenuous and long journey to reconstruct the 10th World Cleft Lip, Palate & Craniofacial Congress normal habitus. lost anatomical structures and give the patient's face the proper projection and

The following case report describes our experience with reconstruction of the face of a young patient suffering from bilateral cleft lip and palate, presentingCase presentation after the loss of the pre-maxilla while treated somewhere else.

A seven years old boy born with bilateral cleft lip and palate was presented to our craniofacial surgery unit. Medical history revealed that he was born with bilateral cleft lip and palate. At the age of one year he underwent resection of the pre maxilla, and lip closure. On physical examination severe maxillary retrusion, with lack of nasal support and widening of the nasal alar base, was noted, together with a two centimeter wide oronasal fistula due to the absence of the primary palate. He was lacking the primary maxillary incisors bilaterally (figures 1,2 &3). Goals of therapy were aimed at closing the oronasal fistule, reconstructing of maxillary alveolar process continuity and horse shoe shape, attaining a proper support for the nasal alar base, reversal of the existing facial concavity and reconstruction of proper facial habitus, and reconstruction of the nasal projection. In the first stage he went through maxillary reconstruction using a bicrotical bone graft from the iliac crest. Soft tissue coverage was achieved by local mucosal flaps. This procedure created a proper division between the oral and nasal cavities. A full integration of the graft to the lateral segments of the maxilla and sealing of the oronasal fistule was noticed 11 months post operatively. In the second stage a le fort 1 osteotomy and advancement by Distraction Osteogenesis was conducted one and a half year later. Thus a Le Fort 1 Osteotomy was made via a vestibular approach, and a Rigid External Distractor (KLS martin Inc.) was applied. Distraction commenced three days post operatively at a rate of one millimeter a day for two weeks. Due to logistic difficulties which included a rural place of living of the patient with limited access to proper medical treatment in case of need, It was not safe enough to release him home while the distraction frame of a Rigid External Distractor is mounted on his head. On the other hand the patient could not stay the whole period of distraction and consolidation under supervision. Therefore, after four weeks the distraction gear was removed and the osteotomy sites were fixed rigidly by 1.5mm titanium plates (Walter Lorenz CMF) in the331 10th World Cleft Lip, Palate & Craniofacial Congress piriform aperture and 2.0 mm in the zigomatico-maxillary buttress on each side. The plates were removed under general anesthesia six months later. At the end of this phase of treatment, we received a proper support for the nasal alar base and the maxilla was advanced anteriorly enough to receive a proper anterior facial projection of the face (fig 4). One month later the nose was reconstructed using rib cartilage graft in the form of an "L" strut to support the dorsum and tip of the nose, and provide proper collumelar projection. The collumela was reconstructed by a transverse bipedicle flap raised from the skin along the alar bases (fig 5). After this final stage we have received a proper facial profile with adequate nasal projection (fig.Discussion 6).

Absence of premaxilla due to resection or necrosis is very rare though when present it leaves the patient with a significant deformity which its correction is a surgical challenge. Resection of the premaxilla to enable closure of bilateral cleft lip and palate was abandoned many years ago due to the unacceptable attempteddeformity it creates. However, rarely, one may encounter such a case, most often due to4,5 necrosis of the premaxilla when osteotomy and mobilization are . The literature regarding reconstructive options in such cases is scanty. We are presenting this case in order to share our experience and to encourage the discussion about the proper way of treatment in such cases.

The loss of6 the premaxilla entails several problems to be dealt with during ●reconstruction● : ●● oronasal fistule ●● maxillary restriction and severe concavity of the midfacial skeletal profile ●● lack of upper lip support. ●● lack of nasal support missing collumela Each problem should be addressed in the final reconstruction, taking into account the severity of the deformity, and the surrounding scarred tissue. The strategy chosen was a gradual and staged treatment, while each surgery is set to achieve a specific goal. By doing so we were able to reduce the risks of complication such as graft exposure, infection, and relapse. The initial bone graft from the iliac crest addressed the closure of a large oronasal332 fistule and creation of a continuous bony segment and obtaining the 10th World Cleft Lip, Palate & Craniofacial Congress

infrastructure for the coming surgeries. It was possible to achieve coverage of the graft by local flaps. If the defect had been wider, it would necessitate the mobilization of distant tissues such as a facial artery musculomucosal flap, submental flap or a free microvascular flap, but these are rare when considering that 7,8even an adults premaxillary segment does not reach the width of 5 centimeters. After bone grafting we addressed the maxillary restriction, the severe profile convexity, and lack of support to the upper lip. This convexity results from the absence of the growth centers of the premaxilla and vomer, the restriction created by the scar tissue at place, and the lacking AP dimensions of the bone graft. At this stage we planned for maxillary advancement. Considering the surrounding scarred tissue, the possible vascular compromise as well as dehiscence and bone graft resorption and possible relapse of the maxillary anterior movement, a distraction osteogenesis was preferred over single stage Le Fort 1 advancement. Because the patient could not stay under our supervision any longer and it was considered unsafe to release him with a metal distractor frame attached to his skull, since it will take him too long to get to a medical center in case of complications, we replaced the Distraction gear and put in titanium fixation plates for the remaining consolidation period. Thus, we combined the advantages of osteodistraction with those of traditional osteosynthesis. After maxillary advancement, and improvement of the facial profile, additional support to the alar base was achieved. The surgical reconstruction of nasal projection included a cartilaginous strut harvested from the ribs using an open rhinoplasty approach. This enabled a stable support for the nasal structure including reconstruction of nasal tip projection, and proper nasolabial angle. The most challenging aspect in this part of the reconstruction was obtainingcollumelar skin. Collumelar lengthening in cases of bilateral cleft lip is often performed using a fork flap, however with the lack of prolabium, cover and lining of the cartilage graft, was accomplished with a local bipedicle transverse skin graft (figure 5). A different option may have been a forehead flap to gain cover in case the skin defect was larger but would necessitate a secondConclusion stage for splitting the flap from its pedicle.

Loss of the premaxilla is a rare event while treating cleft lip and palate patients. Its resection is not an acceptable method of treatment in modern practice. When encountered it obligates a meticulous reconstruction of all 333 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 1- Demonstrating maxillary restriction and severe concavity of profile along with lack of support to the upper lip and lack of support to the alar base, dorsum and tip of nose.

Figure 2- demonstrating the oronasal Figure 3- A lateral cephalomaetric X fistule and lack of continuity of the ray demonstrating the Class III like alveolar process. intermaxillary relationship after bone grafting.

Figure 4- after advancement using an Figure 5- Demonstrating the External Distractor Device proper support technique for nasal reconstruction of the alar base, and a proper facial profile using costochondral cartilage and a are gained. bipedicled skin flap for coverage.

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Figure 6- the final result some 2 months after last operation with an adequate facial profile and nasal projection facial features affected by the premaxillary loss. In this case we presented our experience and considerations for such a facial reconstruction, while contemplatingReferences other treatment options.

01. Santoni-Rugiu P, Sykes PJ, A History of Plastic Surgery, 1st edition, Berlin Heidelberg, Springer, 2007, pp 222-223. 02. S. Bhattacharya, V. Khanna, R. Kohli, Cleft lip: The historical perspective, Indian J Plast Surg. 2009 Oct; 42(Suppl): S4–S8. 03. Bittermann GK, De Ruiter AP, Janssen NG, Bittermann AJ, Van der Molen AM, Van Es RJ, Rosenberg AJ, Koole R, Management of the premaxilla in the treatment of bilateral cleft of lip and palate: what can the literature tell us?, Clin Oral Investig., cited 2015 Dec 3, available on link.springer.com. 04. Carlini JL, Biron C, Gomes KU, Da Silva RM, Surgical repositioning of the premaxilla with bone graft in 50 bilateral cleft lip and palate patients. J Oral Maxillofac Surg. 2009 Apr;67(4):760-6 05. Heidbuchel KL, Kuijpers-Jagtman AM, Freihofer HP. An orthodontic and cephalometric study on the results of the combined surgical-orthodontic approach of the protruded premaxilla in bilateral clefts. J Craniomaxillofac Surg. 1993 Mar;21(2):60-6. 06. Motohashi N, Pruzansky S., Long-term effects of premaxillary excision in patients with complete bilateral cleft lips and palates. Cleft Palate J. 1981 Jul;18(3):177-87 07. Kazanijan V.,Bone transplantation to the mandible. Am. J. Surg. 1952;83:633. 8. Kovach SJ, Levin LS, Skeletal Reconstruction In: Neligan PC, Plastic Surgery ,3rd edition, London, Saunders, 2013, Volume 4, pp 174-188. 335 10th World Cleft Lip, Palate & Craniofacial Congress

Surgical and Orthodontic correction of a case of unilateral cleft lip and palate including alveolus

Upasak Mukherjee1, US Krishna Nayak2 1Dept. of Orthodontics and dentofacial Orthopaedics, 2Principal & Dean, AB Shetty Memorial Institute of Dental Sciences, Mangalore, Karnataka

A 19 year old girl with previously operated cleft lip and palate reported with the chief complaint of irregularly placed upper and lower front teeth. There were no associated syndromes. On EXTRAORAL examination, the patient’s cephalic index was dolicocephalic and leptoprosopic facial type.She had convex facial profile. Both maxilla and mandible were retrusive in relation to the cranium. She had posterior divergence of the face,steep mandibular plane angle and increased lower facial height.She had incompetent lips with previous history of operated cleft lip and palate including alveolus at one and half years of age.There was 5mm of incisor display at rest with a hypertonic lower lip.She had recessive chin and a shallow mentolabial sulcus.

Fig 1.1 showing frontal view Fig 1.2 showing lateral view On INTRAORAL examination, the maxillary arch was V shaped with severe crowding in the labial segment;22 is labially placed ,23 is palatally placed which is in line with the scar of the previously repaired cleft.The mandibular arch was U shaped with severe crowding in the lower anterior segment,along with336 missing 36 and a mild mesial inclination of 37.Upper midline is shifted to 10th World Cleft Lip, Palate & Craniofacial Congress

the left side with an anterior open bite of 2mm.She had Angle’s Class ІІ molar relationRadiographic on right Examination side and Class І on the left side.

Pretreatment Orthopantomograph(OPG) and Lateral Cephalogram were

made.Various cephalometric analysis were done including. Steiner’s,Down’s,Tw eed’s,McNamara’s and others. . The patient had Class II skeletal base(ANB =3)with both retrusive maxilla andDiagnosis mandible with vertical growth pattern(FMA=48). the upperNon andgrowing lower female anterior patient teeth withalong a withClass anterior II skeletal open base,vertical bite. growth pattern having repaired cleft palate,lip and alveolus with severe crowding of Treatment Objectives ●●

●● To achieve harmonious soft tissue and skeletal base relationship. ●● Correction of severe malaligned teeth with respect to the repaired cleft TreatmentCorrection Plan of the open bite and replacement of missing teeth. ●●

●● Initially non extraction ●● Upper arch Expansion with quad helix ●● Secondary alveolar bone grafting with extraction of 15,34 and 44 ●● Alignment and levelling of arches ●● Pre surgical decompensation Maxillary Lefort I osteotomy and mandibular advancement genioplasty337 10th World Cleft Lip, Palate & Craniofacial Congress ●●

TreatmentPost surgical finishing and settling

Treatment was started with quad helix cementation in the upper arch for expansion(Fig 4).Upper and lower bonding were done for initial levelling and alignment followed by extraction of 15,34 and 44 for correction of crowding. Open coil spring was placed between 21 and 24 along with 0.018 Stainless steel wire for creating space and bringing back labially placed 22 into the arch;the lower arch was continued with 0.016 Niti(Fig 5).After 2 months a lower open coil spring was placed between 31 and 33 to create space and bringingbring back the lingually maxilla spaceddownward 32 into and theforward arch.Pre and surgical an augmentation decompensation genioplasty was done prior to surgery.The surgical procedure involved Lefort I osteotomy for for the retrusive mandible(fig 7& 8).Settling and box elastics were continued for another 4 months before the patient was put on retainers and was advised prosthetic rehabilitation.(fig 10 & 11)

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Fig 12 : Occlusion following retention Fig 13:Post treatment extraoral

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Age aspects in application of preoperative orthodontic treatment using the device NAM for children with cleft lip and palate in the Republic of Uzbekistan

Murod M. Jafarov, Laylo I. Pirmuhamedova Republican Center of Pediatric Plastic Surgery, Tashkent pediatric medical institute, , Tashkent, Uzbekistan

Introduction:

The use of pre-operative orthodontic treatment with the device NAM greatly increases the ability to achieve positive results after chelio - and palatoplasty and requires no additional rhinoplasty, reduces tissue tension and the formation of rough scars, does not lead to abuses and distortions of the upper jaw. But there are age-related aspects of this system, which significantly reducesObjective the range of its application.

To determine the efficacy of pre-operative orthopedic treatment with the deviceMaterial NAM and depending research methodson the age of the patient.

We applied the method of pre-operative orthodontic treatment with the device NAM in 24 patients, of these 16 patients for up to 4 months, 8 patients older than 4 months. Among the 24 patients have been 10 bilateral cleft lip and palate, 11 unilateral cleft lips and palate, from 3 incomplete cleft lips with cleft palate median. Treatment was carried out in two stages, depending on the age of the patient at admission. Children under the age of 4 months at admission were conducted from the beginning of the 1st stage- formation of alveolar arch of the maxilla the duration of 3 months after the formation conducted 2nd stage- nose, which lasted an average of 2,5-3 months. Children older than 4 months at admission, these steps were carried out at the same time. 340 10th World Cleft Lip, Palate & Craniofacial Congress Results

Children under the age of 10-14 days at admission pre-operative orthodontic treatment with the device NAM lasted an average of 4.5-5 months, and they were ready for the first stage of the operation - chelioplasty. Children aged 2-3 months were prepared for surgery up to 6-6.5 months. However, it was significantly difficult to achieve the best results with infants older than 4 months at admission, especially in the formation of alveolar arch of the maxilla using the device NAM and it took 2 times longer period than children under the age of 1 month. Given the data can say that, pre-operative orthopedic treatment with the device NAM started before 1 month of age give better and faster results that associated with the presence of glucuronic acid in the blood of the child synthesized during birth under the influence of the mother’s hormone (estrogenConclusion and), bringing softness to bones and excreting in the first 4 months.

It is advisable to start the treatment of patients with cleft lip and palate up to 1 month.

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Long-term effect of maxillary protraction using facemask and miniplate anchorage in cleft lip and palate patients: a long-term follow-up study

Sung Woon On1, Seung-Hak Baek2, Jin-Young Choi1 1Department of Oral and Maxillofacial surgery, 2Department of Orthodontics, School of Dentistry, Seoul National Universit, Seoul, Republic of Korea

Abstract Introduction

patients.The purpose of this study were to investigate long-term effect of maxillary protraction using facemask and miniplate anchorage in cleft lip and palate Materials and methods

A total of 19 patients (Mean protraction duration, 64.63 months) who were treated with facemask and miniplate anchorage for maxillary protraction were included in this retrospective study. Lateral cephalogram were taken before (T1) and after (T2) protraction. Eighteen cephalometric variables were measured for evaluation. Paired t-test and Wilcoxon signed rank test were used forResults statistical analysis.

There were statistically significant differences in eleven cephalometric variables between the T1 and the T2 stage (P<0.05). The point A showed statistically significant forward movement (P<0.05). In addition, counterclockwise rotation of the palatal plane was observed between the T1 and the T2 stage (∆palatal plane angle, -1.37, P<0.05). However, clockwise rotation of the mandible was not observed between the two stages (∆SN to mandibular plane angle, -1.46, P<0.05).

342 10th World Cleft Lip, Palate & Craniofacial Congress Discussion

Treatment with protraction facemask and miniplate anchorage can be an effective modality for adolescent cleft lip and palate patients with maxillary hypoplasia. Although it might not be possible to prevent counterclockwise of the maxilla completely, it is suggested that clockwise rotation of the mandible might be avoidable during treatment with facemask and miniplate anchorage.

343 10th World Cleft Lip, Palate & Craniofacial Congress

Pre natal Diagnosis of Neural tube defects - Screening, Diagnostic aids and their implications

K. Jaghandeep PG 1st Year, MDS OMFS, SRM Dental College, Ramapuram, Chennai, India

Abstract

palate to The extreme incidence malformations of Facial Clefts that may with be associated life threatening. neural It is tube essential defects that is increasing in the recent past with the deformities ranging from simple cleft lip these malformations are identified at the earliest so that the parents are well informed and necessary steps are taken to prevent morbidity form the time of the child birth. The advent of advanced diagnostic aids has made the pre natal diagnosis of these malformations easy and these give an added benefit to the parents who can plan and prepare for the infant and their future pregnancies. The aim of this paper is to describe in detail the prenatal diagnosis and screening for neural tube defects including Fetal Ultrasound, Anomaly scan, fetal MRI and amniocentesis. Reference B. R. BENACERRAF*†, P. M. SADOW‡, C. E. BARNEWOLT§¶, J. A. ESTROFF§¶ and C. BENSON*† Cleft of the secondary palate without cleft lip diagnosed with three-dimensional ultrasound and magnetic resonance imaging in a fetus with Fryns’ syndrome Ultrasound Obstet Gynecol 2006; 27: 566–570 Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/uog.2778

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Compliance of pregnant women of low socio economic group with peri-conceptional folate therapy

Arul Prakash.P, V.B. Krishnakumar Raja, Sneha Pendem SRM Dental College, Ramapuram, Chennai, India

Abstract:

Neural tube defects with facial clefting are more prevalent amongst the low social economic group in our country. Varied etiological factors have been attributed for the same including genetic mutations, Nutritional deficiencies, maternal smoking and alcoholism. Genetic mutations affecting the folate metabolism have gained greater acceptance as a primary etiological factors for the development of cleft lip and palate. It is now established that folate supplementation in the peri-conceptional period can prevent the incidence of fetal NTD’s. The aim of our study is to assess the compliance of the pregnant women of low socioeconomic groups with peri conceptional folate therapy.

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Cleft Lip and Palate: Prenatal diagnosis, prevention and treatment

SREEJEE GOPALAKRISHNAN1, N. ARAVINDHA BAB2 13rd year BDS, 2Oral Pathology and Microbiology, Sree Balaji Dental College & Hospital, Velachery Main Raod, Pallikaranai, Chennai, India

INTRODUCTION ●●

●● Cleft lip +/- cleft palate is one of the commonest of neonatal facial anomalies. ●● In ~80% of cases the two features tend to occur together. ●● It is estimated to occur with an incidence of ~1 in 700 to 1000 live births. ●● The condition results during the 4th to 6th weeks of gestation. It occurs due to failure of fusion of one or more embryonic facial processes. medial nasal prominences lateral nasal prominences maxillary processes FIG.1: Facial processes

FIG.2: Unilateral cleft of a) soft palate, b) nose and lip, c) nose, lip and 346 10th World Cleft Lip, Palate & Craniofacial Congress

hard palate, d) nose, lip, hard and soft palates. FIG.3: Bilateral cleft of e) soft palate, f) nose and lip, g) nose, lip and hard

palate, h) nose, lip, hard and soft palates. ETIOLOGICAL FACTORS environmental factors genetic factors ENVIRONMENTAL●● FACTORS ●● Teratogenic drugs: ●● corticosteroids (anti - inflammatory) ●● phenytoin(anticonvulsant)retinoid ●●

●● Infections: ●● like Rubella during pregnancy Alcohol consumption 347 10th World Cleft Lip, Palate & Craniofacial Congress ●●

●● Smoking ●● Hypoxia during pregnancy ●● Dietary and vitamin deficiencies(like folic acid and vitamin A deficiency) GENETICMaternal ASSOCIATIONS age

• Autosomal dominant developmental malformations, • deafness and dystonia — ACTB • Familial gastric cancer and CLP — CDH1 • Craniofrontonasal — EFNB1 • Roberts — ESCO2 • Holoprosencephaly — GLI2 • ‘Oro facial digital’ — GLI3 • Hydrolethalus — HYLS1 • Van der Woude/popliteal pterygium — IRF6 • X linked mental retardation and CL/P — PHF8 • Stickler type 1 — COL2A1 • Stickler type 2 — COL11A1 • Stickler type 3 — COL11A2 • Desmosterolosis — DHCR24 • Smith–Lemli–Opitz — DHCR7 • Miller — DHODH • Craniofrontonasal — EFNB1 • Kallmann — FGFR1 • Crouzon — FGFR2 • Apert — FGFR2 • Otopalatodigital types 1 and 2 — FLNA • Pierre Robin — SOX9 • DiGeorge — TBX1 348 10th World Cleft Lip, Palate & Craniofacial Congress

• X‑linked cleft palate and ankyloglossia — TBX22 • Treacher Collins — TCOF1 • Loeys–Dietz — TGFBR1 • Loeys–Dietz — TGFBR2 PRENATAL DIAGNOSIS

FIG.4: Prenatal diagnostic aids

MOLECULAR LEVEL ●●

●● CHORIONIC VILLI SAMPLING (CVS) Chorionic villus sampling (CVS) is a prenatal test in which a sample of ●● chorionic villi is removed from the placenta for testing. During pregnancy, the placenta provides oxygen and nutrients to the ●● growing baby and removes waste products from the baby's blood. The chorionic villi are wispy projections of placental tissue that share the ●● baby's genetic makeup. Chorionic villus sampling is usually done between weeks 10 and 13 of pregnancy — earlier than other prenatal diagnostic tests, such as ●● amniocentesis. Chorionic villus sampling can reveal whether a baby has a chromosomal ●● condition, such as Down syndrome. 349 Chorionic villus sampling can also be used to test for other genetic 10th World Cleft Lip, Palate & Craniofacial Congress

●● conditions, such as cystic fibrosis. The sample can be taken through the cervix (transcervical) or the abdominal wall (transabdominal). FIG.5: CVS: A) transcervical, B) transabdominal

FIG.6: Steps in CVS: a) Ultrasound, b) and c) transcervical route, d) Chorionic villi sampling

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FIG.7: CVS procedure

FIG.8: Sampled Chorionic Villi – viewed under microscope AMNIOCENTESIS ●● the foetus in the uterus is withdrawn with a needle and is then sent to the Amniocentesis is a procedure in which the amniotic fluid surrounding

●● laboratory for genetic testing. ●● Approximately 20 ml of amniotic fluid is withdrawn. Cells shed from the skin of the foetus and float in the amniotic fluid. These ●● cells are identified in the laboratory and used for analysis. Genetic amniocentesis is usually done between week 15 and 20 of pregnancy. 351 10th World Cleft Lip, Palate & Craniofacial Congress ●●

Amniocentesis done before week 15 of pregnancy has been associated with ●● a higher rate of complications. If the mother has a Rhesus negative blood group, she will be given an injection of Anti-D after the procedure.

FIG.9:a) Amniocentesis, b) Amniotic fluid in ultrasound

FIG.10: Steps in Amniocentesis

352 FIG.11: Ultrasound of Amniocentesis 10th World Cleft Lip, Palate & Craniofacial Congress

IMAGING ULTRASOUND ●●

It is a noninvasive diagnostic tool used to examine the fetal anatomy to ●● detect malformations. Cleft lip may be detected with ultrasound beginning around the 13th week ●● of pregnancy. As the fetus continues developing, it may be easier to accurately diagnose a cleft lip

FIG.12: Ultrasound of cleft lip

●●

●● Cleft palate that occurs alone is more difficult to see using ultrasound. If prenatal ultrasound shows a cleft, then an amniotic fluid sample is taken (amniocentesis) to identify if the fetus has inherited any genetic syndrome that might cause other birth defects. 353 10th World Cleft Lip, Palate & Craniofacial Congress ●●

●● MRI -MAGNETIC RESONANCE IMAGING Unilateral incomplete cleft lip can be subtle and undetectable until the third trimester. However, minor labial clefting is not usually associated ●● with other malformations and has an excellent prognosis. ●● On MRI, sequential coronal views show the foetal nose and lips. Axial views of the alveolus are helpful to exclude involvement of the gum, ●● which is variable in isolated cleft lip. ●● In contrast to sonography, MRI allows direct visualization of the soft palate. Unilateral cleft lip and palate is best visualized sonographically and on MRI ●● in the axial and coronal planes. Axial views show the characteristic deviation of the nasal septum (i.e., the mid portion is bent to the side of the defect and the anterocaudal portion is deviated to the noncleft side). In addition, axial images show abnormal or ●● missing tooth buds in the medial alveolar ridge. Bilateral clefts extending into the secondary palate are directly visible on ●● MRI. However, Isolated cleft of the secondary palate is rarely identified on prenatal sonography or MRI. FIG.14: Axial MRI shows abnormal contour of tooth bearing alveolar ridge 3D ULTRASOUND “REVERSE FACE” VIEW ●●

Campbell et al described a technique for examining the hard palate by obtaining a 3D volume and rendering an image and viewing the facial features from●● the “inside out”. They termed this imaging approach the “reverse face”●● view. By scrolling the 3D rendered image from inside out, they examined the hard palate in cross section FIG.15: 3D Ultrasound – Reverse face view

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“FLIPPED FACE” VIEW ●●

In the “flipped face” manoeuvre, the foetal face is imaged in the sagittal ●● plane to identify facial anatomy. The 3D static sweep mode is selected instead of the 4-dimensional mode ●● because the former provides a higher-resolution image for 3D rendering. The 3D flipped face technique is a rapid and highly effective method for identification of the foetal lips, alveolar ridges, and hard and soft palates as early as the second trimester of pregnancy. FIG.16: 3D Ultrasound – Flipped face view

ADVANTAGES OF PRENATAL DIAGNOSIS ●●

Time of parental education. 355 10th World Cleft Lip, Palate & Craniofacial Congress ●●

●● Time for parental psychological preparation. ●● Opportunity to investigate other associated anomalies. ●● Gives parents the choice of continuing the pregnancy. PREVENTIONOpportunity for foetal surgery. FOLIC ACID SUPPLEMENT ●●

●● Healthy folate-rich diet Folic acid or multivitamin supplementation: to achieve the red blood cell ●● folate levels associated with maximal protection against neural tube defect. All women in the reproductive age group (12–45 years of age) who have preserved fertility (a pregnancy is possible) should be advised about the benefits of folic acid in a multivitamin supplementation during medical wellness visits. GENE THERAPYFIG.16: Foods rich in folic acid

●●

Gene therapy is a novel treatment method which utilizes genes or short oligonucleotide sequences as therapeutic molecules, instead of conventional ●● drug compounds. This technique is widely used to treat those defective genes which ●● contribute to disease development. Gene therapy involves the introduction of one or more foreign genes into Syndromican organism Associations to treat hereditary or acquired genetic defects.

356 Gene associated anomalies can occur in up to 30-50% of cases. They are mutable and include 10th World Cleft Lip, Palate & Craniofacial Congress

1)aneuploidy syndromic: tends to occur with types II, III and IV • trisomy 13 • trisomy 18 2)Non aneuploidy syndromic • ectrodactyly-ectodermal dysplasia-clefting syndrome-EEC syndrome • • Fryns syndrome • Gorlin syndrome • Juberg-Hayward syndrome • Kallmann syndromeetc.… 3)Non aneuploidy non syndromic • Dandy walker continuum • holoprosencephaly : often with type IV GENETIC• pentalogy ASSOCIATIONS of Cantrell Cleft lip ± Cleft palate

• Autosomal dominant developmental malformations, • deafness and dystonia — ACTB • Familial gastric cancer and CLP — CDH1 • Craniofrontonasal — EFNB1 • Roberts — ESCO2 • Holoprosencephaly — GLI2 • ‘Oro facial digital’ — GLI3 • Hydrolethalus — HYLS1 • Van der Woude/popliteal pterygium — IRF6 Cleft palate• X linked only mental retardation and CL/P — PHF8

• Oculofaciocardiodental — BCOR 357 • CHARGE — CHD7 10th World Cleft Lip, Palate & Craniofacial Congress

• Lethal and Escobar multiple pterygium — CHRNG • Stickler type 1 — COL2A1 • Stickler type 2 — COL11A1 • Stickler type 3 — COL11A2 • Desmosterolosis — DHCR24 • Smith–Lemli–Opitz — DHCR7 • Miller — DHODH • Craniofrontonasal — EFNB1 • Kallmann — FGFR1 • Crouzon — FGFR2 MID –• LINE Apert CLEFT — FGFR2

• Opitz G/BBB — MID1 • Oro facial digital type I — OFD1 FIG.17: Mid – line cleft phenotypes FIG.18: A) and B) Nonsyndromic cleft lip and/or palate (CLP) subclinical

INTRA UTERINE CLEFT LIP SURGERY

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●●

One of the most exciting developments from the human fatal surgery ●● enterprise has been the study of scarless fatal skin wound healing. Unlike adult tissue, fatal dermal repair seems to occur in a fundamentally ●● different way, resulting in scarless wound healing. Fatal skin heals without macroscopic evidence of prior injury, and the ●● dermis reveals a highly organized collagen architecture This phenomenon of scarless fatal skin repair has been observed in mice, ●● rats, rabbits, monkeys and humans. Using the fatal rhesus monkey model, it is determined that an intermediate gland"transition" patterns. wound occurs whereby the wound heals with a normal collagen pattern but without restoration of normal hair follicle and sweat ●●

Wounds created in the ectoderm heal without scar formation on clinical ●● and histological examination. Despite the excellent results obtained but postnatal correction of cleft lips, distortion.it is important to explore the possibility of achieving a scarless repair by intrauterine surgery and its potential in preventing nasal and maxillary

FIG.19:Scarless lip of primate 3 months after birth FIG.20:Biopsy specimen taken from the lip of the same animal, which was operated on at 95 days of gestation.

●●

Epithelial and muscle continuity suggests healing by regeneration. 359 10th World Cleft Lip, Palate & Craniofacial Congress

●●

The results of this fetal cleft lip surgery performed on primates can be REFERENCES:extrapolated for potential application on humans. • Michael J. Dixon, Mary L. Marazita, Terri H. Beaty and Jeffrey C. Murray. Cleft lip and palate: understanding genetic and environmental influences, Nature reviews| Geneticsvolume 12 | MARCH 2011, Pg: 167-178. • The Center for Children with Special Needs Seattle Children’s Hospital, Seattle, WA. Cleft Lip and Palate critical elements of care. Fifth Edition, Revised 10/2010. • NPTEL – Bio Technology – Genetic Engineering & Applications Joint initiative of IITs and IISc – Funded by MHRD Page 1 - 69. Module 8: lecture 1. Gene therapy: introduction and, methods. • Cunningham FG, et al. Prenatal diagnosis. In: Williams Obstetrics. 24th ed. New York, N.Y.: The McGraw-Hill Companies; 2014. http://www.accessmedicine.com. • Gabbe SG, et al. Prenatal genetic diagnosis. In: Obstetrics: Normal and Problem Pregnancies. 6th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.clinicalkey. com. • Patient information: Chorionic villus sampling. http://www.uptodate.com/home. • American College of Obstetricians and Gynecologists (ACOG) Committee on Practice Bulletins — Obstetrics. ACOG Practice Bulletin No. 88: Invasive Prenatal Testing for Aneuploidy. Obstetrics and Gynecology. 2007; 110:1459. • Lawrence D. Platt, MD, Greggory R. DeVore, MD, Dolores H. Pretorius, MD. Improving Cleft Palate/Cleft Lip Antenatal Diagnosis by 3-Dimensional Sonography, the “Flipped Face” View. 2006 by the American Institute of Ultrasound in Medicine • J Ultrasound Med 2006; 25:1423–1430 • 0278-4297/06 • A. Stroustrup Smith1, J. A. Estroff2,3, C. E. Barnewolt2,3, J. B. Mulliken3,4, D. Levine5. Prenatal Diagnosis of Cleft Lip and Cleft Palate Using MRI. AJR: 183, July 2004.

360 10th World Cleft Lip, Palate & Craniofacial Congress • N. JOHNSON, M.SC., M.ORTH, F.D.S.R.C.S. JONATHAN R. SANDY, M.SC., PH.D., M.ORTH, F.D.S.R.C.S. Prenatal Diagnosis of Cleft Lip and Palate. Cleft Palate– Craniofacial Journal, March 2003, Vol. 40 No. 2. • SOGC clinical practice guideline. Pre-conception Folic Acid and Multivitamin Supplementation for the Primary and Secondary Prevention of Neural Tube Defects and Other Folic Acid-Sensitive Congenital Anomalies. No. 324, May 2015 (Replaces #201, December 2007). JUNE JOGC JUIN 2015. • Moore KL, et al. Placenta and fetal membranes. In: The Developing Human: Clinically Oriented Embryology. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2013. • Fadekemi O. Oginni and Anthony T. Adenekan.Prevention of oro-facial clefts in developing world

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Prevalence of superstitious beliefs as an predisposing factor for cleft lip, palate and other birth defects among expectant mothers and their parents

Srinivasan Neduntheru Narasimhan 1, Nalini Velukumar 2 (2nd BDS) Srimathi N.N. 3, Emeritus.E.G.R.Solomon 4 1Thai Moogambigai Dental College & Hospital, 2Dr.M.G.R.Educational & Research Institute University, India INTRODUCTION

It is a general belief among pregnant women that certain superstitions like exposure to eclipse, full moon and several other misconceptions can cause birth defects. This is seen among the educated and uneducated people in India and other developing and developed countries since early times. There is a general fear and apprehension of its consequence to birth defects among the mothers.expectant mothers which is not reported in the literature. Keeping this in mind a randomised study was conducted among the pregnant women and their OBJECTIVES OF THE STUDY

So far there is no evidence to show how far these superstitious beliefs are prevalent among the various population in our country and few other countries. Therefore it was decided to conduct a randomized study to record the various views and opinions among these selected sample groups in a questionnaire formMATERIALS to ascertain AND the METHODOLOGY validity of the hypothesis.

An informative 12 point questionnaire( Annexure i) specific to the investigation wasSample formulated size: to record all the necessary relevant particulars. An total number of 1000 samples which included pregnant women,Inclusive mothers criteria: and close relatives were interrogated.

362 Expectant mothers(pregnant women),mothers and close 10th World Cleft Lip, Palate & Craniofacial Congress

relatives.Method of collection of samples: Beside the direct enquiry of obtaining beliefsinformation, among overseas them. information was also obtained from known contacts through email and social media with a view to ascertain the superstitious

TheRESULTS data thus collected were tabulated and certain conclusions were drawn.

Total number of samples studied were 1000, of which 260 were pregnant women. 800 samples were of Indian origin.The remaining consisted of persons from overseas. Among the Indian women samples 210 women were pregnant, the remaining were mothers in their productive age. Among the overseas population 50 women were pregnant Further the pregnant women divided into three groups according to the status of their trimester.(Table 1.) ------Trimester status Indian pregnant ladies Overseas pregnant ladies st

nd 1 60 12 rd 2 82 20 3 68 18 ------Table1-Trimester status and distribution among the survey population A team consisting of dental students and a social worker was constituted to conduct the survey their beliefAmong in thesuperstitions. 800 samples from India 680 persons agreed that they believed in superstitions, while in the overseas samples of 200 persons 170 indicated

The common superstitions observed were eclipse, rural samples avoided squatting in the main door step, sewing during pregnancy, exposure to full moon and new moon, passing by burial grounds, climbing hill in public or private transport, avoid going out of their house after sunset. In the total samples studied 790 samples(79%) which included pregnant women and mothers strongly believed in avoiding eclipse as it was probably a predisposing factor.760 (76%) persons believed that sewing during pregnancy should 363 be 10th World Cleft Lip, Palate & Craniofacial Congress avoided. The remaining causes which they feared are given in Table.No.2 their beliefAmong in thesuperstitions. 800 samples from India 680 persons agreed that they believed in superstitions, while in the overseas samples of 200 persons 170 indicated

The common superstitions observed were eclipse, rural samples avoided squatting in the main door step, sewing during pregnancy, exposure to full moon and new moon, passing by burial grounds, climbing hill in public or private transport, avoid going out of their house after sunset. In the total samples studied 790 samples(79%) which included pregnant women and mothers strongly believed in avoiding eclipse as it was probably a predisposing factor.760 (76%) persons believed that sewing during pregnancy should be avoided.SUPERSTITIONS The remaining causes which they feared are given in Table.No.2 ●●

●● Avoid wearing new clothes ●● Two pregnant women shouldn’t see each other ●● Husband should not go for fishing or snorkelling ●● Should not kill any insect or animal ●● No henna ●● Avoid sitting during thunderstorm ●● Avoid noise and crowd ●● Avoid gifts ●● WipeTie red the ribbon acne witharound red the cloth waist of pregnant lady ●●

●● Avoid looking at an object for longer time ●● Should not dig well / holes ●● Should not cut/pluck any part of tree ●● Should not undergo any dental procedure ●● Should not tie garland/scarf around neck ●● Hands should not be raised above shoulder level ●● Baby products should not be bought during pregnancy 364 Should not visit river/water bodies 10th World Cleft Lip, Palate & Craniofacial Congress ●●

●● Should not walk under spider web ●● Should not visit parlours ●● Should not take bath In hot water ●● Couples should exchange information about their ●● previous relationship ●● Round fruits and vegetables should be consumed Table.No.2.ListWet clothes ofshould other be superstitious kept on head beliefs during hiccups.

Food which they avoided during pregnancy are given in Table No.3 Table.No.3(a to g) List of food products that need to be avoided during pregnancy Watching horror/violent movies was seriously avoided during the term of pregnancy in as many as 79% of the persons questioned. Only 33% developed phobia in seeing handicapped children. None of the persons have read nor heard in the media that superstition may cause birth defects. 59% have heard from other pregnant women that superstition is likely to cause birth defect.80% of persons interviewed expressed that they will advice the future generation about the significance of superstitions and traditional beliefs to be

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followedDISCUSSION during pregnancy.

In the present day period with the scientific and educational advancements, old age superstitions and traditional beliefs are generally in the decrease. The belief in superstitions among previous generations is not generally accepted by the present day generation in the urban areas. However certain superstitions which affect the health of the individual is still a cause for fear and apprehension. One such fear is the traditional superstitions observed among families during the term of pregnancy in spite of the educational and social status, this fear persists among the pregnant mothers as it can affect the health of their off springs. With an unbiased mind this study was conducted to prove or disprove this hypothesis, if so its magnitude. The most common traditionally believed superstition is the exposure to eclipse and new moon which is rather difficult to remove from the minds of expectant mothers. It was seen in a large percentage. To find out such influence as one of the predisposing factor a longitudinal study right from inception of pregnancy till parturition has to be conducted which is beyond the scope of this study. In its absence one cannot point out that exposure to eclipse is harmful to pregnant women. The traditional belief exists but its not proved that it is harmful. Unless it is shown that these traditional beliefs are a source of contributing cause to birth defects, these traditional beliefs will continue to exist nor it will be possible to propagate the community from its dissociation366 to these beliefs. 10th World Cleft Lip, Palate & Craniofacial Congress

This study has been beneficial from the view point to know that traditional beliefs still continue to exist among the various sections of populations and their conviction to avoid them during pregnancy. with their mothers.The study is planned to a larger sized samples extending This belief is not only confined to pregnant women but its also associated overACKNOWLEDGEMENT a period of time.

We would like to thank the following persons for helping us in collecting data from various parts of the world viz., Lakshmi.N (Tamil nadu), Geetha velukumar (Karnataka), Anuj Marhetta(Haryana), Zareena Mir (Leh & Ladakh), Manas Bajpai (Rajasthan), Meeta Sharma (Gujarat), Deepesh Prajapati (Rajasthan), Hemeshwari Laishram (Manipur), Afsha Wani (Jammu & Kashmir), Upasna Dutt (West Bengal), Radhakrishnan (Kerala), Rameela syed (Kerala), Lalitha narasimhan (Telengana), Shalini dinesh (Tamil nadu), Suman rishi (Punjab), Alaka rao (Maharasthra). Sindhuja (Srilanka), Nawaz Ali(Pakistan), Shamma Mattar Altunaiji (UAE),Moza Matar Altunaiji (UAE), Roumaissa Belkadi (Algeria), Williams(U.K.), Ahmad Badruddin Ghazali (Malaysia), Thanakorn Thimkam (Thailand), Latyshev Andrey (Russia), Houssam Al Deen Hamad Askar (Lebanon), Abdullah Taufiq (Yemen), Ilunga Mulopwe Sylvian (Congo), Merna Maher Firky Gres (Egypt), Bibhav dubey (Nepal), Christina (USA). ANNEXURE ENGLISH VERSION OF THE QUESTIONNAIRE FORM:-

PREVALENCE OF SUPRSITIOUS BELIEFS AS A PREDISPOSING FACTOR FOR CLEFT LIP AND CLEFT PALATE BIRTH DEFECTS AMONG EXPECTANT MOTHERS AND THEIR PARENTS. NAME ______AGE ______STATUS Expectant / Mother/Relative CONTACT NUMBER______FINANCIAL STATUS OF FAMILY______NATIVITY ______COMMUNITY ______RELIGION______

367 10th World Cleft Lip, Palate & Craniofacial Congress

NUMBER OF CHILDREN______EDUCATIONALMOTHERHOOD QUALIFICATION STATUS: ______OCCUPATION______PREGNANCY STATUS : EXPECTANT MOTHER / MOTHER FIRST/ SECOND/ THIRD TRIMESTER 1. Do youq believeq in superstitions? Yes No 2. Mention the list of superstitions: 3. Any qcongenital q anomalies among your family members ? Yes No 4. Are there any specific advice and restrictions given to you by your elders in the familyq duringq pregnancy to avoid birth defects? Yes No 5. If yes: Mention the restrictions 6. What are the specific advices given about the foods to be avoided during Vegetables______pregnancy for the fear of Greens______birth defects? Fruits ______

7. Have you watched violent/horror movies,television shows during your termq of pregnancy?q Yes No 8. Have you heard from other pregnant women that superstitious beliefs may causeq birthq defects? Yes No 9. Do you have any phobia of seeing or meeting handicapped children during yourq termq of pregnancy? Yes No 10. Have you anytime read that superstitions may cause birth defects? Yes No If so how? News paper, Magazine or visual media 11. Do any of your blood relatives have congenital anomalies which they relate it to qthe superstitions?q

368 Yes No 10th World Cleft Lip, Palate & Craniofacial Congress

12. Would you ask your children to believe in the ancient traditional superstitions?q q Yes No

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Gestational smoking and Alcohol abuse as etiological factors for cleft lip and palate. a growing concern in india

Manonmani Pavithra .R 1, Naveen kumar.J2, Ravindran.C3, 1Final year 2Professor, 3Professor and Head Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Sri Ramachandra University, Porur, Chennai 600116, India

Abstract

Oro-facial clefts are a grossly disfiguring deformities with multifactorial aetiology. Aetiology for these clefts can be broadly grouped into genetic and environmental aspects. But it is often hypothesised that a combination of factors could contribute significantly towards this condition rather than a lone causative. Though, vices like maternal smoking and alcohol consumption during gestation are often implicated for orofacial clefting, the typical cause-effect relationship is yet to be concretely established. Several studies investigating the effects of smoking and alcohol to oral clefts have only reported conflicting results. A meta analysis of these studies concluded that the overall odds ratio for cleft lip due to smoking was 1.29. A gene-environment interaction has been proposed in the occurrence of oral clefts in children with the rare allele C2 of the transforming growth factor- alpha, a gene born to women who smoked during pregnancy. Though the current evidence only suggests a weak correlation with smoking and alcohol consumption during the first trimester of gestation, the condition is bound to have a significant impact on public health. Improvement in lifestyle and social status in a developing economy like India does have a few down-sides, one among them is the adoption of vices which were hitherto considered a social stigma. Smoking and alcoholism amongst women especially of child bearing age is of growing concern for our country and this paper aims at highlighting the particular issue to create awareness amongst healthcare professionals who are first line of contact for majority of the patients. 370 10th World Cleft Lip, Palate & Craniofacial Congress Introduction

Cleft lip / palate happens to be the most common congenital malformation reported, with an average incidence of about 1 in 700 live-births. Being a polygenic, multifactorial disorder, oro-facial clefting, has both genetic and environmental factors contributing to its aetiology. Any factor(s) inhibiting the flow of cells or decrease their number, making the contact between the facial prominences inadequate or impossible , will result in orofacial clefting1. Maternal iniquity , especially smoking and alcoholism are thePathogenesis commonest environmental causes for adverse birth outcomes.

Epidemiological and experimental stats suggest that environmental risk factors play a significant role in NON SYNDROMIC CLEFT LIP AND PALATE. David Precious outlined the major events and processes leading to the formation of●● oro-facial clefting : ●● Abnormal migration of neural crest cells ●● Altered rate of cell division and/or cell death ●● Abnormal localisation of post migratory neural crest cells ●● Defective epithelial-mesenchymal interactions Gene defects in the production of cell specific molecules such as collagen type 1 and 2 or cartilage specific proteoglycan , defective synthesis, ●● deposition, and/or degradation of extracellular matrices ●● Inability to maintain the differentiated cell state

systemsImpaired interaction between skeletal, muscular, nervous and/or vascular components because of defective development in one or more of these ●●

Abnormal hormonal environment caused either due to defective production ●● of hormone or impaired receptor function. Changes in the timing of development leading to uncoupling of normally coupled process2. Cellular level interplay between ectoderm and mesoderm involving differentiation, growth, apoptosis, adhesion etc contributes to the development of oro-facial architecture. And naturally, alteration this complex mechanism at genetic or environmental level will result in clefting3 371 10th World Cleft Lip, Palate & Craniofacial Congress Smoking and Oro-facial Clefts

There is uncertainty over whether maternal smoking is associated with birth defects. Smoking is the only consistent common risk factor, although the strength of the effect is very modest (Leite et al., 2002). Allan Hacksaw et al conducted the first ever comprehensive systematic review involving 173687 malformed children to establish which specific malformations are associated with smoking. Amongst other congenital anomalies, significant positive associations with maternal smoking were found for oro-facial clefts (OR 1.28, 95% CI 1.20–1.36)4. Smoking during the first trimester of gestation is proposed to be associated with a high risk of having a cleft child (11- 12%) The mechanism behind this is supposed to be tissue hypoxia to the developing embryo impeding growth, particularly in the palate. This is concurrently proven by animal studies also 5. Smoking also causes folate deficiency, which directly affects the developing embryo resulting in oro-facial clefting. Malformed infants born to smoking mothers have deficient detoxification mechanisms involving cytochrome p-450. Oro-facial clefting attributed to maternal smoking is found to be dose- related. Investigating possible embryonic influence of smoking is of prime concern since it could provide scope for counselling and malformation prevention. Amongst the several biomarkers of exposure to cigarette smoking, cotinine, the main nicotine metabolite is the most resourceful marker for nicotine exposure. This biomarker can be evaluated from blood, saliva, urine andMaternal amniotic Alcoholism fluid. and Clefting

Alcohol exposure during early neurulation induces significant changes in DNA methylation patterns and gene expression that may contribute to Foetal Alcohol Syndrome (FAS). FAS is well-documented entity which is a possible result of maternal alcoholism during the first trimester of pregnancy. It is believed that oro-facial clefting as a result of FAS is also dose dependant, occurring in cases where consumption of alcohol is more than 8 to 10 drinks per day. Commonly performed assessment about intensity of maternal alcoholism include 1.Maximal number of drinks in any one day (Maximal Intensity) 2.Number of drinking days per week (Frequency), 3.Number of drinks per drinking day (Average intensity) 6 Various biological markers of alcohol consumption includes mean red cell volume, I3 glutamyl-transpeptidase, acetaldehyde in whole blood and372 carbohydrate deficient transferrin. All four have different sensitivity 10th World Cleft Lip, Palate & Craniofacial Congress and specificity. Stole et al (1998) found that concurrent use of all 4 markers providedConclusion more accurate interpretations than each of them used individually.

Oro-facial clefting causes significant physical and psychological morbidity to a child and the parent which is often life-long irrespective of whether the condition is treated early or otherwise. Commensurate to this is the burden of healthcare for the individual as well as society. Though the significance level of the relation to maternal vices like smoking and alcoholism is sub- optimal, the condition does not permit a casual attitude amongst healthcare personnel and women of child bearing age. The exponentially westernising Indian subcontinent also imbibes lifestyle modernisation and hence along with the other benefits, has a huge price to pay in terms of conditions which were hitherto attributed to western lifestyle such as smoking and alcoholism. Hence, it is advisable to include advise on quitting smoking/alcoholism during gestationReferences in heath-educational materials, particularly targeting young women. 01. Bhatia SN. Genetics of cleft lip and palate. Br Dent J. 1972;132:95–103. 02. Precious DS. Oral and Maxillofacial Surgery. 1st ed. Philadelphia: WB Saunders Company; 2000. Cleft lip and palate. 03. Prescott NJ, Winter RM, Malcolm S. Nonsyndromic cleft lip and palate: complex genetics and environmental effects. Ann Hum Genet. 2001;65:505–15. 04. Allan Hackshaw. Maternal smoking in pregnancy and birth defects: a systematic review based on 173 687 malformed cases and 11.7 million controls Hum Reprod Update. 2011 Sep-Oct; 17(5): 589–604. 05. Wyszynski DF, Beaty TH, Maestri NE. Genetics of nonsyndromic oral clefts revisited. Cleft Palate Craniofac J. 1996;33:406–17. 06. Cohen MM. 2nd ed. New York: Oxford University Press; 1997. The child with multiple birth defects.

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Association of DHFR and 10q25.3 with Non- syndrome Cleft Lip with or without Cleft Palate in Northern China

Li Zengjian Hospital of Stomatology, China Medical University, Liaoning, China

Abstract

Non-syndrome Cleft lip with or without cleft palate (NSCL/P) is one of the most common congenital deformity, notable for significant lifelong morbidity and its complex etiology . Recently,the researches about NSCL/P focus on its gene mapping, some of which have shown that DHFR gene and 10q25.3 chromosome may contribute. We recruited 220 NSCLP patients (including 138 core families) and 180 healthy concrols, to explore their associations with the risk of this deformity in the northern Chinese population. The variant detections in this study were single nucleotide polymorphism analysis. Firstly, we extracted Genomic DNAs from peripheral blood of the samples, and then used the nucleic acid spectrometer to quantify the density of the DNAs. Polymerase chain reaction of the target gene was performed using the primers designed by Zucchero. PCR-restriction fragment length polymorphism(PCR-RFLP) was used to identify the genotypes of the experimental group, which were analyzed by case-control family study at last. The gene frequency of case and control were all conformed to HW test. In case-contral analysis, both of the genes have no significant differences in frequency distributions of genotypes or alleles in the group with cleft lip and cleft palate or cleft lip and palate from those in controls. TT(GA) genotype of rs11742688 and rs7078160 in case groups were normal (DHFR rs11742688, p>0.05, 10q25.3 rs7078160, p>0.05, respectively). A transmission disequilibrium test showed that they were all transmitted sequilibrium in rs11742688 and rs7078160. In conclusion, the polymorphism of rs11742688 and rs7078160 is not related to NSCL/P in northern Chinese population. There is no significant difference in C/T(G/A) genotype between case and control groups in this experiment, and there is no risk who carry 374CT(GA) allele. 10th World Cleft Lip, Palate & Craniofacial Congress

Association study of candidate genes ABCA4 and MAFB with NSCL/P in Northeast Han Population

Wu Nan Heping District, People’s Republic of China

Objective

To explore the relationship of candidate gene ABCA4 loci rs481931, rs560426 and MAFB loci rs17820943 of single nucleotide polymorphisms and mutations in the pathogenesis of the non-syndromic cleft lip and palate in NortheastMethods Han population;

It was a case-control study conducted in China Medical University, Hospital of Stomatology. 5 ml peripheral blood were drawn form 210 patients and 184 healthy controls, including 98 nuclear families (children and parents), a total of 590 blood samples. The DNAs in the blood sample were amplified by polymerase chain reaction - restriction fragment length polymorphism (PCR- RFLP) technique. The single nucleotide polymorphism analysis was carried for rs481931, rs560426, rs17820943 site. The Hardy-Weinberg equilibrium inspection was used to test whether the objects of this study were from the same group of Mendelian inheritance. Genotype and allele of the patients, their parents and control group were calculated. Genotype and allele frequencies and constitution were compared between case group and control group. The OR value and 95% confidence interval were used to express the results. FBAT was used for genetic pedigree relevance analysis of complex diseases. In this study, all the statistical analysis was done by SPSS20.0 statistical software. And FBATResults was done by FBAT5.0 software.

Among all the cases of non-syndromic cleft lip and palate in this study, there were 116 males, with an average age of 11.8; and 94 females, mean age 11.3 years. Compared with the control group, the research showed that the genotype and allele frequencies of rs481931, rs560624 had no statistical 375 10th World Cleft Lip, Palate & Craniofacial Congress significant (P>0.05). But the genotype frequencies of rs17820943 in cleft lip group (P=0.035) and the total number of cases group (P=0.010) had statistical significant. Case-control study of rs17820943 was separated to find out the influence of genotype. CT genotype [P=0.023, OR=0.446 (0.219-0.906)] was much higher than the normal control group, which showed statistical significant. But the TT, CT+TT genotype frequencies of rs17820943 had no significant difference (p>0.05), compared with the CC genotype. FBAT analysis showed, in C allele of rs481931, Z=2.063, P=0.039<0.05 which had statistical significant. It meant excessive transfer of C allele from parents to children and indicated the the C allele associated with the occurrence of cleft lip and palate. HBAT analysis showed that haplotype combinations was C allele (rs481931) and G allele (rs560426), the Z=2.882, P=0.004<0.05 which showed statistical significant. The haplotype combinations of rs481931 C allele and rs560426 G alleleConclusions were associated with the occurrence of cleft lip and palate.

CT genotype of rs17820943 allele MAFB gene is a protective factor in the incidence of cleft lip and palate, which carry the risk of people suffering from cleft lip CT genotype was 0.466 times the CC genotype. ABCA4 rs481931 sites C allele gene is passed over, and is therefore related to the occurrence of cleft lip and palate. Two loci rs481931 and rs560426 ABCA4 gene haplotype combinations when the C allele of rs481931 and rs560426 G allele and the incidence of cleft lip and palate related.

376 10th World Cleft Lip, Palate & Craniofacial Congress

A heterogygous mutation of ARHGAP29 gene in Indian

Deepak C Professor of Orthodontics, Balaji Dental College & Hospital, Velacherry, Chennai, India ABSTRACT

Mutations in genes that regulate of palatal region of oral cavity have been associated with occurrence of non-syndromic cleft palate, cleft lip with or without cleft palate (NS-CP/CL/CLP). ARHGAP29 is a Rho1 specific GTPase that acts to convert active form of Rho1-GTP to inactive Rho1-GDP. Base change and nonsense mutations have been identified in exon 1 of ARHGAP29 gene that impairs its function in subjects with NS-CP/CL/CLP of North American and Filipino decent. Studies from the Indian Genome Variation Consortium (IGVC) have suggested the genotype of Indian population to be distinct from HapMap populations. This prompted us to design the present study to investigate whether exon 1 of ARHGAP29 carried mutation in Indian subjects with NS-CP/CL/CLP. Total genomic DNA extracted from peripheral blood of 72 subjects with NS-CP/CL/CLP and equal number of control healthy subjects was amplified with primers flanking exon 1 of ARHGAP29 gene. Direct sequencing of the ARHGAP29 PCR amplicons showed a nonsense mutation at codon 32 within exon 1 in a subject with NS-CLP. The mutation, however, occurred in heterozygous condition and caused substitution of lysine to stop codon in one copy of ARHGAP29. None of the other subjects carried mutation in this region. The study has thus identified a rare but novel truncation mutation inKey ARHGAP29 words: gene for the first time in NS-CLP.

ARHGAP29, ARHGAP29 mutation, ARHGAP29 mutation in Indian subjects, gene mutation in cleft lip and cleft palate, gene mutation in Indian cleft lipINTRODUCTION and cleft palate

The development of maxillary division of oro-facial region is regulated by a cascade of signaling molecules, the expression and/or activation of which is tightly regulated. Disturbances in the signaling cascade due to function377 10th World Cleft Lip, Palate & Craniofacial Congress impairing defects in developmental genes may cause failure of meeting and fusion of the developing primordium. The extent of manifestation though may vary among the affected children and may include either cleft lip (CL) or cleft palate (CP) (isolated CL or CP) or cleft lip with cleft palate (CL/P)[1], and may involve either one or both halves of the facial region, in which case they are classified as unilateral or bilateral clefts.[2] Oro-facial cleft may either occur as an isolated event or as a part of other clinical symptoms and hence are designated as non-syndromic or syndromic cases respectively. Nonsyndromic clefts makes about 70 percent of all reported oro-facial clefts.[1,2] Syndrome associated clefts occur in several hundreds of mendelian (autosomal dominant, autosomal recessive, or X-linked) chromosomal conditions[3], that may at times include mental retardation, myopia and cardiac anomalies[4-8] as additional clinical manifestation. Of the above two, nonsyndromic clefts of the lip and palate (CL, CP, CL/P) occurs in every 1/500–1/2500 of live births[9,10] and hence is considered as the most common of all congenital defects. The cause of development of CL, CP and CL/P is believed to be multi- factorial in nature and may include both environmental agents and genetic defects.[11] Two major environmental agents that the developing embryos get exposed to are byproducts of tobacco smoke and alcohol binging by expectant mothers especially during the early trimester. Both of these agents have been found to be associated with development of oro-facial clefts in infants.[12- 23] Besides the above, oro-facial cleft has also been observed in the fetus of mothers undergoing phenytoin drug therapy for epileptic conditions or who were consuming dietary supplement, retinoic acid.[21,22] Though the exact the mechanism of development of oro-facial clefts in embryos exposed to these environmental and dietary agents remains yet to be understood, association between functional impairment of molecules responsible for detoxification of environmental agents and drugs and oro-facial clefts have been observed in infants. For example, significant over transmission of G590A variant of NAT2 gene coding N-acetyltransferase 2 enzyme that activates and deactivates arylamine and hydrazine drugs and carcinogens was observed in subjects with oro-facial clefts relative to controls.[23,24] On the other hand, genetic alteration in developmental regulators of palatal shelf such as TGFA, TGFβ,[25,26] MSX1,[27] IRF6,[28,29] PVR, PVRL1, PVRL2,[30,31] TBX22,[32] FGFR1,[33] ABCA4,[34,35], MAFB1[34,35] and ARHGAP29 have also been observed in several subjects with oro-facial clefts. Of the above developmental regulators, ARHGAP29 gene encodes for a GTPase activating protein (GAP), which has a strong affinity to the small GTP binding378 protein RhoA than Rac1 or CDC42.[36] By binding to the active form 10th World Cleft Lip, Palate & Craniofacial Congress

of RhoA, the GTPase function of ARHGAP29 converts RhoA-GTP to RhoA- GDP.[37] Expression of ARHGAP29 was observed in the mandibular and maxillary processes of developing mouse embryo at E10.5 and the shelves of secondary palate at E13.5.[37] Nonsense mutation with consequent premature truncation of the protein has been identified within the coding region of exon 1 of ARHGAP29 gene in oro-facial cleft subjects from other races.[37] Studies from the Indian Genome Variation Consortium (IGVC) have suggested that most of the populations in the Indian subcontinent are distinct from HapMap populations, and that the genetic basis of diseases in the Indian population may be different due to differences in the risk allele frequency and pattern of linkage disequilibrium.[38] This prompted us to design the present study, in which, the exon 1 of ARHGAP29 gene was investigated in a small group of Indian subjects withMATERIALS CL, CP or AND CL/P, METHODS who belonged to a defined South Indian Dravidian race.

Study design and subjects: A cross sectional study was designed and sixty subjects with either non-syndromic cleft lip or cleft palate or cleft lip with cleft palate, but otherwise medically able were included in the study after obtaining informed consent. As controls, an equal number of race matched healthy volunteers were included. Subjects with syndromic cleft lip or cleft palate or cleft lip with cleft palate, and those who were not willing to be a part of the study were excluded. DNA extraction and PCR: 200 µl of peripheral blood was processed to extract chromosomal DNA with Gen-Elute Blood DNA Extraction Kit (Sigma- Aldrich, USA) according to the manufacturer’s protocol. The exon 1 region of ARHGAP29 was amplified with intronic primers (sequence information available upon request) flanking splice donor and acceptor sites. The gene region was amplified under following conditions: Initial denaturation at 94°C for 5 min, followed by 35 cycles of denaturation at 94°C for 45 seconds, primer annealing at 55°C for 45 seconds, primer extension at 72°C for 1 min, with a final extension at 72°C for 5 min. The PCR amplicons were confirmed in comparison to DNA size markers and were subsequently eluted with Genelute DNA gel elution kit (Sigma Aldrich, cat# NA1111) and subjected to direct sequencing withRESULTS same set of primers that were used for PCR amplification.

In order to determine the occurrence of mutation in exon 1 of ARHGAP29 gene in Indian subjects with either non-syndromic cleft lip or cleft palate or cleft lip with cleft palate, and thus to understand the risk potential 379 of mutant ARHGAP29 in the development of CL/CP/CLP, we screened the 10th World Cleft Lip, Palate & Craniofacial Congress chromosomal DNA from sixty CL/CP/CLP subjects along with equal number of healthy volunteers. DNA from peripheral blood was amplified with exon 1 specific primers and the PCR amplicons were subjected to direct sequencing after gel purification. Analysis of the sequencing data showed a nonsense mutation, c.94A>T, in a subject with NS-CLP that resulted in the substitution of lysine amino acid with stop codon at codon position 32 in exon 1 (AAG32TAG). However, the mutation was identified to be heterozygous as both wild type AAG andDISCUSSION mutant TAG codons were present at codon position 32 [Figure 1].

A total of 60 subjects with non-syndromic CL, CP or CLP and 60 normal subjects were analyzed for the presence of mutation in ARHGAP29 gene. Both groups of subjects were selected from Tamil speaking Dravidians from Southern region of India, in order to minimize race dependent genetic variations, as genetic diversity has been observed in population from different parts of India.[38] – Direct sequencing analysis of PCR amplicons of coding regions of ARHGAP29 identified a single mutation in a subject with NS-CLP. The mutation caused substitution of lysine amino acid at codon 32 with a premature stop codon in one copy of ARHGAP29, as the mutant codon occurred along with wild-type lysine codon (indicated by two arrows in mutant ARHGAP29 panel in Figure 1). The identification of K32STOP mutation in only one of sixty CL, CP or CLP subjects who were investigated in the present study indicates that the mutation may be a rare event in CL, CP or CLP from this region. By analyzing 1440 and 360 subjects with CL, CP or CLP from Philippines and US respectively, Leslie EJ et al. identified a nonsense (c.62_63delCT) and a missense mutation (c.76A>G) in two independent subjects from Philippines, and a missense mutation (c.137A>G) in a subject from the US.[37] Considering the occurrence of c.94A>T mutation in a single NS-CLP subject from a sample size of fifty , it may be inferred that the relative prevalence of this mutation may be higher than those observed by Leslie EJ et al. This claim, however, requires to be confirmed by analyzing a higher sample size by including subjects from similar racial background. Both nonsense mutations, c.94A>T identified by us, and c.62_63delCT causes premature truncation of the ARHGAP29 protein. While c.94A>T creates an in-frame stop codon, c.62_63delCT causes a frame-shift that consequently results in the premature occurrence of stop codon after twenty misread codons. Both mutations, however, are expected to cause loss of function of ARHGAP29 protein. In contrast, the two missense mutations, c.76A>G and c.137A>G that causes380 substitution of threonine with alanine at codon 26 (T26A) and lysine 10th World Cleft Lip, Palate & Craniofacial Congress

with arginine at codon 46 (K46R) respectively are expected to have a benign effect on the function of ARHGAP29.[37] The identification of c.94A>T mutant allele of ARHGAP29 in heterozygous condition along with wild-type allele suggests that the mutation event may result in haplo-insufficiency. It is important to note that the deletion mutation (c.62_63delCT) reported by Leslie EJ et al. was of homozygous in nature.[37] Although biallelic inactivation of genes has often been described as a cause for development and progression of diseases, haplo-insufficiency of genes has also been observed in several disease conditions. For example, haplo-insufficiency has been observed in cancerous lesions,[39,40,41] neurodegenerative disorders,[42] myopathies,[43] inflammatory conditions,[44] and developing tissues leading to birth defects including syndromes such as Frias syndrome (haplo-insufficiency of BMP4),[45] and CNS malformation syndrome (haplo-insufficiency of NFIA).[46] These findings clearly suggest that the heterozygous c.94A>T mutation identified in the present study may present haplo-insufficiency effect during the development of oro-facial cleft region. As expression of ARHGAP29 transcript is detected strongly in medial and lateral nasal processes and to a lesser extent in the mandibular and maxillary processes at E10.5 and shelves of the secondary palate at E13.5,[37] the haplo- insufficiency effect of c.94A>T mutant ARHGAP29 may have However, a knock-in experiment to recreate the above heterozygous condition along with other reported mutations in transgenic mice is essential to validate the above observation.CONCLUSION

The present study has identified for the first time a novel nonsense mutation c.94A>T in a South Indian Dravidian subject with NS-CLP. The occurrence of the mutation in one of fifty subjects with NS-CL/CP/CLP suggests thatREFERENCE the mutation event may be rare. 1. Lidral AC, Moreno LM and Steven A. Genetic Factors and Oro-facial Clefting. Sem in Orthod 2008;14:103-114. 2. Jugessur A, Murray JC. Oro-facial clefting: recent insights into a complex trait. Current Opinion in Genetics & Development 2005;15:270-278. 3. Carinci F, Scapoli L, Palmieri A, Zollino I, Pezzetti F. Human genetic factors in nonsyndromic cleft lip and palate: an update. Int J Pediatr Otorhinolaryngol. 2007;71:1509- 19.

381 10th World Cleft Lip, Palate & Craniofacial Congress 4. Hu DN, Li JH, Chen HY, Chang HS, Wu BX, Lu ZK, Wang DZ, Liu XG. Genetics of cleft lip and cleft palate in China. Am J Hum Genet 1982;34:999-1002. 5. Venkatesh R. Syndromes and anomalies associated with cleft. Indian J Plast Surg. 2009;42:S51-5. 6. Murray JC. Face facts: genes, environment, and clefts. Am J Hum Genet. 1995;57:227-32. 7. Lacombe D, Pedespan JM, Fontan D, Chateil JF, Verloes A. Phenotypic variability in van der Woude syndrome. Genet Couns.1995;6:221-226. 8. Stottmann RW, Bjork BC, Doyle JB, Beier DR. Identification of a Van der Woude syndrome mutation in the cleft palate 1 mutant mouse. Genesis. 2010;48:303-8. 9. Chaturvedi P, Banerjee KS. Spectrum of congenital malformations in newborns from rural Maharashtra. Indian J Pediatr. 1989;56:501-7 10. Grosen D, Chevrier C, Skytthe A, Bille C, Mølsted K, Sivertsen A, Murray JC, Christensen K. A cohort study of recurrence patterns among more than 54,000 relatives of oral cleft cases in Denmark: support for the multifactorial threshold model of inheritance. J Med Genet. 2010;47:162-8. 11. Mossey PA, Little J, Munger RG, Dixon MJ, Shaw WC. Cleft lip and palate. Lancet. 2009;374:1773-85. 12. Chung KC, Kowalski CP, Kim HM, Buchman SR. Maternal cigarette smoking during pregnancy and the risk of having a child with cleft lip/palate. Plast Reconstr Surg 2000;105:485-491. 13. Munger RG, Romitti PA, Daack-Hirsh S, Burns T, Murray JC, Hanson J. Maternal alcohol use and risk of oro-facial cleft birth defects. Teratology. 1996;54:27-33. 14. Romitti PA, Lidral AC, Munger RG. Candidate genes for nonsyndromic cleft lip and palate and maternal cigarette smoking and alcohol consumption: evaluation of genotype- environment interactions from a population-based case-control study of oro-facial clefts. Teratology 1999;59:39-50. 15. Romitti PA, Sun L, Honein MA. Maternal periconceptional alcohol consumption and risk of oro-facial clefts. Am J Epidemiol 2007;166:775-78. 16. Saad AY, Gartner LP, Hiatt JL. Teratogenic effects of nicotine on palate formation in mice. Biol Struct Morphog 1990;3:31-35. 17. Saito T, Cui XM, Yamamoto T. Effect of N′-nitrosonornicotine (NNN) on murine palatal fusion in vitro. Toxicology 2005;207:475-485.

382 10th World Cleft Lip, Palate & Craniofacial Congress 18. Seller MJ, Bnait KS. Effects of tobacco smoke inhalation on the developing mouse embryo and fetus. Reprod Toxicol 1995;9:449-459. 19. Shi Min, Wehby GL, Murray JC. Review on Genetic Variants and Maternal Smoking in the Etiology of Oral Clefts and Other Birth Defects. Birth Defects Res C Embryo Today. 2004;84:16-29. 20. Kang P, Svoboda KK. Nicotine inhibits palatal fusion and modulates nicotinic receptors and the PI-3 kinase pathway in medial edge epithelia. Orthod Craniofac Res. 2003;6:129- 142. 21. Hunt JR. Teratogenicity of high vitamin A intake. N Engl J Med. 1996;334:1197. 22. Cobourne MT. The complex genetics of cleft lip and palate. Eur J Orthod. 2004;26:7-16. 23. Ramirez D, Lammer EJ, Iovannisci DM, Laurent C, Finnell RH, Shaw GM. Maternal smoking during early pregnancy, GSTP1 and EPHX1 variants, and risk of isolated oro- facial clefts. Cleft Palate Craniofac J. 2007;44:366-73. 24. Shi M, Christensen K, Weinberg CR, Romitti P, Bathum L, Lozada A, Morris RW, Lovett M, Murray JC. Oro-facial cleft risk is increased with maternal smoking and specific detoxification-gene variants. Am J Hum Genet. 2007;80:76-90. 25. Lidral AC, Murray JC, Buetow KH. Studies of the candidate genes TGFB2, MSX1, TGFA, and TGFB3 in the etiology of cleft lip and palate in the Philippines. Cleft Palate- Craniofacial J 1997;34:1-6. 26. Loeys BL, Chen J, Neptune ER, Judge DP, Podowski M, Holm T, Meyers J, Leitch CC, Katsanis N, Sharifi N, Xu FL, Myers LA, Spevak PJ, Cameron DE, De Backer J, Hellemans J, Chen Y, Davis EC, Webb CL, Kress W, Coucke P, Rifkin DB, De Paepe AM, Dietz HC. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet. 2005;37:275-81. 27. Van Den Boogaard MJ, Dorland M, Beemer FA, van Amstel HK. MSX1 mutation is associated with oro-facial clefting and tooth agenesis in humans. Nat Genet 2000;24:342- 343. 28. Vieira AR, Cooper ME, Marazita ML, Orioli IM, Castilla EE. Interferon regulatory factor 6 (IRF6) is associated with oral-facial cleft in individuals that originate in South America. Am J Med Genet A2007;143A:2075-2078. 29. Pan Y, Ma J, Zhang W, Du Y, Niu Y, Wang M, Zhang Z, Wang L. IRF6 polymorphisms are associated with nonsyndromic oro-facial clefts in a Chinese Han population. Am J Med Genet A. 2010;152A:2505-11.

383 10th World Cleft Lip, Palate & Craniofacial Congress 30. Sözen MA, Hecht JT, Spritz RA. Mutation and association analysis of the PVR and PVRL2 genes in patients with non-syndromic cleft lip and palate. Genet Mol Biol. 2009;32:466-9. 31. Sözen MA, Hecht JT, Spritz RA. Mutation analysis of the PVRL1 gene in caucasians with nonsyndromic cleft lip/palate. Genet Test Mol Biomarkers. 2009;13:617-21. 32. Kaewkhampa A, Jotikasthira D, Malaivijitnond S, Kantaputra P. TBX22 Mutation Associated With Cleft Lip/Palate, Hypodontia, and Limb Anomaly. Cleft Palate Craniofac J. 2012;49:240-4. 33. Wang H, Zhang T, Wu T, Hetmanski JB, Ruczinski I, Schwender H, Liang KY, Murray T, Fallin MD, Redett RJ, Raymond GV, Jin SC, Chou YH, Chen PK, Yeow V, Chong SS, Cheah FS, Jee SH, Jabs EW, Scott AF, Beaty TH. The FGF and FGFR Gene Family and Risk of Cleft Lip With or Without Cleft Palate. Cleft Palate Craniofac J. 2013;50:96-103. 34. Beaty TH, Murray JC, Marazita ML, Munger RG, Ruczinski I, Hetmanski JB, Liang KY, Wu T, Murray T, Fallin MD, Redett RA, Raymond G, Schwender H, Jin SC, Cooper ME, Dunnwald M, Mansilla MA, Leslie E, Bullard S, Lidral AC, Moreno LM, Menezes R, Vieira AR, Petrin A, Wilcox AJ, Lie RT, Jabs EW, Wu-Chou YH, Chen PK, Wang H, Ye X, Huang S, Yeow V, Chong SS, Jee SH, Shi B, Christensen K, Melbye M, Doheny KF, Pugh EW, Ling H, Castilla EE, Czeizel AE, Ma L, Field LL, Brody L, Pangilinan F, Mills JL, Molloy AM, Kirke PN, Scott JM, Arcos-Burgos M, Scott AF. A genome- wide association study of cleft lip with and without cleft palate identifies risk variants near MAFB and ABCA4. Nat Genet. 2010;42:525-9. 35. Yuan Q, Blanton SH, Hecht JT. Association of ABCA4 and MAFB with non-syndromic cleft lip with or without cleft palate. Am J Med Genet A. 2011 ;155A:1469-71. 36. Birnbaum S, Ludwig KU, Reutter H, Herms S, Steffens M, Rubini M, Baluardo C, Ferrian M, Almeida de Assis N, Alblas MA, Barth S, Freudenberg J, Lauster C, Schmidt G, Scheer M, Braumann B, Bergé SJ, Reich RH, Schiefke F, Hemprich A, Pötzsch S, Steegers-Theunissen RP, Pötzsch B, Moebus S, Horsthemke B, Kramer FJ, Wienker TF, Mossey PA, Propping P, Cichon S, Hoffmann P, Knapp M, Nöthen MM, Mangold E. Key susceptibility locus for nonsyndromic cleft lip with or without cleft palate on chromosome 8q24. Nat Genet. 2009 Apr;41(4):473-7. 37. Leslie EJ, Mansilla MA, Biggs LC, Schuette K, Bullard S, Cooper M, Dunnwald M, Lidral AC, Marazita ML, Beaty TH, Murray JC. Expression and mutation analyses implicate ARHGAP29 as the etiologic gene for the cleft lip with or without cleft palate locus identified by genome-wide association on chromosome 1p22. Birth Defects Res A Clin Mol Teratol. 2012 Nov;94(11):934-42.

384 10th World Cleft Lip, Palate & Craniofacial Congress 38. Indian Genome Variation Consortium. Genetic landscape of the people of India: a canvas for disease gene exploration. J Genet 2008;87:3-20. 39. Huang N, Lee I, Marcotte EM, Hurles ME. Characterising and predicting haploinsufficiency in the human genome. PLoS Genet. 2010 Oct 14;6(10):e1001154. 40. Philipp-Staheli J, Payne SR, Kemp CJ. p27(Kip1): regulation and function of a haploinsufficient tumor suppressor and its misregulation in cancer. Exp Cell Res. 2001 Mar 10;264(1):148-68. 41. Kumar MS, Pester RE, Chen CY, Lane K, Chin C, Lu J, Kirsch DG, Golub TR, Jacks T. Dicer1 functions as a haploinsufficient tumor suppressor. Genes Dev. 2009 Dec 1;23(23):2700-4. 42. Ganji H, Nouri N, Salehi M, Aryani O, Houshmand M, Basiri K, Fazel-Najafabadi E, Sedghi M. Detection of Intragenic SMN1 Mutations in Spinal Muscular Atrophy Patients With a Single Copy of SMN1. J Child Neurol. 2014 Feb 20. 43. Lamandé SR, Bateman JF, Hutchison W, McKinlay Gardner RJ, Bower SP, Byrne E, Dahl HH. Reduced collagen VI causes Bethlem myopathy: a heterozygous COL6A1 nonsense mutation results in mRNA decay and functional haploinsufficiency. Hum Mol Genet. 1998 Jun;7(6):981-9. 44. Szigeti R, Pangas SA, Nagy-Szakal D, Dowd SE, Shulman RJ, Olive AP, Popek EJ, Finegold MJ, Kellermayer R. SMAD4 haploinsufficiency associates with augmented colonic inflammation in select humans and mice. Ann Clin Lab Sci. 2012 Fall;42(4):401-8. 45. Martínez-Fernández ML, Bermejo-Sánchez E, Fernández B, MacDonald A, Fernández- Toral J, Martínez-Frías ML. Haploinsufficiency of BMP4 gene may be the underlying cause of Frías syndrome. Am J Med Genet A. 2014 Feb;164A(2):338-45. 46. Lu W, Quintero-Rivera F, Fan Y, Alkuraya FS, Donovan DJ, Xi Q, Turbe-Doan A, Li QG, Campbell CG, Shanske AL, Sherr EH, Ahmad A, Peters R, Rilliet B, Parvex P, Bassuk AG, Harris DJ, Ferguson H, Kelly C, Walsh CA, Gronostajski RM, Devriendt K, Higgins A, Ligon AH, Quade BJ, Morton CC, Gusella JF, Maas RL. NFIA haploinsufficiency is associated with a CNS malformation syndrome and urinary tract defects. PLoS Genet. 2007 May 25;3(5):e80.

385 10th World Cleft Lip, Palate & Craniofacial Congress

Genetics in clefting

Jiji Pamila Rossi II nd year MDS, Dayananda Sagar College of Dental Sciences, Bangalore, Karnataka, India

ABSTRACT :

Cleft lip and cleft palate, also known as orofacial cleft, is a group of conditions that includes cleft lip (CL), cleft palate (CP), and both together (CLP). They are a type of birth defect of which the cause is unknown in most cases. Risk factors include smoking during pregnancy, diabetes, obesity, an older mother, and certain medications. The development of the face is coordinated by complex morphogenetic events and rapid proliferative expansion, and is thus highly susceptible to environmental and genetic factors, rationalising the high incidence of facial malformations. Genetic factors contributing to cleft lip and cleft palate formation have been identified for some syndromic cases, but knowledge about genetic factors that contribute to the more common isolated cases of cleft lip/palate is still patchy. Many clefts run in families, even though in some cases there does not seem to be an identifiable syndrome present possibly because of the current incomplete genetic understanding of midfacial development. This presentation aims to throw light into the deep phantoms of genetics in clefting.

386 10th World Cleft Lip, Palate & Craniofacial Congress

Team Organization and Standard of Cleft Care in Nigeria: Charting a way forward for provision of comprehensive cleft care

Rafel Adetokunbo Adebola,1 Joshua Biodun Adeoye,2 Akinwale Adeyemi Efunkoya 1 1 Oral and Maxillofacial Surgery Department, 2 Preventive Dentistry Department, Bayero University, Kano, Nigeria

ABSTRACT Introduction:

Every year, there are about 220,000 new Cleft lip and palate cases worldwide, and the social and healthcare costs borne by the affected and their families are considerable. Organized teams that provide integrated care with a focus on overall health - not just repair of the cosmetic deformity - provide the best care. The America Cleft Palate-Craniofacial Association (ACPA) guidelines on team organization and standards of care offer comprehensive overview of the organization, function, and composition of the ideal Cleft lip and palate team (CPT) or craniofacial team (CT). We surveyed Nigerian cleft-care providers against this backdrop, examined team organization and standards of care nationwide,Methodology: and charts a course towards concerted improvement of cleft care.

Cleft care organizations in Nigeria were compiled from the Nigerian Association for Cleft Lip and Palate (NACLP) and the Treatment partners’ list of the Smile Train organization, the major sponsor of cleft care provision in Nigeria. Cleft teams were contrasted with ACPA criteria using information providedResults: by the surveyed organizations via a mailed questionnaire.

Fifteen (15) cleft care centers were identified, 13 (86.7%) of which had organized teams. None met all the ACPA basic CPT criteria, and only 3 (20%) met 10 or more of the 35 additional criteria. None met the 45 criteria to be considered a CT also. 387 10th World Cleft Lip, Palate & Craniofacial Congress

The major challenge for team organization was in manpower, management of disabilities other than cosmesis, and support and counseling. Existential challenges including widespread poverty and incessant industrial action were theConclusion: greatest challenges to cleft care provision countrywide.

Reorganization of cleft care is necessary to change the focus from large-volume surgical cleft repairs to high-quality, comprehensive, cleft care. this.Regional centers serving the socio-political regions of the country, supported by concerted capacity development and database management can help achieve

388 10th World Cleft Lip, Palate & Craniofacial Congress

Naso alveolar moulding : after surgery???

Dr. Ramasamy M Professor, dept of Orthodontics, Sri venkateswara Dental college and hospital, Thalambur, Chennai.

Abstract

The use of naso alveolar moulding for correcting the collapsed nasal cartilages before the corrective surgery have been practiced since the time of its conception by Grayson in 1993. The maintenance of the cartilage shape and integrity after surgery especially in the initial active periods of growth is equally important. The use of conformers is very rarely practiced nowadays. This paper stresses the importance of the use of conformers post surgically. The materials used for the construction of conformers, the timing of the use of conformersIntroduction will be discussed.

Presurgical infant orthopedics has been employed since 1950 as an adjunctive neonatal therapy for the correction of cleft lip and palate. Most of these therapies did not address deformity of the nasal cartilage in unilateral and bilateral cleft lip and palate as well as the deficiency of the columella tissue in infants with bilateral cleft. The nasolaveolar molding (NAM) technique a new approach to presurgical infant orthopedics developed by Grayson reduces the severity of the initial cleft alveolar and nasal deformity. This enables the surgeon and the patient to enjoy the benefits associated with repair of a cleft deformity that is minimal in severity. But in majority of these cases the nasal stent is discontinued post operatively. This paper discusses the importance of maintainingLiterature review the nasal and shape discussion with nasal conformers post operatively.

Grayson et al1, 2 proposed the presurgical nasal molding as an adjunctive neonatal management for preoperative correction of nasal deformities by utilizing the malleability of alar cartilage shortly after birth. The goals of presurgical nasoalveolar molding are to align the alveolar segments, approximate the alveolar cleft, mold the deformed nasal cartilage, and lengthen389 10th World Cleft Lip, Palate & Craniofacial Congress the deficient columella. Post surgical moulding was first used by Osada5 and Skoog in 1969.6 Freide7 used an acrylic conformer and noted improvement in nasal contour. Matsuo8 used the presurgical nasal stent as the conformer for 3 months full time and for 12 months at night time post surgically. The manipulatedidea behind the at theuse early of nasal stages conformer of growth. is to prevent the nasal contracture and stenosis post operatively. The cartilage is considered to be plastic enough to be

In a study by Eric Jein-Wein Liou et al it was found that the nasal asymmetry significantly relapsed in the first year after corrective surgery and then remained stable in the second and third years after surgery. The nasal asymmetry was acceptable at 3 years postoperatively. The relapse in nasal asymmetry was the result of a significant differential growth/relapse between the cleft and noncleft sides in the first year postoperatively. On the cleft side, the growth of nostril height and nasal dome height was significantly less, the columella length shortened (relapsed) significantly, and the growth of nostril width and nasal basal width was significantly less than on the noncleft side. The relapse stopped and the nasal growth was the same between the cleft and noncleft sides in the second and third years. To compensate for the relapse resulting from the differential growth in the first year postoperatively, they recommended narrowing down the alveolar cleft as well as possible by nasoalveolar molding, overcorrecting the nasal vertical dimension surgically, and maintaining the surgical results by using a nasal conformer. The use of conformer helps enabling the vertical growth by increasing the height of conformers.Material used

Initially acrylic conformers were used but now the use of silicone conformers is prevalent. Silicones are polymers that include any inert, synthetic compound made up of repeating units of siloxane, which is a chain of alternating silicon atoms and oxygen atoms, frequently combined with carbon and/or hydrogen. They are typically heat-resistant and rubber-like in nature. Acrylic material is hard and not rubber like. It is essential for the conformer to simulate the part which it mimicks or manipulates. Hence it is more appropiriate to use a silicone conformer rather than an acrylic one. The heat resistance of silicone over acrylic also adds to the advantage. The patency of airway is maintained by Fig 1: nostril conformer Adjustment of nasal conformer: placing breathing holes in the conformer (fig 1).

The conformers need periodic adjustments for increasing the height of the390 nostril on the cleft side. The adjustments are done by adding silicone sheets 10th World Cleft Lip, Palate & Craniofacial Congress

of one mm thickness on the top of the dome on cleft side every 2-4 weeks. Nasal Fig 2: Different size of the conformers. conformers are also available in various preformed sizes (fig 2). Conclusion:

The use of conformers helps in maintaining the corrected nasal structures postoperatively. The use of conformers should be encouraged at least for a year postoperatively.References: 01. Grayson, B. H., Santiago, P. E., Brecht, L. L., and Cutting,C. B. Presurgical nasoalveolar molding in infants with cleft lip and palate. Cleft Palate Craniofac. J. 36: 486, 1999. 02. Grayson, B. H., Cutting, C. B., and Wood, R. Preoperative columella lengthening in bilateral cleft lip and palate. Plast. Reconstr. Surg. 92: 1422, 1993. 03. The Progressive Changes of Nasal Symmetry and Growth after Nasoalveolar Molding: A Three-Year Follow-Up Study Eric Jein-Wein Liou, Murukesan Subramanian, Phil K. T. Chen, and C. Shing Huang, plastic and reconstructive surgery, september 15, 2004. 04. Osada M,Hashimoto K, Akiyama T. Apllication of intra and extra nasal silicone prosthesis after operation of nasal deformities. Jpn J plast reconstr. Surg 1969;11:191. 05. Skoog T. Repair of unilateral cleft lip deformity : maxilla,nose and lip. Scand J plast reconstr surg. 1969;3:109-133. 06. Friede H,Lilja J, Johanson B. Lip nose morphology and symmetry in unilateral cleft lip and palate patients following a two stage lip closure. Scand J plast reconstr surg 1980;14:55-64. 07. Matsuo K,Horose T. Non surgical correction of cleft lip nasal deformity in the early neonate. Ann Acad Med Singapore 1988; 17:358-365.

391 10th World Cleft Lip, Palate & Craniofacial Congress

Distractor - A magic wand for cleft patients

S.KANMANI PREETHI 2nd Year PG, RMDC&H, Annamalai University, Chidambaram, India

INTRODUCTION

Cleft lip and palate patients usually present with a collapsed maxillary dental arch and impaired forward and downward growth of the maxilla [2–4]. Two factors have been proposed for the growth deficiency [2]: One such factor is the intrinsic factor,mainly introduced by developmental deficiency leading to the formation of a cleft and the growth potential of midfacial skeleton. The other factor is the iatrogenic factor, including surgical repair. Therefore, management of cleft-related maxillary hypoplasia is more complex due to the larger degree of malocclusion and advancement, the risk of post-surgical relapse and the potential velopharyngealPARADIGM SHIFT incompetence OF SURGRY following ONE STEP maxillary TO DISTRACTION advancement OSTOGENESIS [1, 5].

Patients with cleft lip and palate always have some disadvantages of maxillary growth because of postsurgical scar contraction and anatomical anomalies and generally have some deformity of the maxillary dental arch. The aim of orthodontic treatment for those patients is to solve these deformities and to produce the ideal maxillo-mandibular relationship. Many cases require orthodontic surgical treatments to achieve this. Various surgical orthodontic techniques have been advocated to correct the deformity of maxillary dental arch, but it is clear that the recent introduction of the concept of DO has increasedDISTRACTION the variation OSTEOGENESIS of treatment procedures.

The distraction osteogenesis (DO) is a method developed for induction of new tecidual formation between two segments of a bone for a slow and gradual force of traction (Ilizarov, 1989a; Ilizarov, 1989b; Meyer et al., 2001a) It can be classified in two basic types: the fiseal distraction, that involves 392 10th World Cleft Lip, Palate & Craniofacial Congress

the epiphysis and diaphysis of a long bone separation, and the calotasis, that consists of the gradual distension of a bone callus formed around the line broken or osteotomic stimulating and keeping the regeneration and the growth activity of hard and soft tissues (Mckibbin, 1978; Wangerin & Gropp, 1997). Ilizarov (1989a) described 2 basic types of distraction: callotasis, or distraction of the fracture callus, and physiologic distraction. They are also described, respectively, as distraction epiphysiolysis (fast traction: 1-1.5 themm/day) linear regenerationand chondrodiastasis of tissues. (slow traction: 0.5 mm/day). The concept of callotasis is of interest in implant surgery, especially monofocal callotasis for

When segmental defects are reconstructed, bifocal FERNANDES, F. H. C. N.; ORSI, I. A. & BEZZON, O. L. Distraction osteogenesis in dentistry. Int. J. Morphol., 28(3):743-748, 2010. 745 or trifocal distraction is used. This technique was only spread out when Ilizarov presented his studies in a Symposium occurred inHISTORICAL New York inBACKGROUND the year of 1988 OF (1989a,DISTRACTION 1989b). OSTEOGENESIS

More than 2000 years ago, Hipócrates was credited by describing the mechanics manipulation of the segment bone breaking. In the century XIV, Guy de Chauliac was the first one to study the continuous traction for reduction of breakings. The concept of osteotomies was established in the century XIX when Malgaine described the concept of the external setting in the treatment of patelares composed breakings (McCarthy et al., 2001). Manipulation of the bone segments was first described by Codivilla in 1905 [6]. Gavriel Awramowitch Ilizarov performed several studies to advance timethe technique periods. in orthopaedic therapy in 1950s [7–10]. Ilizarov performed this technique in two steps and observed the bone regeneration between the two

Application of mandibular lengthening in maxillofacial complex with the usage of external fixation device was first performed in a canine animal model by Synder et al. [11] in 1972. In 1977, Michielli and Miotti [12] used internal devices in lengthening of mandible in a canine animal model by gradual distraction. McCarthy and colleagues [13] first used distraction osteogenesis in humanPRESERVATION mandible OFin 1992.VELOPHARYNGEAL FUNCTION

The introduction of distraction osteogenesis (DO) (fig 1) has increased the variation of surgically assisted orthodontic treatments for patients with cleft393 10th World Cleft Lip, Palate & Craniofacial Congress lip and palate, especially for those cases with severe morphological problems of the maxillary bone and maxillary alveolar arch. Among these surgical procedures of DO, maxillary advancement combined with Le Fort osteotomy has already become one of the standard treatments for patients with cleft lip and palate[14], because its benefit of the preservation of velopharyngeal function and fewer limits on the advancement amount compared with simultaneous maxillary advancement. The external and internal usage of distraction osteogenesis in the treatment of maxillary hypoplasia in patients with cleft lip and palate is a reliable, reproducible and stable alternative method to conventional one-step LeFort I advancement techniques and these patients may have velopharyngeal insufficiency, which may complicate surgical repositioning of the maxilla. Several recent papers have discussed segmental anterior maxillary distraction as a way to correct the anterior posterior maxillary discrepancy without affectingINTERNAL the vs velopharyngeal EXTERNAL DISTRACTION: mechanism.[15,16]

However, any procedures of maxillary DO using an internal distractor have limitations of the advancement amount and vector, and those using an external distractor, which allows complicated 3-dimensional movement, an external distraction system cause mental stress for patients because of having to wear a large head-gear type of external distractor for long periods and also having the risk of severe brain injury by the distractor (fig 3). Therefore, an ideal internal distractor would allow some complicated movements of the maxillary bone, including rotational movement. An external distraction system is beneficial in treating patients with cleft lip and palate because it has fewer limitations regarding the amount and direction of jaw advancement.[17] The patient, however, has to wear a relatively large device, which is fixed in the lateral temporal area for a long consolidation period and may turn out to be a serious cause of postoperative complications caused by psychological stress and the potential risk of accidental head injury. InDISTRACTION contrast, the OSTEOGENESISinternal distractor WITH offers INTRAORAL some benefit. DEVICE

Intraoral device cause it causes less psychological stress and demands a shorter hospitalization period[18]fig 2. Moreover, DO with internal distractors does not necessarily require the patient’s cooperation during the retention period, and compared with the ED system, it does not leave scars caused by 394 fixation screws. 10th World Cleft Lip, Palate & Craniofacial Congress

Potential complications of the internal distractor are defective distraction vectors and insufficient distraction; disadvantages include less freedom of choice of direction and restricted jaw advancement. Maxillary DO with internal distractors generally requires bilateral devices. Some reports have emphasized that it is important but difficult to place two devices in parallel, while keeping their positions rigid and controlling their directions.[19] Essentially, the two internal distractors can move the maxilla in a straight line, but they are not designed for advancement that requires some rotational movement. Moreover, it is impossible to adjust the vertical direction of jaw advancement to compensate for the cant of the occlusal plane. On the other hand, it may be possible to perform some rotational\ movement with the flexibility of the device, although as demonstrated, risk of device breakage may resultMAXILLARY from excessive ANTERIOR force deliveredSEGMENTAL to the fragileDISTRACTION points of theOSTEOGENESIS device. (MASDO) T

otal maxillary advancement by DO combined Le Fort osteotomy has been a standard procedure for correction of the position of anterior maxillo- mandibular relationship by maxillary advancement in patients with cleft lip and palate, but the recently reported procedure, the maxillary anterior segmental distraction osteogenesis (MASDO),[20-21] which advances only the anterior portion of maxillary bone, is also an effective treatment for correction. This procedure has some merits for the treatment of small and retruded maxillary bone in cleft patients, such as conservation of velopharyngeal function and molar occlusion, and this procedure may become one of standards of treatment SEGMENTAL DISTRACTION OSTEOGENESIS for retruded maxilla in patients with cleft lip and palate.

Segmental distraction osteogenesis of the anterior process is a powerful technique that can prevent extractions in patients with dental crowding. The technique can also compensate for retruded anterior alveolar process and accelerate or decelerate the curve of Spee. Patients with constricted periodontal health and those with a thin mandibular symphysis, however, cannot be treated with this technique because of the increased risk of dental tipping. Severe gingival recession must also be considered a possible side effect associated withPROSTHETIC this technique[22] MANAGEMENT

Management of partially dentate patients with anterior maxillary defects when there is a full complement of lower teeth is challenging. Restoration395 is 10th World Cleft Lip, Palate & Craniofacial Congress especially difficult when there is lack of upper lip support in a skeletal Class III individual. This clinical situation obligates the patient to a denture flange filling the vestibule to give support to the upper lip. Construction of the prosthesis can be even more of a challenge when there is scarring in the anterior vestibule. Patients who have had repair of a bilateral cleft lip and palate and have lost their anterior teeth frequently have this clinical situation. When a removable prosthesis is present, the fulcrum effect on abutment teeth from the prosthesis is detrimental to the long-term periodontal health of these teeth. Repetitive trauma to the edentulous segment from the cantilevered prosthesis results in the loss of the anterior alveolar ridge. Implants have radically changed the management of edentulous and partially edentulous patients by giving support to remaining alveolar bone and reducing the fulcrum effect on the abutment teeth.[23–24]. The vector of distraction was both vertical and horizontal to correct the patient.anterior posterior deficiency and give more vertical height. This not only allowed an improved implant position, but it restored the anterior vestibule for

Anterior segmental maxillary distraction in a partially edentulous patient canCONCLUSION greatly facilitate : prosthetic management of these individuals[25]

It may be concluded that it has become possible to correct a variety of severely deformed maxillary dental arches using MASDO. After this treatment, the produced distracted gap can have enough bone volume, which can be used for tooth alignment and/or dental implant treatment. treatment.Therefore MASDO may become one of the standards of surgical treatment for small and retruded maxilla, before teeth alignment by orthognathic REFERENCE: [1] Aksu M, Saglam-Aydinatay B, Akcan CA, El H, Taner T, Kocadereli I, Tuncbilek G, Mavili ME Skeletal and dental stability after maxillary distraction with a rigid external device in adult cleft lip and palate patients. J Oral Maxillofac Surg. 2010;68:254-259. [2] Ross RB Treatment variables affecting facial growth in complete unilateral cleft lip and palate. Cleft Palate J. 1987;24:5-77. [3] Cheung LK, Chua HDP A meta-analysis of cleft maxillary osteotomy and distraction. Int J Oral Maxillofac Surg. 2006;35:14-24.

396 10th World Cleft Lip, Palate & Craniofacial Congress [4] Olmez S, Dogan S, Pekedis M, Yildiz H Biomechanical evaluation of sagittal maxillary internal distraction osteogenesis in unilateral cleft lip and palate patient and noncleft patients-a three dimensional finite element analysis. Angle Orthod. 2014;84:815-824. [5] Austin SL, Mattick CR, Waterhouse PJ Distraction osteoegnesis versus orthognathic surgery for the treatment of maxillary hypoplasia in cleft lip and palate patients: a systematic review. Orthod Craniofac Res. 2015;18:96-108. [6] Codivilla A On the means of lengthening in the lower limbs, the muscles and tissues which are shortened through deformity. Clin Orthop Relat Res. 1994;301:4-9. [7] Ilizarov GA Basic principles of transosseous compression and distraction osteosynthesis. Orthop Travmatol Protez. 1971;32:7-15. [8] Ilizarov GA The principles of the Ilizarov method. Bull Hosp Jt Dis Orthop Inst. 1988;48:1- 12. [9] Ilizarov GA The tension-stress effect on the genesis and growth of tissues. Part I. The influence of stability of fixation and soft tissue preservation. Clin Orthop Relat Res. 1989a;238:249-281. [10] Ilizarov GA The tension-stress effect on the genesis and growth of tissues. Part II. The influence of the rate and frequency of distraction. Clin Orthop Relat Res. 1989b; 239:263- 285. [11] SynderCC, Levine GA, Swanson HM, Browne EZ Jr Mandibular lengthening by gradual distraction. Preliminary report. Plas Reconstr Surg. 1973;51(5):506-508. [12] Michielli S, Miotti B Lengthening of mandibular body by gradual surgical-orthodontic distraction. J Oral Surg. 1977;35(3):187-192. [13] McCarthy JG, Schreiber J, Karp N, Thorne CH, Grayson BH Lengthening of the human mandible by gradual distraction. Plas Reconstr Surg. 1992;89(1):1-8. [14] Scolozzi P. Distraction osteogenesis in the management of severe maxillary hypoplasia in cleft lip and palate patients. J Craniofac Surg 2008;19:1199-214. [15] Sa´ndor GK, Ylikontiola LP, Serlo W, Pirttiniemi PM, Carmichael RP. Midfacial distraction osteogenesis. Atlas Oral Maxillofac Surg Clin North Am. 2008;16:249–272. [16] Karakasis D, Hadjipetrou L. Advancement of the anterior maxilla by distraction (case report). J Craniomaxillofac Surg. 2004;32:150–154. [18] Mofid MM, Manson PN, Robertson BC, Tufaro AP, Elias JJ, Vander Kolk CA. Craniofacial distraction osteogenesis: a review of 3278 cases. Plast Reconstr Surg. 2001;108: 1103– 1114.

397 10th World Cleft Lip, Palate & Craniofacial Congress [19] Rachmiel A. Treatment of maxillary cleft palate: distraction osteogenesis versus orthognathic surgery. Part one: Maxillary distraction. J Oral Maxillofac Surg. 2007;65:753–757. [20] Alkan A, Bas¸ B, Ozer M, Bayram M, Yüzbasiog˘lu E. Maxillary anterior segmental advancement of hypoplastic maxilla in cleft patients by distraction osteogenesis: report of 2 cases. J Oral Maxillofac Surg 2008;66:126-32. [21] Dolanmaz D, Karaman AI, Ozyesil AG. Maxillary anterior segmental advancement by using distraction osteogenesis: a case report. Angle Orthod 2003;73:201-5. [22] Wolfgang Zemann, Philipp Metzler, Christine Jacobsen , Joachim Anton Obwegeser, Segmental Distraction Osteogenesis of the Anterior Alveolar Process Using Tooth-Borne Devices: Is It Skeletal Movement or Mainly Dental Tipping? ,American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 70:1292-1299, 2012 [23] Zitzmann NU, Marinell CP. Treatment plan for restoring the edentulous maxilla with implant supported restorations: removable overdenture versus fixed partial denture design. J Prosthet Dent. 1999;82:188–196. [24] Jivraj S, Chee W, Corrado P. Treatment planning of the edentulous maxilla. Br Dent J. 2006;201:261–279. [25] Cleft Palate Patient Joseph E. Van Sickels, DDS1* Behruz Abadi, DMD2 Reed Attisha, DMD1

Anterior Segmental Distraction for a Class III Maxillary Prosthetic Defect .

398 10th World Cleft Lip, Palate & Craniofacial Congress

Longitudinal and cross-sectional model analysis of transverse maxillary growth in cleft patients: comparison of two treatment strategies

Natalie Loomans1 Oona Decombel2 An Verdonck2 Carine Carels2 Joseph Schoenaers 2Maurice Y. Mommaerts3 1GZA Hospitals, Antwerp, Belgium, 2University Hospitals Leuven, Leuven, Belgium, 3Universitair Ziekenhuis Brussel, VUB, Brussels, Belgium

Purpose:

The aim of the retrospective study was to compare differences in maxillary growth, in particular in the transverse dimension following cleft repair using two different treatment protocols in 2 different centers. In cleft center A the soft palate was closed with a push back palatoplasty, in cleft center B a Furlow palatoplasty was performed. In each center, a two-staged palatal closure was opted for. The hard palate was closed at the age of 4 year with mixeda minimal dentition. and epiperiosteal technique at the age of 4 years in groupA and B. Transverse growth was evaluated from 1 month after birth till the end of the Materials and methods:

Two groups of 10 patients with complete clefts of lip, alveolus and palate (CLAP) were matched based on gender, age and palatal morphology at the age of 1 month. Models of the maxilla were measured at 4 corresponding ages. Intra-class correlation coefficient was measured to determine intra-and inter-observer reliability; a Wilcoxon two-sample test for differences in small samplesResults: and the Kruskal-Wallis Test was used.

There was no significant difference at the age of 5 years for the intercanine distance and intermolar distance in the UCLAP groups and the BCLAP groups. In the UCLAP groups there was significant difference in increase in intermolar 399 10th World Cleft Lip, Palate & Craniofacial Congress distance of 8mm (P= 0,0553) at the age of 12 years, not in intercanine distance. In the BCLAP groups there was a significant difference regarding the increase of the intercanine distance of 2.8mm (P=0,1508) and intermolar distance of 7.5mm (P= 0,0317) between the age of 5 years and 12 years. For each age group and distance, it was group B that showed more increase. Conclusion: transversal growth till the age of 5 years old is independent of technique of soft palate closure. The difference in transverse dimension observed between 5 and 12 years are most probably due to earlier interceptive orthodontic treatment with quadhelix devices in group B, and possibly also to the amount of subperiosteal dissection by the 2 different surgeons at the time of hard palate closure.

400 10th World Cleft Lip, Palate & Craniofacial Congress

Cleft Maxillary deficiency addressed with anterior maxillary distraction and dental implants - A case report

K.Sriraman, M.Veerabahu, J. A. Natha Post graduate, OMFS, Ragas Dental College and Hospital

ABSTRACT

Patients with cleft lip and palate are often present with maxillary hypoplasia and may have a compromised dentition. Restoration of anterioranteroposterior maxillary deficient segment maxilla is rehabilitated is done either with dentalthrough implants. orthognathic surgery or by distraction osteogenesis. This paper discusses about the distracted

401 10th World Cleft Lip, Palate & Craniofacial Congress

Effectiveness of intravenous haemocoagulase on haemorrhage control in bi-maxillary orthognathic surgery in cleft lip and palate patients :

Vikram Shetty1, Ganapathy Sriram S2, B. Rajendra Prasad3, Nidarsh Hegde4, Shambhavi Singh5 1Director, Nitte Meenakshi Institute of Craniofacial Surgery, K.S. Hegde Charitable Hospital, Nitte University, Mangalore, Karnataka, India.2Senior Resident, Nitte Meenakshi Inst. of Craniofacial Surgery, K. S. Hegde Medical College and Hospital (A Unit of Nitte University), Deralakatte, Mangalore, Karnataka, 575018, India 3Head of Department and Director P.G. Studies, Department of Oral and Maxillofacial Surgery A.B. Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, Karnataka, India.4Professor,Department of Oral and Maxillofacial Surgery, A.B. Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, Karnataka, India.5Junior Resident, Department of Oral and Maxillofacial Surgery, A.B. Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, Karnataka, India.Email- [email protected],

ABSTRACT INTRODUCTION

Haemocoagulase is a snake venom protein derivative that is known to possess haemostatic activity. It is reported to minimise blood loss in orthopaedic, otorhinolaryngologic, and abdominal surgeries. The purpose of this trial is to study the efficacy of haemocoagulase in haemorrhage control in orthognathicMATERIALS ANDsurgery. METHODS:

This study was approved by the ethics committee of the Nitte University and was conducted from March 2015 to March 2016. Patients with cleft lip and palate deformity without systemic diseases (e.g., blood dyscrasias, endocrine disorders) were enrolled in the study. A total of 46 consecutive patients undergoing bi-maxillary orthognathic surgery were recruited into the study. The patients were segregated into two groups. Group 1 comprised 23 patients in whom the study drug was administered, whereas group 2 comprised 23 patients in whom placebo was administered. To maintain homogeneity and to avoid bias, only those patients requiring bi-maxillary osteotomy were included in the study. Standard Le 402 10th World Cleft Lip, Palate & Craniofacial Congress sagittal split osteotomy with genioplasty for mandible was performed in all of theFort patients. I osteotomy with pterygomaxillary dysjunction for maxilla and bilateral

Depending on patient's diagnosis, the maxilla and mandible were repositioned to an appropriate aesthetic position. Titanium plates and screws were used for stabilization of the jaws. Randomization was computerised, and patients were allotted to either receive the study drug or placebo. The surgical team, anaesthesiologist, and the investigator were blinded to allocation. A person who was independent of the study prepared the test drug/placebo to be administered to the patient. Haemocoagulase (Botropase; Juggat pharmaceuticals, Benga- luru, India), the drug to be tested, was in a concentration of 1 International Units (IU) in a 1-ml aqueous solution containing 0.9% sodium chloride, 0.3% phenol, and water for injection. All the surgical procedures were performed by the same surgical team. All patients underwent surgery with hypotensive anaesthesia. Intraoperative blood loss, operating time, drop in haemoglobin and haematocrit wereRESULTS: the variables analysed in the study.

In this study, haemocoagulase caused an 11% (52 ml) reduction in blood patients. loss in the study group. There was no adverse reaction in any of the CONCLUSION

Based on our findings, a single bolus dose of 1 IU haemocoagulase preoperatively helps with haemorrhage control in cleft lip and palate patients undergoing bi- maxillary orthognathic surgery. None of the patients in this study developed any adverse reaction.

403 10th World Cleft Lip, Palate & Craniofacial Congress

Utility Of Corticocancellous Iliac Crest Bone Graft In Management Of Alveolar Cleft

Jagadish Patil1, Abhay Datarkar2, Prashant Pandilwar3, Pravin Chouhan4 1First year Post Graduate Student, 2Professor and Head, 3Associate Professor, 4Third year Post Graduate Student, Department of Oral and Maxillofacial Surgery, Govt Dental College and Hospital, Nagpur, India

Introduction

75 per cent of cleft lip and palate affected1,2 population are accompanied by the osseous defects of alveolar bone. Bone grafting procedures for correction of these cleft alveolus has become3,4 an integral part in the sequence of management of cleft lip and palate. The objective of this procedure is to provide a predictable and adequate amount of bone for tooth eruption, stability tissueof dentition, of alar orthodonticbases. tooth movement, closure of oro-nasal fistulae and endosteal implant placement.5,6 Not only this, it also provides support foundto the thatsoft Reviews of the literature on alveolar7 clefts l5grafting has been given by Koberg (1973) and Wittsenberg(1985). BOYNE et a secondary autogenous bone graft creates a continuous regenerated area of viable new bone, and thus improves alveolar and facial contours. preferredDespite treatment of the options numerous regarding researches, the ideal alveolar graft, bonethe appropriate grafting has age been for one of the most controversial procedures. There are multiple philosophies and 8 grafting and other adjunctive orthodontic procedures.

At present, alveolar cleft osteoplasty using autologous6 bone grafts is symphysisthe standard or treatmenttibia. for the management of alveolar clefts. At present the source of autogenous bone grafts are from iliac crest, calvarium, mandibular

Many studies suggest that the graft from the iliac crest is the gold standard for the alveolar cleft grafting. It provides adequate amount of cancellous bone with pluripotent9 cells for alveolar osteogenesis. It is also easy to get access to the iliac crest. We have repaired the cleft alveolus by grafting the iliac cortico-cancellous bone done for various reasons and at different stages of dental development including the repair of oro-nasal fistulae. The objective of this study is to evaluate the versatility of iliac cortico-cancellous bone graft in alveolar10 cleft. The uptake of404 graft was evaluated radiographically by Kendalan index. 10th World Cleft Lip, Palate & Craniofacial Congress Materials and methods 1.1 Patient

52 patients were included in this study from 2003 to 2016, having cleft lip and palate was operated for same and alveolar grafting was done by iliac cortico-cancellous bone. The mean age was 9 years (3 -21 years). Out of 52 patients, 21 were males and 31 females. 41 patients were having unilateral cleft and remaining 11 were bilateral clefts. The iliac bone grafting was done at different stages for various reasons along with other cleft lip palate procedures like lipThe repair, reasons palatoplasty, for grafting septoplasty,etc. were to allow for eruption of permanent lateral and canine eruption, placement of endosteal implant, correction of oro-nasal fistulae, support for alar base correction and stability of maxillary dentition in bilateral cleft cases. In 5 patients, grafting was done for alar base support and nasalTable reconstruction1. Various reasons and in for five performing for oro-nasal ABG fistula correction.

REASONS FOR ABG NO OF PATIENTS INCLUDED PRIMARY BONE GRAFTING 5 PATIENTS AT THE TIME OF ERUPTION OF 8 PATIENTS LATERAL INCISORS AT THE TIME OF ERUPTION OF 18 PATIENTS CANINE FISTULA IN ALVEOLUS 5 PATIENTS NASAL FLOOR RECONSTRUCTION 5 PATIENTS AND ALAR BASE SUPPORT STABILISATION OF PREMAXILLA IN 5 PATIENTS BILATERAL CLEFT FOR PLACEMENT OF ENDOSSEOUS 6 PATIENTS 1.2 Timing of theIMPLANTS Graft

52 patients were enrolled and randomly grouped based on the stages of repair. Group A in whom grafting was done as primary repair. Secondary grafting as Group B and late secondary as Group C. Out of 52 patients, primary grafting with iliac bone was done in 5(9.60%) patients. Secondary grafting in 31(59.60%) and late secondary in 16(30.80%) 405 patients. In secondary grafting, the procedure was performed in 8 (15.38%) 10th World Cleft Lip, Palate & Craniofacial Congress patients showing the eruption of lateral incisor and 18(34.61%) patients were duringTable 2. the Alveolar eruption bone of canine grafting eruption. done at different stages of repair

Age group Group 1 (primary Group 2 Group 3 (late grafting) (secondary ) secondary) patients Number of 5 31 16

In 6(11.53%) patients, the grafting was done for placement of endosteal implant as a late secondary procedure. In 5(9.61%) patients grafting was done as1.3 stabilisation Cleft alveolus of premaxillasurgery in bilateral clefts.

All the surgical procedures were performed by a single surgeon. The procedure was done under general anaesthesia and pre-operative antibiotics. After induction, the cleft area was infiltrated with 2 % lignocaine and 1:2,00,000 adrenaline for local haemostasis. The incision was given at buccal gingiva along the margins of cleft alveolus and extending around at least single tooth on both sides of alveolar cleft. Vertical releasing incision was given mesially and distally. Buccal and palatal mucoperiosteal flap was elevated from the cleft. The cleft alveolus was exposed and the margins of bone around the roots of teeth were carefully preserved.

Fig.1. Exposure of cleft alveolus

performed.If oro-nasal fistula was present, the nasal layer was elevated and properly reconstructed. Adjuvant procedures like septoplasty, palatoplasty were 1.4 Iliac crest bone graft harvesting

After the cleft recipient site was exposed and prepared, the harvesting procedure was performed. Separate sets of surgical instruments were used for406 iliac harvesting and contamination between the two surgical sites was 10th World Cleft Lip, Palate & Craniofacial Congress

avoided. After local infiltration with lignocaine and adrenaline, skin incised over the anterior iliac crest. Incising the subcutaneous and periosteum layer, the anterior iliac crestal rim exposed.

Fig.2. Exposure of iliac crest and harvesting of iliac bone

The cortico-cancellous bone was separated with osteotomy saw. The underlying1.5 Grafting cancellous of the alveolus bone was scooped out and prepared for harvesting.

The iliac cortical bone was cut as chunk and fitted into the cleft alveolus ofsuch bone that resorption. it fits into the defect. Remaining spaces in the cleft is filled with the scooped cancellous bone. The defect is mildly overfilled to expect some amount

Fig.3. Placement of graft in alvolar cleft If nasal and

alar base support is to be corrected, the harvested bone is filled over the pyriform region. The buccal and the palatal mucosa is repositioned to approximate each other over the grafted cleft alveolus. If tension is perceived in the flap, the periosteum is stripped and scored under the buccal flap. Suturing is done with 5-0 vicryl (polyglactin) suture. Post operatively injectable antibiotics are continued for 5 days and appropriate oral mouth wash and hygiene prescribed. Patient is made ambulatory on the second day or when the patient is comfortable. 407 10th World Cleft Lip, Palate & Craniofacial Congress 1.6 Evaluation of alveolar cleft bone grafting

Orthopentomograph was taken in all patients preoperatively and post operatively and at annual basis. The post-operative radiopacity in the alveolus is evaluated with the Kendalan index. The post-operative complication rate and theTable status 4. Kindelan of canine J eruptionD Radiographic were evaluated. assessment of alveolar bone grafting in Cleft lip and palate patients . Cleft Craniofacial Journal 1997,34,195-98

SCORE 1 MORE THAN 75% VOLUME OF BONE UPTAKE ON RADIOGRAPH SCORE 2 50% TO 75% VOLUME OF BONE ON RADIOGRAPH SCORE 3 LESS THAN 50% VOLUME OF BONE ON RADIOGRAPH SCORE 4 NO BONE BRIDGE FORMATION ON 1.7 Statistical evaluation RADIOGRAPH

The results are evaluated by student t-test to assess any statistically significant difference. Results and Observations

Post-operative the outcome of grafting was checked by clinical eruption of canine, adequate support of alar base, closure of oro-nasal fistula and radio- graphicallyTiming of Repair bridging of alveolar bone.

During primary repair (Group A), the uptake was good in 4 (80%) out of 5 patients. The remaining one (20%) showed dehiscence of flap and exposure of graft with subsequent resorption. In Group B (n = 31), grafting was done as secondary measure. All (100%) the patient showed the eruption of canine in the grafted bone with dental development. One patient (3.22%) in Group B showed resorption of bone. In Group C, grafting done as late secondary for various reasons, 3(60%) out of 5 patients showed significant closure of oro- nasal fistula.

408 10th World Cleft Lip, Palate & Craniofacial Congress

Fig.4. Operated oro-nasal fistula by iliac crest graft

One (20%) patient showed resorption of bone. Dental implants were placedTable 4. in Kindelan 6 patients Volumetric having adequate Assessment stability. Score

Score 1 Score 2 Score 3 Score4 (Highly Successful) (Successful (Failure) (Failure) 75% to bridging seen on More than 75% of Less than 50% No bone 50% of radiograph radiographic uptake radiographic radiographic uptake uptake)

38 PATIENTS (73%) 10 PATIENTS 2 P A T I E N T S 2 PATIENTS (4%) (19%) (4%)

• Score 1 & 2 =92% Successful uptake radio graphically

• Fig.5 Before Grafting

409 10th World Cleft Lip, Palate & Craniofacial Congress

ComplicationsFig.6 After bone grafting

Infection in grafted site was seen in 4 patients. Bone resorption without infection in 2 patients. Wound dehiscence occurred in 3 patients. The gait of theDiscussion patient was impaired (limpic gait) seen in one patient post operatively.

An autogeneous bone from iliac crest is considered as a gold standard for the cleft alveolar reconstruction. Anterior iliac crest provides good amount of cortico-cancellous bone for reconstructing the alveolar defect. It provides enough amounts11 of osteo-progenitor cells for the synthesis of new bone in alveolar cleft. Advantages include easy surgical access, adequate amount, low donor site morbidity rate. Two different teams of surgeons can separately work for procedure, one for cleft site preparation and one for harvesting the iliac bone, thus reducing the time between the grafting procedures. However everything comes with limitations. The reflection of muco- prolongedperiosteal hospitalistion. flap for exposure of iliac may produce significant morbidities including hematoma, pain,12,13 discomfort while walking, delayed ambulation and 410 10th World Cleft Lip, Palate & Craniofacial Congress

In our studies the overall success rate was 92 % (score 1 and 2). This is in consistent with other studies. Boyen & Sands 1972 reported 88% success rate5 of Alveolar Bone Grafting when done in 8-12 years of age for different reasons. Mecarde and Vig 2009 reported 92% success rate when Alveolar Bone Grafting done at the time of canine eruption. The success rate was seen in about 80 % of cases (4 out of 5 patients) done at primary cleft repair and 93.3 % success rate during the secondary repair. In a study by Brattstrom, there was an increased incidence of malformation of permanent lateral incisors in the primary graft group and decreased success of graft with only 41% of primary graft (54 % if pregrafting orthopedics was included)16 resulting in adequate bone height when measured with Bergland scale. In our study, the canine erupted acceptably well in the grafted bone. Some authors have indicated that in 30% to 73% of patients, eruption of the canine study.into the alveolar graft requires surgical uncovering of the tooth or uncovering and orthodontic intervention. The overall complication rate is 19.23 % in our Conclusion

The iliac crest cortico–cancellous bone is best option with relative less complication. The high bone volume provides excellent condition for long term outcome. In all our patients bone height reached Kendalan’s index 1 and 2 score. ABG provides the sound platform for the placement of the Osseo integrated dental implants. Hence iliac bone is a standard and a versatile bone graftReferences for alveolar cleft correction at various stages. 01. Waite PD, Waite DE: Bone grafting for the alveolar cleft defect. Semin Orthod 2: 192e196, 1996 02. Guo J, Li C, Zhang Q, Wu G, Deacon SA, Chen J, et al: Secondary bone grafting for alveolar cleft in children with cleft lip or cleft lip and palate. Cochrane Database Syst Rev: CD008050, 2011 03. Eppley BL, Sadove AM: Management of alveolar cleft bone grafting—State of the art. Cleft Palate Craniofac J 37:229, 2000 04. Daw JL, Patel PR: Management of alveolar clefts. Clin Plast Surg 31:303, 2004 05. Boyne PJ, Sands NR. Secondary bone grafting of residual alveolar and palatal clefts. J Oral Surg 1972: 30: 87–92.

411 10th World Cleft Lip, Palate & Craniofacial Congress 06. Rychlik D, Wojcicki P: Bone graft healing in alveolar osteoplasty in patients with unilateral lip, alveolar process, and palate clefts. J Craniofac Surg 23: 118e123, 2012 07. Wilfred AB, Kihi LW. M. Heidbuchel, Hans PM. Freihofe, Anne MK. Early Secondary Bone Grafting of Alveolar Cleft Defects Early Secondary Bone Grafting of Alveolar Cleft Defects J. Cranio-Max.-Fac. Surg. 18 (1990) 08. Horswell, B.B., Henderson, J.M., 2003. Secondary osteoplasty of the alveolar cleft defect. J. Oral Maxillofac. Surg. 61 (9), 1082–1090. 09. Ma’amon AR, Hani T. Secondary Alveolar Bone Grafting: the Dilemma of Donor Site Selection and Morbidity. Br J Oral and Maxillofacial Surgery 46 (2008) 665–670 10. Kindelan J D Radiographic assessment of alveolar bone grafting in Cleft lip and palate patients . Cleft Craniofacial Journal 1997,34,195-98 11. Sameh A. Seifeldin. Is alveolar cleft reconstruction still controversial? (Review of literature). The Saudi Dental Journal (2016) 28, 3–11 12. Baqain, Z.H., Anabtawi, M., Karaky, A.A., Malkawi, Z., 2009.Morbidity from anterior iliac crest bone harvesting for secondary alveolar bone grafting: an outcome assessment study. J. Oral Maxillofac. Surg. 67 (3), 570–575. 13. Rawashdeh, M.A., Telfah, H., 2008. Secondary alveolar bone grafting: the dilemma of donor site selection and morbidity. Br. J. Oral Maxillofac. Surg. 46 (8), 665–670. 14. Kwon JK, Waite DE, Stickel FR, Chisholm T. The management of alveolar cleft defects. J Am Dent Assoc 1981;102:848-853. 15. Eldeeb M, Messer LB, Lehnert MW, et al. canine eruption into grafted bone in maxillary alveolar cleft defects. Cleft palate J 1988;19:9-16. 16. Brattstrom V, McWilliam J. The influence of bone grafting age on dental abnormalities and alveolar bone height in patient with unilateral cleft lip and palate. Eur J Orhod 1989;11:351- 358.

412 10th World Cleft Lip, Palate & Craniofacial Congress

Efficacy of alveolar bone grating with autologus PRP and without PRP in cleft alveolus patients- A Comparative Study

Yadav Abhilasha Wardha, Maharashtra, India

Aim

The aim of this longitudinal study was to evaluate the efficacy of alveolar bone grafting with autologus platelet rich plasma (PRP) over cancellous bone graftingMaterial alone and method- in cleft alveolus patients.

Total thirty patients were evaluated, out of which fifteen patients were subjected to alveolar bone grafting with cancellous bone (control group) and 15 patients underwent alveolar bone grafting with autologus PRP along with cancellous bone graft. Autologus PRP was extracted from Plasma Centrifuge machine. Chi Square test was used for statistical analysis, which revealed statisticallyResults- significance (P<0.05) in study group and control group.

Evaluated on the amount of graft resorption at the end of six months post- operatively was done with the help of radiographs. Assessment of the success ofConclusion- graft was made with the help of Bergland’s Scoring Criteria. results obtained from the study, suggested that resorption was comparatively less in study group as compared to control group.

413 10th World Cleft Lip, Palate & Craniofacial Congress

An Evaluation of autogenous bone grafting with PRF in alveolar cleft patients

Somuri Anand Vijay1, V.K.V Naidu2, L. Krishna Prasad3, P. Srinivas Chakravarthy4, M. Sridhar5 1Reader, Sibar institute of dental sciences2Sibar institute of dental sciences3Principal, Sibar institute of dental sciences4Professor, Sibar institute of dental sciences5Professor, Sibar institute of dental sciences Department(s) and institution(s) Department of Oral & Maxillofacial Surgery, Sibar Institute Of Dental Sciences, Takkellapadu, Guntur Somuri Anand Vijay Address – Sibar Institute Of Dental Sciences, Takkellapadu, Guntur, Phone numbers

ABSTRACT

Introduction and Aim: The aim of this study is to evaluate the outcome of autogenous iliac bone grafting along with Platelet rich fibrin (PRF) placement inMaterials patients and with Methods: cleft alveolus.

The study consisted of 10 patients who were surgically treated for the closer of cleft alveolus to improve aesthetics and speech by secondary alveolar bone grafting. Preoperatively the marginal bone level was assessed radiographically on IOPA by using Bergland grading scale and radiographic measureddensity was at measured one month on and direct three digital months. radiographs by using DIGORA Software. Postoperatively marginal bone level and radiographic density were again Results:

Among ten cases Marginal bone level was improved in 8 cases from type- IV to type- I and II, in one case type-IV to type-III and in one case graft was exposed. In 9 cases radiographic density was significantly improved when patient.compare with pre-operative and post-operative one and three month interval. One case failed due to poor oral hygiene and lack of post-operative care by Conclusion:

In conclusion that fresh autogenous cancellous bone graft along with 414 PRF is a good graft material for SABG to reconstruct the cleft alveolus and it 10th World Cleft Lip, Palate & Craniofacial Congress

was more helpful for osteogenesis. Treatment of the alveolar cleft should not be an afterthought in the care of cleft lip and palate patients. It has functional and aesthetic significance and reconstructive potentially carries a significant benefit to the patient. It is also felt that a more extensive study involving a larger sample and longer follow up should be undertaken to confirm its advantages andKey disadvantageswords: of autogenous iliac bone grafting in cleft alveolus patients.

Secondary alveolar bone grafting; Autogenous cancellous graft placement inINTRODUCTION cleft alveolus; Radiographic density; Cleft Alveolus

Initial attempts for the treatment of alveolar cleft dates back to 1901, when Von Eiselberg used pedicled bone to fill alveolar defect, Since that time, the complex management of cleft alveolus has advanced tremendously.1 After closure of the hard and soft palate the residual cleft does not usually cause any severe problems initially but as the patients matures the maxilla grows and the related problems like alveolar clefts deepening become more noticeable and creates future functional, surgical, orthodontic and prosthodontics problems. Although a number of autogenous and allogeneic materials are used in maxillofacial reconstruction, autogenous grafts are preferred in alveolar clefts. marrowThough thereof ilium. are varieties of autogenous graft materials like iliac crest bone, tibia, rib, mandibular symphysis, calvarium etc., the preferred one is cancellous

Timing of bone graft placement is an important issue in alveolar cleft management. When graft material is placed at an early age, before 2.5 years, it is called primary. Early secondary is placement of graft between 2-5 years, dentition.intermediate The secondary preferred between 6-13 years and late secondary after the age of 13 years. Alveolar cleft requires bony repair to allow proper eruption of time for grafting is at the age of 9-12 years when about 2/3 of the root of permanent canine has formed and the crown is not clinically visible.2,3 The present study attempts to evaluate autogenous bone grafting along with Platelet rich fibrin (PRF) placement in alveolar cleft patients and the objectives are to observe the changes in marginal bone level and radiographic densityMATERIALS to assess & METHODS: graft uptake.

This study consisted of 10 patients who had alveolar clefts with or without oro-nasal fistula. Ethical clearance was obtained by the ethical committee415 10th World Cleft Lip, Palate & Craniofacial Congress before the commencement of the study. ASA Type-III and Type IV patients were excluded from the study. A thorough case history was taken , clinical eamination was done and routine blood investigations were advised pre-operatively. The radiographic investigations done before surgery included an Intra oral periapical radiographs (IOPA) and a Digital Intra oral periapical radiographs (Digital IOPA). Under general anesthesia the alveolar defect was exposed. Cancellous bone obtained from the anterior illiac crest and the PRF obtained from the patients blood was used to augment the alveolar cleft defect. Subjects were not allowed to have any food or drink by mouth for the first 8 hours. Clear fluids were permitted for the next 2 hours, liquid diet for 3 days and then soft diet for one week. Routine oral hygiene procedures were advised. All the patients were recalled after 1 month and 3months following surgery. Evaluation of the treatment results was done by measuring the marginal bone level and radiographic density at six months post operatively. Marginal bone level was assessed by using Bergland grading scale and considering standard reference point’s i.e apex of the tooth adjacent to the cleft area and cement enamel junction (CEJ) of tooth adjacent to the cleft area on IOPA. Based on bergland grading scale, the alveolar cleft defect was graded as type-1, type-2, type-3, and type-4.4 Radiographic density was measured by DIGORA software on digital intra oral peri-apical radiographs by using bisecting angle technique. Direct standardized digital images were taken and using DIGORA Software system; an imaging plate (IP) with dimensions equal to 1.6 x 35 x 45mm was used. Image read out was displayed automatically on the computer screen. The radiographs taken for evaluation of the amount of graft take up by using the standard intra oral periapical radiographs(IOPA) and digital intra oral periapical radiographs. (DIGITAL IOPA) The machine was set at tube voltage 70kvp and tube current 13 Ma. The focus to skin distance was 20cms. Exposure time was maintained for conventional IOPA at 0.5sec and for digital IOPA at 0.4 sec. All the films were taken in bisecting technique, film was oriented close to the tooth and the central beam was through the cleft site at 40-45 degrees verticalRESULTS angulation, 60-70 degrees horizontal angulation.

10 patients were included in this study with a mean age 12 ±3.84. 6 were male and 4 were female patients. 2 patients (20%) had cleft on the right side and416 8 patients (80%) had cleft on the left side. 7 patients had naso-labial fistula 10th World Cleft Lip, Palate & Craniofacial Congress

/ naso-palatal fistula (70%) and remaining 3 were not having any naso-labial fistulas (30%). The pre-operative and post-operative changes in the marginal bone level is shown in table 1. In the present study mean preoperative, post- operative 1 moth and post-operative 3 months radiographic density (table-2) was recorded by using DIGORA Software as 54.9, 112.3 and 122.10 respectively. Mean difference comparison of radiographic density between pre-operative and post-operative 1month was 57.4, pre-operative and post-operative 3 months was 67.2, post-operative 1 month and 3 months was 9.8. Wilcoxon signed rank testDISCUSSION: (Z-test) was performed and p- value ≤ 0.05 was considered significant.

Primary alveolar grafting has a very detrimental effect on facial growth and esthetics. It inhibited the forward growth of the maxilla. Tertiary grafting cause progressive root resorption on the cervical third of roots of teeth adjacent to the cleft, which is caused by the contact of the grafted bone to the exposed root surface. Therefore alveolus repair should be performed in the mixed dentition period when ‘’secondary alveolar bone grafting’’ (SABG) offers great clinical benefits. The indications for anterior alveolar maxillary bone reconstruction are multiple, but the primary reason is to provide sufficient quantity and quality of bone in the cleft area to allow for eruption of teeth into the bone grafted area. Additional reasons include closure of oral nasal fistulas, providing adequate support to the nasal base on the unilateral cleft side, and possibly allowing for osseointegrated implants to be placed when teeth are missing.5,6 Cancellous bone is considered to be the material of choice, because of its rich cellularity, its rapid revascularization, and its potential to induce new bone formation. The anterior iliac crest is generally regarded as the donor site of choice. Cancellous grafts consist of actual cells and the incorporation process is much faster due to osteoinduction and osteoconduction. The cancellous grafts are also found to better enable tooth eruption. Cancellous bone grafts have greater osteogenic potential than solid blocks of cortical bone; Fresh autogenous bone grafts seem to support these procedures and can be still seen as the “gold standard”.7 Marginal bone level was assessed based on the height of the interdental bony septum between the adjacent teeth on either side of the cleft alveolus (usually a central incisor and canine).8 Based on bergland grading scale, Type- I indicates that the interdental septum height is essentially normal, while type- II indicates an interdental height that is ¾ of normal, type-III is less than ¾, and IV 417 10th World Cleft Lip, Palate & Craniofacial Congress indicates a lack of a continuous bony bridge and is considered a failure.4 In our study out of ten cases, 1 case showed type -1 MBL bone fill(10%) , 6 cases showed type -2 MBL bone fill (60%), and 2 patients showed type -3 MBL bone fill(20%) after a follow up period of 3 months. In one case graft was resorbed.(type-4 bone fill-10%). Out of ten cases Marginal bone level was improved in 7 cases from type- IV to type- I and II, in 2 cases type-IV to type-III and in one case graft was exposed due to infection. Final Clinical success rate was 70% (type I and II) and clinical failure rate (type III and IV) was 30%. This result coincides with the studies done by Yi –Lin Jia et al9 and Azahar Sheikh et al.10 Alveolar ridge morphology also improved appreciably in nine out of ten patients, facilitating orthodontic and prosthodontic rehabilitation across the cleft site. In nine cases radiographic density was significantly improved when compare with pre-operative and post-operative one and three month interval. In one case, the graft was infected and failed due to poor oral hygiene and lack of post-operative care. Bone formation by grafting is affected by many factors such as type of cleft, age of patient, width of cleft, size of oronasal fistula and dental development at the time of grafting. However, the ideal result is not always obtained. Retrospective studies of literature showed that grafted particulate cancellous bone and marrow (PCBM) was completely absorbed in 2% to 7.4% of cases and acceptable vertical bone formation was obtained only in 60 to 70% of cases by conventional SABG.11 Meij et al12 reported that greater than 30% of the volume of transplanted iliac crest bone in the cleft area was resorbed after 1 year. Honma et al13 reported that bone volume at the grafted cleft site at 1 year was significantly lower when compared with that of 3 months. Tai et al71 also showed a total mean volume loss of 43.1% at approximately 1 year after SABG. Nasreen Amanat et al15 analyzed a series of 47 cases and noted that graft was completely resorbed in 3 cases (6.4%) only. Feitchinger et al16 reported that mean bone loss in the 1st year after surgery to be as much as 49.5%. We found that 7 patients have no post-operative donor site pain, in the remaining 3 patient’s pain may be aggravated by local pressure, and by walking, and many of the examined patients were unable to lie comfortably on the affected side. All the patients reported walking within the second post- operative day. There was only one hematoma seen in one patient that resolved without surgical intervention, well respond to antibiotic treatment. 418 10th World Cleft Lip, Palate & Craniofacial Congress SUMMARY AND CONCLUSION

In summary that fresh autogenous cancellous bone graft was good graft material for SABG to reconstruct the cleft alveolus and it was more helpful for osteogenesis. Even though the total number of cases and period of follow up were too small to arrive at any definite conclusion, the encouraging result show that this technique has a significant role in the management of alveolar clefts. It is also felt that a more extensive study involving a larger sample and longer follow upBIBILOGRAPHY should be undertaken to confirm its advantages and any disadvantages. 1. Koberg WR. Present view of bone grafting in cleft palate (review). J maxillofac surg.1973;1:185-93. 2. Amin kazemi, Jeffrey W. stearns and Raymond J Fonseca. Secondary grafting in the alveolar cleft patients. Oral maxiollofacial surg clin N Am 2002;14:477-490. 3. Cohen M et al. secondary alveolar bone grafting. clin plast surgery. 1993;4:691-705. 4. Bergland O, Semb G, Abyholm FE. Elimination of the residual alveolar cleft by secondary bone grafting and subsequent orthodontic treatment. Cleft Palate J.1986;23:175-205. 5. Hans Enemark, Erik Krantz-Simonsen and Jan Erik Schramm Secondary bone grafting in unilateral cleft lip palate patients: indications and treatment procedure. Int. J Oral Surg. 1985;14:2-10. 6. Hans Enemark, steen sindent Pederson. Long term results after secondary bone grafting of alveolar clefts, J oral maxillofacial surgery, 1987;45:913-918. 7. Mitsuyoshi Lino, Hiroaki Ishi Junichi Sato and Kanichi Seto. Histological evaluation of autogenous iliac particulate cancellous bone and marrow grafted to alveolar clefts – a preliminary report of five young adult cases. Cleft palate-Craniofacial Journal.2000;1:55-60. 8. Ivy Kiemle Trindade, Reinaldo Mazzottini, Omer Gabriel da Silva Filho, Inge Elly Kleme Trindade and Maria Cristina Zindel Deboni. Longterm radiographic assessment of secondary alveolar bone grafting, outcomes in patients with alveolar clefts. Oral surg Oral med Oral pathol Oral radiol Endod.2005;100(3):271-277. 9. Yi-Lin Jia, David R.James and Michael Mars. Bilateral alveolar bone grafting; A report of 55 consecutively-treated patients. European Journal of Orthodontics.1998;20:299-307. 10. Azhar sheikh, Ulfat bashir, owaiskhalid durrani, rafia aneeqa lahooti, mohsin fazal. Evaluation of secondary alveolar bone grafting procedures. Pakistan Oral & Dental Journal 2011;31(2):246-48

419 10th World Cleft Lip, Palate & Craniofacial Congress 11. Shuji Yoshida, Kenichiro Suga et al Postoperative evaluation of grafted bone in alveolar cleft using 3-D computed tomography data. Cleft palate craniofacial journal.2013;50(6):671-677. 12. Van der Meij AJW, Baart JA, Prahl-Anderson B, et al, Computed tomography in evaluation of early secondary bone grafting. Int J Oral Maxillofac surg.1994;23:132-136. 13. Honma K, Kobayashi T, Nakajima T, et al: computed tomographic evaluation of bone formation after secondary bone grafting of alveolar clefts.J Oral Maxillofac Surg.1999;57:1209-1211. 14. Tai CC, Sutherland IS, McFadden L: Prospective analysis of secondary alveolar bone grafting using computed tomography. J Oral Maxillofac Surg.2000;42:1241-1246. 15. Nasreen Amanat and John D. Langdon. Secondary alveolar bone grafting in clefts of the lip and palate. Journal of cranio-Maxillofacial Surgery.1991;19(1):7-14. 16. Feichtinger M, Mossböck R, Kärcher H. Assessment of bone resorption after secondary alveolar bone grafting using three-dimensional computed tomography: a three year study. Cleft Palate Craniofac J. 2007;44:142-148.

Table 1: Distribution of study subjects based on Marginal Bone Level

420 10th World Cleft Lip, Palate & Craniofacial Congress Table 2: Distribution of study subjects based on Radiographic density

421 10th World Cleft Lip, Palate & Craniofacial Congress

Donor site morbidity associated with anterior iliac crest harvest for alveolar cleft bone grafting

Nalini Sailaja, M. Veerabahu, J. A. Nathan Post Graduate, OMFS, Ragas Dental College and Hospital, Chennai, India

Abstract

Autogenous bone grafting for the alveolar clefts was well established in the treatment of cleft lip and palate patients. Various donor sites for secondary alveolar bone grafting have been described. Iliac crest, calvarium, mandibular symphysis and tibia are among them. The iliac crest is considered to be the gold standard of donor site. Because of its accessibility and the quantity of bone available, relative ease of bone harvest and the defect is well covered and less prone to pathological fracture, iliac crest provides suitable site for augmentation. However the iliac bone harvesting is associated with many postoperative complications in the donor site such as infection, inguinal hernia, nerve injury, gait abnormality, peritoneal tear. This paper highlights about our study which gives brief outcomes of donor site morbidity in anterior iliac crest following bone harvesting for alveolar clefts.

422 10th World Cleft Lip, Palate & Craniofacial Congress

Management of Premaxilla in Bilateral Cleft Lip and Palate

G. VIVEK, M. Veerabahu, J. A. Nathan Post Graduate, OMFS, Ragas Dental College and Hospital

Abstract

The patient with a complete bilateral cleft lip and palate (BCLP) often presents with displaced posterior maxillary segments and premaxilla. The treatment of this condition can challenge even the most experienced clinicians. An important aspect of the bilateral cleft lip and palate (BCLP) patient is that the alveolar clefts cause the premaxilla to be mobile from birth and only apically fixed to the vomer bone. The premaxilla is often protruding due to the lack of sphincter function of the orbicularis oris muscle. This causes extreme abnormalities in the position of the premaxilla; sometimes, the whole segment is rotated and functional and cosmetic disorders result. Over time, there have been many forms of treatment aimed at changing the position of the premaxilla. This paper discusses about the management of the position of the premaxilla in complete bilateral cleft lip and palate (BCLP) patients.

423 10th World Cleft Lip, Palate & Craniofacial Congress

Prosthodontic Aspects Of Cleft Lip And Palate : A Review

Shankar Padmasree Department of Prosthodontist, Chettinad Dental College, Chennai, India

Management of cleft lip and palate is always a challenge since it involves multiple complications and demands for a multi-disciplinary approach. The treatment aims at providing a rehabilitation which would help the patient to lead a better social life. This in-turn aim at fulfilling the functional, esthetic, social and psychological demands of the patients. A prosthodontist plays an important role in management and rehabilitation. Prosthetic therapy aids the patient in developing normal speech, promoting deglutition and mastication and in closing off the oral cavity from the nasal cavity. In addition the prosthodontic therapy also aims at replacing the missing teeth and the osseous deficiencies. Obturators are the most common modality of prosthodontic treatment option. With the advent of implants, implant supported and retained prostheses have greatly aided in successful treatment for such patients. The paper reviews on the various prosthodontic treatment options , the success and acceptance of the various treatments.

424 10th World Cleft Lip, Palate & Craniofacial Congress

Oral Health – Related Quality Of Life & Dental Caries Status In Children With Orofacial Cleft. An Indian Outlook

N.Nagappan1, P.Rajesh2, S.Manikandan3, M.Muralimani4 1Senior Lecturer, Department of Public Health Dentistry, 2Prof & HOD, Department of Oral & Maxillofacial Surgery, 3Tutor, Department of Oral & Maxillofacial Surgery, Chettinad Dental College & Research Institute, Kelambakkam, Chennai 4Reader, Department of Oral and Maxillofacial Surgery, Sri Venkateswara Dental College & Hospital, Thalambur

ABSTRACT Aim

To assess the dental caries status and OHRQOL among orofacial cleft childrenMaterials reporting and methods: to a hospital in India.

Subjects were divided into two groups. Group 1: Cleft childrens (n=80), Group 2: Non Cleft childrens (n=80). DMFT, deft Index and Children Oral Health ImpactResults Profile questionnaire were recorded.

The mean DMFT was high in non cleft (3.51±2.45) childrens than cleft childrens (2.75±2.68), the mean deft was high in high in non cleft (1.11±0.96) childrensConclusion than cleft childrens (0.86±3.07).

Key wordsCleft childrens have negative impact on OHRQOL than non cleft childrens.

IntroductionCleft lip, OHRQOL, Orofacial Clefts, OHIP, Craniofacial.

Cleft lip and/or palate is the most common congenital craniofacial anomaly that has always been found in mankind [1].Oral clefts are birth malformations that involve the oral cavity, and may also affect the face [2].425 10th World Cleft Lip, Palate & Craniofacial Congress

Worldwide incidence of the cleft lip (CL) and palate is 1 in 600 (1:600) [3]. The overall worldwide prevalence of the CL with or without a cleft palate (CP) was 9.92/10,000. The prevalence of the CL was 3.28/10,000, and that of the CL and palate was 6.64/10,000 [4]. Clefts can be caused by a number of factors that affect the expectant mother early in the first trimester of pregnancy. These factors include infection and toxicity, poor diet, hormonal imbalance and genetic interferences [5]. Depending on the elemental characteristics of embryology, anatomy and physiology of the defect, the varieties of cleft lip and palate may be categorized as 1) those involving the lip and alveolus 2) those involving the lip and palate 3) those in which palate alone is affected 4) congenital insufficiency of the palate [6]. Oral health-related quality of life (OHRQoL) measures are subjective indicators based on information provided by individuals about their oral health status and its impact on various aspects of their life. Measures of OHRQoL provide essential information when assessing the treatment needs of individuals and populations, as well as when making clinical decisions and evaluating interventions, services and public health programs. Four domains are used to measure OHRQoL: oral symptoms, functional limitations, social towell-being a hospital and in India.emotional well-being [7]. Hence current study aimed to assess the dental caries status and OHRQOL among orofacial cleft children reporting Materials and methods STUDY DESIGN

STUDYA AREAcross-sectional descriptive study

A Hospital based study (Smile Train Center, Department of Plastic surgery, Sri Ramachandra Medical College & Hospital, Sri Ramachandra University, Chennai).STUDY POPULATION

This study was carried out on patients receiving care at the outpatient ward of the Department of Plastic Surgery, Sri Ramachandra Medical College & Hospital, INCLUSION Sri RamachandraCRITERIA University, Chennai. Cleft Children 426 10th World Cleft Lip, Palate & Craniofacial Congress ●●

●● Age: 8-16 years Childrens having purely congenital cleft lip/cleft palate and those having ●● cleft lip, alveolus and palate who was not operated. ●● Able to give informed consent and willing to participate ●● Not have a medically handicapping children ●● Not have a special class for mental disabilities Non NotCleft have Children a mental disorder ●●

●● Age: 8-16 years ●● Have no significant medical history. EXCLUSIONAble to give CRITERIA informed consent and willing to participate Cleft Children and Non Cleft Children ●●

●● History of any systemic disease. APPROVALNot willing AND to INFORMED participate. CONSENT

Ethical approval was obtained from the Institutional Review Boards of Saveetha University and Sri Ramachandra University. Informed consent was obtainedSAMPLE fromSIZE parentsESTIMATION or guardian of study participants.

Sample size required for the study was calculated to be N = 80 (Each group), with 80% power at 5% α- error, based on the studies conducted by Magdalena Stecslonicz et al (2007) [45] and Tahir Paul et al (1998) [37]. On the last day of examination 5 patients were reported to outpatient ward, they were alsoGROUP included in the study yielding a final sample size of 80.

Group I: Cleft Childrens (n=80) SCHEDULINGGroup II: Non Cleft Childrens (n=80)

Data collection was scheduled for a period of one month from 1st 427 10th World Cleft Lip, Palate & Craniofacial Congress examined per day. December 2012 to 31st December 2012. On an average, 3 individuals were SURVEY INSTRUMENT

status Awere validated obtained. questionnaire was obtained from Broder and Wilson- Genderson, 2007. Information about the demographic data and dental caries CHILD ORAL HEALTH IMPACT PROFILE [8]

The COHIP consisted of 34 items forming 5 conceptually distinct subscales: Oral Health, Functional Well-Being, Social/Emotional Well-Being, School Environment and Self-Image. It also contains 2 items to measure treatment expectancy and 2 items to measure global health. Global health and treatment expectancy items are not used in computing the overall COHIP 25 score. Below is a summary of the items and subscales (used with permission from Ralstrom, 2009) [11]. 01. Oral Health: Items 1, 2, 3, 4, 5, 6, 7, 8, 9, and 10 measure specific oral symptoms that are not necessarily related to one another (e.g., pain, spots on teeth). 02. Functional Well-Being: Items 11, 15, 20, 23, 25, and 27 relate to the child‟s ability to carry out specific everyday tasks or activities (e.g., speaking clearly, chewing). 03. Social-Emotional Well-being: Items 12, 16, 17, 19, 22, 24, 28, and 29 pertain to peer interactions and mood states. 04. School-Environment: Items 13, 18, 21, and 30 pertain to tasks associated with the school environment. self. 05. Self-image: Items 14, 26, 31, 32, 33, and 34 address positive feelings about

06. Treatment Expectancy: Items 35 and 36 measure expectations regarding the treatment process and outcomes. 07. Global Health: Items 37 and 38 assess overall feelings about oral and systemic health. The statements are formatted to elicit self-reports from the subject. Responses for the 7 positively-worded items are recorded as “never” = 0, “almost never” = 1, “sometimes” = 2, “fairly often” = 3, and “almost all of the time”428 = 4. The positively scored items were: 26 (1) Item 14. Been confident (2) Item 26. Felt you were attractive (3) Item 31. Have good teeth (4) Item 10th World Cleft Lip, Palate & Craniofacial Congress

32. Believe you will have good teeth (5) Item 33. Believe you will have good health (6) Item 34. Feel good about yourself (7) Item 35. Will feel better when treatment is completed Scoring of the remaining 31 negatively-worded items were reversed (0 = “almost all of the time”= 0, “fairly often”= 1, “sometimes”= 2, “almost never”= 3, “never”= 4). Higher COHIP scores reflect more positive OHRQoL while lower scores reflect lower OHRQoL. Subscales scores are calculated by summing the responses of the items specific to the subscale. The overall OHRQoL score is computed by summing the subscales scores. Treatment expectation scores and the overall health response are not included in the overall COHIP scale since these items are relevant only when the COHIP is used as part of a treatment assessment. Scores range from 0-140 for the overall scale. If more than two-thirds of the items in a subscale are missing, the subscale and the overall score are set to missing. If fewer items are missing for a subscale, the average of available items used and the sum of the subscaleCLINICAL is EXAMINATION calculated.

Clinical examination was conducted by a single examiner who had been trained through a series of clinical training sessions at the Department of Public Health Dentistry, Saveetha Dental College & Hospital, Chennai. After recording the questionnaire, dental examinations were conducted in a dental chair using a mouth mirror and explorer (No: 5,Shepard’s Crook). Instruments used were sterilized using standard protocol. Only completely filled forms were considered for analysis. Dental Caries examination was done according to decayed, missing and filledSTATISTICAL teeth (DMFT) ANALYSIS Index [9] and deft Index [10].

The data collected were entered in Microsoft Excel and analysed using statistical software package, SPSS 17.0 (SPSS Inc, Chicago, IL, USA). Descriptive statistics were computed and Independent T test was used to compare the meanResults scores of overall COHIP and their subscales.

Figure 1 depicts the distribution of study subjects. Among the 160 study subjects, 80 study subjects were Group I (Cleft Children) and 80 study subjects were Group II (Non Cleft Children). In Group I (Cleft Children), 63.8% & 36.2% were belonged to male and female respectively. In Group II (Non Cleft Children), 55% & 45% were belonged to male and female respectively. 429 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 2 depicts the distribution of the study subjects based on cleft types. Among the 80 study subjects, 26 (32.5%) belonged to Cleft Lip group, 26 (32.5%) belonged to Cleft Palate group and 28 (35%) belonged to Cleft Lip Alveolus and Palate group. Figure 3 depicts the mean deft score between groups. The mean deft score with cleft children and non cleft children was 0.86 ±3.07 and 1.11 ±0.96 respectively. Figure 4 depicts the mean DMFT score between groups. Among the 80 cleft children group, the mean decayed teeth, missing teeth, filled teeth and DMFT were 2.64±2.69, 0.00±0.00, 0.08±0.30 and 2.75±2.68 respectively. Among the 80 non cleft children group, the mean decayed teeth, missing teeth, filled teeth and DMFT were 1.96±0.91, 0.21±0.14, 1.06±2.11 and 3.51±2.45 respectively. Table 1 depicts the mean overall COHIP and subscales between groups. The mean Oral Health Well-being domain was 14.9±7.9 and 31.3±3.21 in cleft and non cleft children group respectively. The mean functional Well- being domain was 7.6±2.7 and 19.3±6.21 in cleft and non cleft children group respectively. The mean Social Emotional Well-being domain was 21.3±8.1and 26.2±5.42in cleft and non cleft children group respectively. The mean School Environment domain were 12.1±2.1and 14.22±0.9 in cleft and non cleft children group respectively. The mean Self Image domain was 14.7±3.9 and 20.8±0.88 in cleft and non cleft children group respectively. The mean Treatment Expectancy domain was 4.2±1.4 and 5.1±0.22 in cleft and non cleft children group respectively. The mean Global health domain was 2.9±0.6and 3.5±1.12in cleft and non cleft children group respectively. The mean overall COHIP was 70.6±14.2 and 111.82±12.34 in cleft and non cleft children group respectively. Independent T test showed that the relationship between mean ‘decayed ‘, ‘missing’ and ‘filled’ component and total mean DMFT score between group was found to be statistically significant in the following domains Functional Well-being, Social Emotional Well-being, School Environment and Overall COHIP.Discussion

The congenital malformation may result in impairment of oral cavity, face and thus knowing the magnitude of the damage and repair it at the earliest time as possible brings benefits for patients, families and caregivers. Cleft lip and palate are defects caused by an incomplete fusion of the nasal processes and the palatal processes between the late embryonic and early foetal period. The430 most important etiologic factors are considered to be genetic in nature, but 10th World Cleft Lip, Palate & Craniofacial Congress

environmental factors also have been identified to have a limited role. These patients usually require a comprehensive medical and dental treatment over an extended period of time [12]. According to epidemiological aspect, the present study indicates that the prevalence of orofacial cleft was found to be more in males (63.8%) than females (36.2%) which is consistent with several other studies [13 - 16]. The most prevalent type of cleft was Cleft Lip Alveolus & Palate (CLAP) 35%. Similarly a study by Al Omari et al (2004) reveals 48% of cases were affected with CLAP [17]. . The mean deft score for the current study were (0.86±3.07) and (1.11±0.96) in cleft and non cleft childrens respectively. The mean DMFT score for the current study were (2.75±2.68) and (3.51±2.45) in cleft and non cleft childrens respectively. Whereas in the study conducted by Rubina Shashni et al (2015), the mean DMFT were (10.14±6.09) and (12.73±3.42) in cleft and non cleft childrens respectively [18]. . The variation in dental caries experience could be due to the difference in methodology, study groups and differing age groups diet and environment factors between the studies. Among the cleft children group, the mean score for Oral Health Well-being domain was 14.9±7.9 which is higher than the study conducted by Jared A ward et al 2011(17.7±4.7) [19], Geels LM et al 2008(17.5 ±6.7) [20]and Pattamawan Konan et al 2015 (35.89±6.10) [21]. The mean score for Functional Well-being domain was 7.6±2.7 which is higher than the study conducted by Jared A ward et al 2011(24.8±5.9) [19], Geels LM et al 2008(21.3 ±6.2) [20] and Pattamawan Konan et al 2015 (23.59+4.37) [21]. The mean score for Social Emotional well being was 21.3±8.1 which is higher than the study conducted by Jared A ward et al 2011(24.3±6.7) [19], Geels LM et al 2008(22.2 ±10.6) [20] and Pattamawan Konan et al 2015 (31.25+6.13) [21]. The mean score for School Environment was 12.1±2.1 which is higher than the study conducted by Jared A ward et al 2011(13.1±3.0) [19], Pattamawan Konan et al 2015 (17.29+2.69) [21] and lower than the study conducted by Geels LM et al 2008(5.3 ±1.8) [20]. The mean score for self image was 14.7±3.9 which is higher than the study conducted by Jared A ward et al 2011(15.7±4.3) [19], Pattamawan Konan et al 2015 (19.75+5.50) [21] and lower than the study conducted by Geels LM et al 2008(8.7 ±3.3) [20]. The mean score for treatment expectancy and global health was 4.2±1.4 and 2.9±0.6 respectively which is which is higher than the study conducted by Jared A ward et al 2011 [19]. 431 10th World Cleft Lip, Palate & Craniofacial Congress

The mean score for overall COHIP was 70.6±14.2 which is higher than the study conducted by Jared A ward et al 2011(95.6±18.3) [19], Geels LM et al 2008(75.1 ±22.5) [20] and Pattamawan Konan et al 2015 (127.80+17.16) [21]. The results of this study indicate that clefts childrens have a significant impact on OHRQoL. Our results confirm that children with orofacial clefts had statistically significantly lower OHRQoL than control children for the overall COHIP score and for the Functional Well-being, Social-Emotional Well-being and school environment subscales. In contrast, some studies (Warschausky et al., 2002; Jokovic et al., 2004; Locker et al., 2005; Wogelius et al., 2009) [22-25] have found that clefts childrens have less impact on HRQoL. These findings may be due to differences in sample size and/or study population, as well as the use ofConclusion different questionnaires.

Cleft childrens have negative impact on OHRQOL than non cleft childrens in the aspects of Functional Well-being, Social-Emotional Well-being and school environment subscales.

Figure 1: Distribution of the study subjects

432 Figure 2: Distribution of study subjects based on cleft types 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 3: Comparison of mean deft score between groups

Figure 4: Comparison of mean DMFT score between groups

groups Table 1: Comparison of COHIP overall and subscale scores between

*indicates p<0.05 statistically significant 433 10th World Cleft Lip, Palate & Craniofacial Congress References 1. Sudheer G. Hongal, Anil Ankola, Laxminarayan Nagesh. Malocclusion and treatment needs of cleft lip and/or palate subjects aged between 12 and 18 years visiting KLE’s hospital, Belgaum, India. Journal of Oral Health & Preventive Dentistry2010; 8(3): 237- 242. 2. Gardenal M, Bastos PRHO, Pontes ERJC, Bogo D. Predominance of orofacials fissure diagnosed in reference service in resident cases in Mato Grosso do Sul State. Arq Int Otorrinolaringol 2011; 15(2): 133-41. 3. Mossey P, Little J. Addressing the challenges of cleft lip and palate research in India. Indian J Plast Surg 2009;42 Suppl:S9-18. 3. 4. IPDTOC Working Group. Prevalence at birth of cleft lip with or without cleft palate: Data from the International Perinatal Database of Typical Oral Clefts (IPDTOC). Cleft Palate Craniofac J 2011; 48:66-81. 5. Berkowitz S. The cleft palate story, ed 1. Chicago IL: Quintessence Publishing Company Inc., 1994. 6. Uma Sudhakar, Vijayalakshmi R, Ramesh Babu M, Anitha V, Bhavana J. Periodontal status of cleft lip and palate patients- A case series. Journal of the Indian Association of Public Health Dentistry 2009; 13: 99-104. 7. Jokovic A, Locker D, Stephens M, Kenny D, Tompson B, Guyatt G. Validity and reliability of a questionnaire for measuring child oral-health-related quality of life. J Dent Res. 2002; 81:459–63. 8. Broder HL, Wilson-Genderson M. Reliability and convergent and discriminant validity of the child oral health impact profile (COHIP child's version). Community Dent Oral Epidemiol 2007;35 Suppl 1:20-31. 9. Klein H, Palmer CE, Knutson JW. Studies on dental caries. I. Dental status and dental needs of elementary school children. Public Health Rep 1938; 53:751-65. 10. 10. Grubbel AO. A measurement of dental caries prevalence and treatment service for deciduous teeth. J Dent Res 1944; 23:163-8. 11. Ralstrom E. The Impact of Oral Health in Adolescent Patients with Sickle Cell Disease. Columbus, OH: The Ohio State University; 2009. Thesis dissertation. 12. Peterson LJ, Ellis E, Happ JR, Tuckup HR. Contemporary Oral and Maxillofacial Surgery. 3RD Edn; 656-679. 13. Gardenal M, Bastos PRHO, Pontes ERJC, Bogo D. Predominance of orofacials fissure diagnosed in refference service in resident cases in Mato Grosso do Sul State. Arq Int Otorrinolaringol 2011; 15(2):133-41. 434 10th World Cleft Lip, Palate & Craniofacial Congress 14. Amstalden-Mendes LG, Xavier AC, Antunes DK, Ferreira ACRG, Tonocchi R, Fett-Conte AC, Silva RN, Leirião VHV, Caramore LPC, Magna LA, Gil-da-Silva-Lopes VL. Time of diagnosis of oral clefts: a multicenter study. J Ped 2011; 87(3):225-30. 15. Cymrot M, Sales FCD, Teixeira FAA, Teixeira Junior FAA, Teixeira GSB, Cunha Filho JF, Oliveira NH. Prevalence of kinds of cleft lip and palate at a Pediatric Hospital in Northeast of Brazil. Rev Bras Cir Plást 2010; 25(4):648-51. 16. George L. Wehby GL, Naderi H, Damiano PC. Comparing the Visual Analogue Scale and the Pediatric Quality of Life Inventory for Measuring Health-Related Quality of Lifein Children with Oral Clefts. Int J Environ Res Public Health 2014, 11:4280-91. 17. Al Omari F, Al-Omari IK. Cleft lip and palate in Jordan: birth prevalence rate. Cleft Palate- Craniofacial Journal 2004; 41(6):609-12. 18. Rubina Shashni et al. Comparison of risk indicators of dental caries in children with and without cleft lip and palate deformities. Contemporary Clinical Dentistry 2015; 6(1): 58- 62. 19. Jared A Ward et al. Oral Health–Related Quality of Life in Children With Orofacial Clefts. Cleft Palate-Craniofacial Journal 2013; 50(2): 174–181. 20. L.M. Geels, J.M. Kieffer, Joh Hoogstraten, B. Prahl-Andersen . Oral Health-Related Quality of Life of Children With Craniofacial Conditions. Cleft Palate–Craniofacial Journal 2008; 45(5): 461-467. 21. Pattamawan Konan et al. Oral Health-Related Quality of Life in Children and Young Adolescent Orthodontic Cleft Patients. J Med Assoc Thai 2015; 98 (Suppl. 7): S84-S91. 22. Warschausky S, Kay JB, Buchman S, Halberg A, Berger M. Healthrelated quality of life in children with craniofacial anomalies. Plast Reconstr Surg. 2002; 110:409–414, discussion 415–416. 23. Jokovic A, Locker D, Stephens M, Kenny D, Tompson B, Guyatt G. Validity and reliability of a questionnaire for measuring child oralhealth-related quality of life. J Dent Res. 2002; 81:459–463. 24. Locker D, Jokovic A, Tompson B. Health-related quality of life of children aged 11 to 14 years with orofacial conditions. Cleft Palate Craniofac J. 2005; 42:260–266. 25. Wogelius P, Gjorup H, Haubek D, Lopez R, Poulsen S. Development of Danish version of child oral-health-related quality of life questionnaires (CPQ8-10 and CPQ11-14). BMC Oral Health. 2009; 9:11, doi: 10.1186/1472-6831-9-11.

435 10th World Cleft Lip, Palate & Craniofacial Congress

Dental Caries In Patients With Cleft Lip And Palate – Predisposing Factors And The Role Of Prevention

V.K. Balakiran III Year BDS- Faculty Of Dental Sciences, SRU, Sri Ramachandra University, Chennai, India

Abstract

The purpose of this paper is to assess the various predisposing factors causing dental caries in a patient with cleft lip and/or palate and discuss a suitable protocol for prevention of the same. There are difficulties in achieving optimal oral health by lack of access to brush all of the teeth, due to loss of elasticity of the surgically repaired lip and the anatomy of the fissure. The presence of a palatal fistula causes drainage of the fluid nasal to the oral cavity and food can escape through the nose and return to the mouth. This could increase the risk of caries, because sugars and cariogenic bacteria are present in the mouth for a longer period of time. From birth the dentist can administer Infant Oral Health care measures that follow the guidelines of the American Academy of Pediatric Dentistry. Anticipatory guidance protocols meticulously followed can possibly bring a child caries free. Using chemical therapeutic agents for cleaning the orthodontic appliances, avoiding cross contamination by not having direct contact, periodic monitoring of bacterial plaque, informing the parents about dentally safe foods and drinks and teaching brushing technique around the area of the cleft are essential for early prevention. The information discussed when used to help in early dental management of such patients may be of paramount importance as the patients are exposed to pediatric dental care very early and itIntroduction serves as a great opportunity to prevent caries in the future.

Cleft lip and palate (CLCP), the most common of the craniofacial anomalies is a congenital anomaly having a global incidence of 1 in 700 births. The mouth of the fetus is formed within the first 3 months of pregnancy. Failure of fusion of the pre-maxilla with the palatine processes and the fusion of upper lip436 an anterior palate within this time can cause the clefting of the upper lip or 10th World Cleft Lip, Palate & Craniofacial Congress

palate. Due to the primary involvement of the oral cavity tissues in formation, surrounding dentition may have some anomalies as well such as, congenitally missing teeth, supernumerary teeth, ectopic primary teeth- lateral incisors being most common, enamel hypoplasia, fused teeth, microdontia, macrodontia, reduced alveolar bone support from teeth adjacent to the cleft leading to mobility, rotated teeth and even sometimes a completely mobile premaxilla due to bilateral clefting of the lip and palate. The care of the oral cavity of a patient with CLCP is a complex and extensive treatment, which requires a maxillofacial surgeon who will perform the necessary surgeries (palatine surgery, for example), as well as an orthodontist, who prepares the patient for surgery and controls the proper growth of the jaws. As a result the role of the general dentist in rehabilitation is often overlooked as the parents have higher expectations of the surgery and orthodontics and are unaware of the great importance of dental attention at this stage. Early childhood caries shows greater prevalence in patients with cleft lip and palate for a wide host of reasons such as poor oral hygiene, enamel hypoplasia, the palatal fistula, intraoral removable as well as orthodontic appliances as will be discussed. Therefore it is the objective of this paper to bring to light of heath care professionals, the importance of early dental attention by means of Infant Oral Health programs, follow up of Anticipatory Guidelines as listed by the American Academy of Pediatric Dentistry (AAPD) and introducing the concepts of Dental Homes and incorporating these into the general CLCP management protocols. These measures can facilitate bringing a childDental with Caries cleft In lip Patients and palate With potentially Cleft Lip cariesAnd Palate free.

Numerous studies have been conducted to investigate the prevalence of caries in patients with CL/P (cleft lip or palate). A study in Japan showed that children with CLCP present greater incidence of caries in both temporary and permanent teeth, unlike patients without this malformation4 The Streptococcus mutans and lactobacillus are important pathogenic species for the formation of dental caries. A study on patients under 2 years with CLCP detected the presence of S. mutans in 45% of patients.5 This suggests the increased effects of S. mutans under two years of age. The first treatment for a patient with CLCP is the placement of attachments for orthodontic treatment, and this type of palate facilitates early colonization of lactobacillus and S. mutans. The classic study of Lauterstein and Mendelsohn (1964),19 as well as the studies conducted by King et al.20 and Tannure et al.21, found no significant differences in caries between children with and without cleft. Zhu et al.16 , Al- Wahadni et al.17 , and Stec-Slonic et al.18 observed that patients with a cleft437 10th World Cleft Lip, Palate & Craniofacial Congress condition, not only are at a high risk of caries, but also have an even higher prevalence of caries than individuals without cleft. Regarding the type of cleft, Ankola et al.22 observed that children with cleft lip were more susceptible to tooth decay than those with cleft palate. When comparing the cleft lip only or cleft palate only with the cleft lip and palate, the authors noticed that the latter showed a higher prevalence of caries. Zhu et al.16 found a higher prevalence of caries in cleft lip with or without palate. Byan et al.15 observed a higher percentage of caries in bilateral, as compared to unilateral, cleft lip and palate. The prevalence of caries seems to be distributed evenly between the genders of children with cleft. This finding was observed in other studies (Byan et al.15 , Neves et al.23 ). Johnsen and Dixon (1984)9, Bokhout et al. (1997) found 3.5 times more decayed surfaces1, Ahluwalia et al. (2007)7 all found patients with oral clefts with higher incidence of childhood caries than non oral. Johnsen and Dixon (1984) illustrated that the carious incisors in children with CP alone resembled early childhood caries.9 However, other studies have found no difference in prevalence of S. mutans between patients with CLCP and those without clefting.2, 6. Some studies like the ones conducted by Chapple and Nunn (2001)3 did not find any significant differences in the caries experience among different cleft types, especially in the permanent dentition. The studies that suggested otherwise provided contrasting opinions, too. Paul and Brandt (1998)8 believed that children with CP or CL had better oral health than those with CLP, which correlated with the findings of Johnsen and Dixon. Factors Predisposing To Dental Caries and Early Childhood Caries in PatientsOral Hygiene with Cleft Lip and Palate

Studies such as the one by Paul and Brandt (1998)8 suggest that not only do patients with cleft lip and palate show poorer oral hygiene in comparison with subjects without any malformation, but patients with CLCP show poorer hygiene even than those with just CP. This may suggest that the patient finds difficulty in accesibility during brushing as a result of the reduced elasticity of the surgically repaired lip. Supernumerary teeth and crowding may further add toPalatal restriction Fistula of and access Enamel for natural Hypoplasia cleansing by saliva as well as brushing9.

Sometimes clefting of the palate can form a fistula with the nasal cavity causing drainage of the nasal fluid into the oral cavity. This nasal fluid promotes the438 adherance of plaque to the teeth and hence promotes cariogenic bacterial 10th World Cleft Lip, Palate & Craniofacial Congress

growth10. This fistula can also lead to passage of food substances into the nasal cavity and regurgitation into the oral cavity. This allows food to be in the oral cavity for a longer period of time and thus acting as substrates for cariogenic bacteria. Enamel hypoplasia is fairly common in patients with CL/P and this results in roughened surface and decreased mineral content9. This can increase theIntraoral susceptibility Appliances to caries.

Some children with cleft palate can use intraoral devices very soon after birth. These devices are made of acrylic and can be used up to 18 months. However, the apparatus of acrylic can facilitate early colonization of S. mutans.6 This early colonization predisposes patients to an early onset of caries in the primary dentition. It has been shown that children with oral clefts treated with oral appliances are more likely to get caries at the age of two years than children with oral fissures but without intra-orals appliances.12 The consistency of a soft diet indicated for orthodontic treatment further increases its retention and alter the swallowing of saliva, allowing substrates to remain in the mouth for a longer period, favoring cariogenic bacteria. In the study performed by Lin and Tsai (1999)11, children with CL/P who were bottle fed at bedtime showed an increased risk of developing baby-bottle tooth decay than did the children with oralDiscussion clefts who did not take a bottle to bed. The or

al cavity has both cariogenic and non cariogenic bacteria as commensal flora. When the resting pH of the oral cavity falls below the critical level of 5.5, deminerelization of tooth structure starts and causes invasion of caries. Salivary ions, salivary pH and enzymes can act as a buffer to these pH levels but ingestion of dietary carbohydrates and starches favours the acidic producion by the cariogenic bacteria such as S. mutans and Lactobacillus due to breaking down of the carbohydrates. This dietary negligence poses especially as a problem in patients with CL/P as it leads to very early onset of caries and manifestation of Early Childhood Caries. The studies discussed above suggest that patients with CL/P or both had a higher onset of early childhood caries as compared to patients without the oral fissures. While some studies discussed have shown otherwise, the inconsistencies in findings among researchers could be largely attributed to the differences in the methodologies used in research, multifactorial nature of ECC, difference in levels of dental awareness of studied test groups and probable differences in their cultural practices. 439 10th World Cleft Lip, Palate & Craniofacial Congress

Among the various factors that could facilitate early colonisation, the literature discussed suggests that patients with a clefting of the palate had better oral hygiene than those with CP. This could be not only because of the restricted access to clean due to the reduced elasticity of the lip that was surgically repaired, but also due to patients not brushing adequately due to fear of using the toothbrush around the cleft region. Moreover, the fissure itself if manifested palatally can cause a palatal fistula with the nasal cavity leading to either, a flow of nasal fluid into oral cavity which promotes plaque accumulation and colonization of cariogenic bacteria and/or regurgitation of food which might escape into the nasal cavity causing greater time of food in oral caviy acting as effective substrate for cariogenic bacterial flora. Use of acrylic intraoral devices for orthodontic purposes as well as other intraoral acrylic devices may be vital post surgically to shape the jaws and prevent collapse of the premaxila but the acrlylic material itself can facilitate early colonization of species such as S. mutans and lead to increased risk of ECC. In addition, treatment with such appliances requires the patient to be on a soft diet which invariably equate to foods with simple sugars which can be broken down by the cariogenic flora to increase intraoral pH. This coupled with the reduced ability to swallow due to presence of intraoral device causes more saliva accumulation and potential for colonization of cariogenic bacterial species. Furthermore, parents of the patient are overwhelmed by the thoughts of their children having CL/P and allow dietary indulgences of the patients which could lead to consumption of foods which favour cariogenic bacterial growth. Seeing that the focus of this discussion is ECC, it is important to discuss how the child can be exposed to cariogenic bacterial colonisation even before the eruption of primary dentition. This can occur before the eruption of teeth and as young as 3 months of age. The primary source of S. mutans is usually the mother (Kohler and Bratthall, 197813; Li and Caufield, 199514). Close contact with the mother such as sharing food and utensils and kissing the lips of the child may provide repeated exposures of SM transmission from adult to child (Wan et al., 2003a).12 Parents of children suffering from CLCP are often overwhelmed by the condition and wish to know as much as they can about treatment. Oral health generally ranks at the lower end of the priority scale, and insufficient attention is devoted toward this area as compared to the other specialities in the multidiscipliniary approach. Parents are usually not aware of their patient’s increased susceptibility to dental caries, and they unintentionally neglect or are unaware of the importance of good oral hygiene and dietary practices. It ofis importantmaintaining for good a member oral health of the and CL/P dietary team, habits preferably before a thedentist, baby to is have born. early The discussions and provide direct information to the parents on the significance 440 10th World Cleft Lip, Palate & Craniofacial Congress

strategies that will be discussed for anticipation and early prevention of ECC in suchPrevention patients Strategies may be vital in bringing such patients completely caries free.

As suggested by the discussed studies, ECC is more prevalent in patients with cleft lip and palate and for the suggested reasons ECC could manifest in such patients much more easily. It is vital as the role of the dentist to not only educate the parents of the children on important preventive strategies that may be practiced from home, but also implementation of their own clinical preventive measures. The AAPD has published a set of policies on approaching the prevention of ECC and providing the appropriate infant oral health care before the age of 6 years by means of anticipatory guidelines. Further the AAPD talks about familiarizing the families of the patient on the concept of Dental Homes and introducing them no later than 12 months of age to provide not only parental dentalguidance histories, with direct thorough relationship oral examination, between dentist proper and age parentappropriate but also brushing caries disease risk assesments. The first visit should include thorough medical and demonstrations and fluoride varnish treatment if required. The patient’s parents must be informed about the prevention plan and intervals for periodic re-evaluation as this is vital. Some of the important policy statements from the AAPD on prevention of ECC include reducing the parents’/siblings’ S. mutans levels to decrease transmission to child; this can be done by educating the parents on the etiology and prevention and their involvement in both, maintaining oral health by their own proper brushing, flossing and rinsing with chlorhexidine mouthwashes, watching their diets to not consume too many high sugar food and periodically visiting a dentist for assesments and removal of any necessary carious lesions or fillings and so forth before the birth of the child. Also minimising salivary sharing activites like sharing utensils and toothbrushes, providing professoinally applied fluoride varnishes for children at risk of ECC (<3years of age requires about a “rice” shaped or “smear” sized withamount added of fluoride sugar for toothpaste; the patients 3-6 is years important. of age requiresChildren ashould “pea” sizedfurther amount) not be , avoiding frequency of consumption of high sugar diets like soft drinks or milk put to sleep with a feeding bottle and can instead replace this with a pacifier as the former could predispose to Nursing Bottle Caries, infants must be weaned from the bottle between 12-18 months of age. Toothbrushing techniques specific for patients with CL/P have to be taught to the parents and they must brush the child’s teeth atleast twice in a day. Excessive breastfeeding (over 7 times a day) can be linked to increased risk of ECC in infants. 441 10th World Cleft Lip, Palate & Craniofacial Congress

Apart from these AAPD policies which largely apply to children with CLCP, some more specific recommendations were introduced at the Children’s Oral Health Service, Royal Children’s Hospital in Brisbane, Australia like- chemo- therapeutic agents such as chlorhexidine could be used to clean intraoral appliances when they are removed from the mouth, parental education on cross-contamination should form a part of the parents’ oral health education preferably before and rein- forced after the birth of the baby and postsurgical oral health education and demonstration of toothbrushing technique is essential especiallyConclusions around the cleft area.

The issue of most paramount importance that we must address is the lack of effective preventive strategies and/or their implementation in order to completely prevent ECC in patients below the age of 3-6 years in order to bring them caries free. The main cause of this problem is the lack of awareness of the parent to approach the dentist early in their child’s life to prevent the onset of ECC. However cases of CLCP serve as a golden opportunity to dentists to see very young patients below 3 years of age as they come to a doctor for treatment very early by the age of 3 months. It is at this stage that the dentist who is part of this multidiscipliniary team, steps forward and starts the informative process to the parents and initiate preventive measures this early against ECC. Patients with CL/P generally display poorer oral hygiene and higher susceptibility to ECC because of a host of reasons such as difficulty in achieving adequate plaque control associated with dental anomalies and defects from lips or palate, fixed appliance orthodontic therapies having a higher risk in plaque accumulation metabolismaround the applianceof simple attachments and formation of incipient enamel lesions due to carious invasion and the higher cariogenic potential of bacteria after sugars that usually fall under the recommended diet by the orthodontist due to these appliances. Hence, it is the duty of the dentist to integrate an oral preventive program into the CL/P treatment protocol for these patients so early in their life and educate of their parents in order to establish desirable habits and a favorable oral environment during the overall managementReferences of their cleft conditions. 01. Bokhout B, Hofman FXWM, Van- Limbeek J, Kramer GJC, Prahl- Andersen B.Incidence of Dental Caries in the Primary Dentition in Children with a Cleft Lip and/or Palate. Caries Res 1997; 31: 8-12. 2. Lucas VS, Gupta R, Ololade O, Roberts GJ. Dental Health Incides and Caries Associated Microflora in Children With Unilateral Cleft Lip and Palate. Cleft Palate Craniofac J 2000; 37:447-452.

442 10th World Cleft Lip, Palate & Craniofacial Congress 3. Chapple JR, Nunn JH. The Oral Health of Children with Clefts of the Lip, Palate, or Both. Cleft Palate Craniofac J 2001; 38:525-528. 4. Ishida R, Yasufuku Y, Miyamoto A, Ooshima T, Sobue S. Clinical survey of caries incidence in children with cleft lip and palate. Shoni Shikagaku Zasshi. 1989; 27: 716-24. 5. Bokhout B, Van-Loveren C, Hofman FXWM, Buijis JF, Van-Limbeek J, Prahl-Andersen B. Prevalence of Streptococcus mutans and Lactobacilli in 18-Month-Old Children with Cleft Lip and/or Palate. Cleft Palate Craniofac J 1996; 33:424-428. 6. van Loveren C, Buijs JF, Bokhout B, Prahl-Andersen B, Ten Cate JM. Incidence of mutans streptococci and lactobacilli in oral cleft children wearing acrylic plates from shortly after birth. Oral Microbiol Immunol. 1998; 13: 286-91. 7. Ahluwalia M, Brailsford SR, Tarelli E, Gilbert SC, Clark DT, Barnard K, Beighton D.Dental Caries, Oral Hygiene, and Oral Clearance in Children with Craniofacial Disorders. J Dent Res 2007; 83:175-179. 8. Tahir P, Brandt R.Oral and Dental Health Status of Children with Cleft Lip and/or Palate. Cleft Palate-Craniofac J 1998; 35:329-332. 9. Johnsen D, Dixon M. Dental Caries of Primary Incisors in patients with Cleft Lip and Palate. Cleft Palate J 1984; 21: 104-109 10. Turner C, Zagirova AF, Frolova LE, Courts FJ, Williams WN. Oral health status of Russian children with unilateral cleft lip and palate. Cleft Palate Crani- ofac J. 1998;35:489–494. 11. Lin YT, Tsai CL. Caries prevalence and bottle-feeding practices in 2-year-old children with cleft lip, cleft palate, or both in Taiwan. Cleft Palate Craniofac J. 1999;36:522–526. 12. Wan AK, Seow WK, Purdie DM, Bird PS, Walsh LJ, Tudehope DI. Oral col- onization of Streptococcus mutans in six-month-old predentate infants. J Dent Res. 2001;80:2060– 2065. 13. Kohler B, Bratthall D. Intrafamilial levels of Streptococcus mutans and some aspects of the bacterial transmission. Scand J Dent Res. 1978;86:35–42. 14. Li Y, Caufield PW. The fidelity of initial acquisition of mutans streptococci by infants from their mothers. J Dent Res. 1995;74:681–685. 15. Byan Z, Du M, Bedi R, Holt R, Jin H, Fan M. Caries prevalence and oral health behavior in Chinese children with cleft lip and/or palate. Pediatr Dent. 2001 Sep-Oct;23(5):431-4. 16. Zhu WC, Xiao J, Liu Y, Wu J, Li JY. Caries experience in individuals with cleft lip and/or palate in China. Cleft Palate Craniofac J. 2010 Jan;47(1):43-7.

443 10th World Cleft Lip, Palate & Craniofacial Congress 17. Al-Wahadni A, Alhaia EA, Al-Omari MA. Oral disease status of a sample of Jordanain people ages 10 to 28 with cleft lip and/or palate. Cleft Palate Craniofac J. 2005 May;42(2):304-8. 18. Stec-Slonic M, Szczepanska J, Hirschfelder U. Comparison of caries prevalence in two populations of cleft patients. Cleft Palate Craniofac J. 2007 Sep;44(5):532-7. 19. Lauterstein AM, Mendelsohn M. An analysis of the caries experience of 285 cleft palate children. Cleft Palate J. 1964 Jul;1(29):314-9. 20. King NM, Wong WL, Wong HM. Caries experience of chinese children with cleft lip and palate. 2012 Mar 1. 21. Tannure PN, Costa MDE, Küchler EC, Romanos IC, Granjeiro JM, Vieira AR. Caries experience in individual with cleft lip and palate. Pediatr Dent. 2012 Mar-Apr;34(2):127-31. 22. Ankola AV, Nagesh L, Hegde P, Karibasappa GN. Primary dentition status and treatment needs of children with cleft lip and/or palate. J Indian Soc Pedod Prev Dent. 2005 Jun;23(2):80-2. 23. Neves LT, Gomide MR, Costa B, Ciamponi AL. Disease behavior caries patients with cleft lip and cleft between 7 and 66 months. Pesqui Odontol Bras. 2002 Aug-Sep; 16 (suppl): 185-7.

444 10th World Cleft Lip, Palate & Craniofacial Congress

What we see commonly is not normal always- Tongue Tie a Midline Defect

MS Saravana Kumar Pediatric Dental Surgeon, Chennai, India

Abstract

Tethered oral tissues are abnormal tissues of the tongue, lips, and cheeks. They are frequently referred to as frenula. Tongue is an organ-not only attached to rest facial planes directly or indirectly, but it also has attachment on the hyoid bone, mandible, temporal bones and other structures in the head. Structurally it can affect the digestive tract and respiratory tract. It has innervation from cranial nerves-trigeminal, facial, glossopharyngeal, vagus and hypoglossal. ONE in TEN babies is born with a tongue tie that affects their ability to BREASTFEED. Symptoms of Infant tongue-tieincludes baby fails to latch on to bare breast, shallow latch, baby constantly slips off the breast, baby makes clicking noises when sucking, and baby gasps for air mid feed. An effect on the breast includesnipple pain that is not improved after help with positioning and attachment, blisters on your nipples, mastitis, blocked ducts and/or incomplete emptying of the breast. Effects on the baby includes weight loss in early days may be greater than expected, be fussy or unsatisfied, be anxious when feeding, have reflux or be on meds and have colic. When considering revision, the parent needs to consider the types of procedures used for revision. Snipping the tongue, this method has a long history and was recorded as being used by midwives to free the tongue of neonates with feeding difficulties as far back as 1697. Results were described as: improving breastfeeding immediately – 57%, and feeding better by 24 hours – 80%. Revision using lasers is relatively new and suitable option for neonates No general anaesthetic is used, but an analgesic gel might be applied. The procedure is very quick, taking only 2 to 3 minutes to perform; there is virtually no bleeding, no pain, no risk of infection and better healing. Babies can be breastfed immediately after the procedure. 445 10th World Cleft Lip, Palate & Craniofacial Congress

"Know it early or late" - psychological effect of prenatal diagnosis of cleft lip and palate - A systematic review

Sreejith VP1, Joy R. Das2, Arjun Gopinath3, Arun V4 1Prof. & Head, 2Senior Lecturer, 3Reader, 4Post Graduate Student, Dept. of Oral & Maxillofacial Surgery, Kannur Dental College, Kannur, Kerala, India

ABSTRACT Introduction

Cleft lip and/or palate is the most common congenital craniofacial anomaly. The knowledge of the same brings about a great psychological disturbance in the parents, mainly because of worries related to the future of the baby. Prenatal diagnosis of the craniofacial anomalies is possible with the adventMaterials of newerand methods imaging modalities like 3D/4D ultrasound and MRI.

PubMed, Cochrane and google scholar searches were made with search strings “prenatal diagnosis cleft lip palate”, “antenatal diagnosis cleft lip palate”, “anomaly scan”, “psychological effect cleft lip palate” “prenatal counselling cleft lip palate”. Out of the results obtained studies which evaluated the psychological aspectsResults of parents of cleft children were considered.

Electronic search yielded more than 1500 articles. Most were the technical aspects of the methods used for the prenatal diagnosis of cleft lip and palate. 8 studies dealt with the psychological effects on the parents by an early diagnosis of the deformity and how it affected the parent child relation, treatment and howDiscussion the parents could be counselled.

Prenatal diagnosis of cleft lip and palate is being used since last few decades. The early diagnosis of the abnormality is further improved by newer imaging446 modalities like 3D/4D ultrasound and MRI. Prenatal diagnosis enables 10th World Cleft Lip, Palate & Craniofacial Congress

appropriate and timely counselling of the parents by the cleft team and helps standardto instil aof sense life. of preparedness for the family which highly improves the quality of treatment received by the child enabling a near to normal quality and KEYWORDS

INTRODUCTIONCleft lip and palate, Prenatal diagnosis, psychological effect Rationale

Cleft lip with or The without pr palate (CL/P) is the most common congenital birth defect occurring1 in the population. it has a varied prevalence of 1 in 500 to 1 in 2500 live births. evalence of orofacial clefts varies by race, and occurs more frequently among Asians and Native Americans, followed by Whites, Hispanics, and African Americans. The critical period for cleft development ranges from the 4th to the 12th week of intrauterine life. Clefts of the primary palate develop between the 4th and 7th weeks of intrauterine life, while clefts of the secondary palate develop between the 8th and 12th embryonic weeks.

The relationship of a mother with her child begins during2 pregnancy in the early and grows over time during the period of pregnancy. When a child is born thewith family. a cleft lip and palate (CLP), parents experience various emotional reactions like shock,3 sadness, fear, grief, guilt, and anger that disrupt the equilibrium of Parents must be able to successfully handle their feelings, cope with this crisis, and reorganize to meet the needs of their affected child. Not only the facial appearance but also functions such as hearing, phonation,4 mastication, deglutition, and ventilation are altered by this malformation. An accurate prenatal diagnosis of the cleft lip and palate anomaly is critical for establishing long-term treatment5 planning, prediction of prognosis, and proper counselling with the parent. Though not a routine procedure according to American Institute of Ultrasound and Medicine, accurate evaluation of craniofacial malformations is usually possible with the ultrasound scan performed during the pregnancy. The accuracy of ultrasonography for prenatal diagnosis of cleft lip and palate is dependent on the experience of the sonologist, maternal body6 type, fetal position, and the amount of amniotic fluid and the type of cleft. 3D ultrasonography and prenatal MRI improve the accuracy of prenatal diagnosis of orofacial clefts. 3D ultrasonography provides7 more precise image of the defect and it enhances 2D examination of fetus. 447 10th World Cleft Lip, Palate & Craniofacial Congress

The parents need to be informed and counselled regarding the severity of the cleft deformity and the predicted outcome and options of repair by a trained cleft palate team in association with the radiology team. Though there is no intrauterine treatment for cleft lip and palate, both mother and child benefit from early diagnosis and counseling. The parents are able to spend time to adjust with the reality of the malformation as well as adequate time to educate themselves about the condition. Thus the initial shock of 8,the 9 diagnosis usually can be overcome by a systematic and planned counselling. A multidisciplinary team approach is now accepted as the standard of care in dealing with these complexObjectives patients.

With improvements in the imaging technology and advent of prenatal diagnosis, several centers across the globe are conducting prenatal screening and counselling for the parents. Though many studies dealt the technological aspects of prenatal diagnosis and the counselling for the deformity post birth, very few studies have discussed the psychological effect of prenatal diagnosis and prenatal counselling on expectant parents. Similarly, there are no systematic reviews that exclusively reviewed this particular aspect of cleft lip and palate. This review is aimed to through light into the less known and discussed psychologicalMATERIALS AND aspect METHODS of prenatal diagnosis of cleft lip and palate deformity. Information sources

PubMed, Cochrane and google scholar electronic searches were made with search strings “prenatal diagnosis cleft lip palate”, “antenatal diagnosis cleft lip palate”, “anomaly scan cleft lip palate”, “psychological effect cleft lip palate” “prenatal counselling cleft lip palate”. All the available articles were screenedSearch criteria by the authors.

Search terms included ("prenatal diagnosis"[MeSH Terms] OR ("prenatal"[All Fields] AND "diagnosis"[All Fields]) OR "prenatal diagnosis"[All Fields]) AND ("cleft lip"[MeSH Terms] OR ("cleft"[All Fields] AND "lip"[All Fields]) OR "cleft lip"[All Fields]) AND ("palate"[MeSH Terms] OR "palate"[All Fields]) OR ("antenatal"[All Fields] AND "diagnosis"[All Fields]) OR "antenatal diagnosis"[All Fields]) AND ("cleft lip"[MeSH Terms] OR ("prenatal care"[MeSH Terms] OR ("prenatal"[All Fields] AND "care"[All Fields]) OR "prenatal care"[All Fields] OR "prenatal"[All Fields]) AND ("counselling"[All Fields] OR 448 "counselling"[MeSH Terms] OR "counselling"[All Fields]) AND ("cleft lip"[MeSH 10th World Cleft Lip, Palate & Craniofacial Congress

Terms] OR ("cleft"[All Fields] AND "lip"[All Fields]) OR "cleft lip"[All Fields]) ANDEligibility ("palate"[MeSH criteria Terms] OR "palate"[All Fields])

Out of the results obtained studies which evaluated the psychological aspects of parents of cleft children were selected and the studies which dealt with the technical aspects were excluded. The abstracts and full text of the selected studies were completely and individually reviewed and only those which dealt with how the prenatal or post-natal diagnosis of the cleft influenced theInclusion parents and were exclusion selected. criteria

The studies were selected if it described or made specific mention of prenatal counselling delivered to parents of a baby with a prenatal diagnosis of one or more congenital anomalies or assessed the psychological outcomes associated with prenatal counselling. The studies were excluded if only post- natal counselling were used and if there was no mention of prenatal diagnosis and its usefulness for the child. The details of the study including the names of researchers, year of publication,RESULTS the conclusions and the effects were narrowed down and compiled

The search resulted in more than 1500 articles. Avoiding duplications resulted in around 500 articles. Most of them dealt the technical aspects of prenatal diagnosis like the use of ultrasound and its modifications or the use of Magnetic Resonance imaging. 8 studies concentrated on the prenatal diagnosis and its implications on the treatment of the cleft child and the psychological effects on parents and also on how well the parents were able to adjust with the diagnosis and how the diagnosis could be utilized for betterment of the child. Most of the studies were questionnaire basedType ones. of study Name of author Year Participants post natally or Davalbhakta and 2000 Retrospective 124 parents either Hall9 questionnaire study prenatally (27) diagnosed to have cleft children

449 10th World Cleft Lip, Palate & Craniofacial Congress

Céline Rey-Bellet, 2004 Retrospective 29 parents of cleft Judith Hohlfeld10 questionnaire study children Rachel Nusbaum & 2008 Interview 20 parents, 12 in pre- Robin E. Grubs et natal group and 8 in al11 post-natal group diagnosed prenatally J. Kuttenberger, J. N. 2010 Retrospective 73 parents, 12 were Ohmer, E. Polska12 questionnaire study study prenatally diagnosed Yueh-Tzu Hsieh & Yu- 2013 Purposive sampling 16 mothers Mei Yu Chao & Judith Shu-Chu Shiao13 Senem Zeytinoglu, 2016 Descriptive 17 couples (10 Maureen P. Davey et qualitative study prenatal and seven al14 postnatal) attended either Matthew R. Greives, 2016 Retrospective 112 parents who Casey L. Anderson et questionnaire study al15 pre-natal (86) or post-natal (26) cleft counselling clinics Sarah Marokakis, 2016 Systematic review Electronic database anomalies Nadine A. search for congenital Kasparian16

There were 4 retrospective questionnaire surveys, 1 systematic review, 1 interview based survey, 1 descriptive qualitative study and 1 purposive sampling study. In all the studies, parental outcomes were assessed after birth. Few of the parents received had children diagnosed with cleft lip prenatally and received a pre-natal counselling by the cleft palate surgery team. Others had not received a prenatal diagnosis and hence attended only the post-natal clinics. 9 Davalbhakta et al conducted a retrospective study of 124 primary cleft lip and/or palate repairs. In their study, 30% were diagnosed antenatally and received antenatal counselling. The rest received postnatal counselling. Of those who had received an antenatal diagnosis 85% felt that the diagnosis prepared them psychologically for the birth of the child with deformity. Most of the parents who received the prenatal counselling was satisfied with the same and those who felt counselling to be confusing had only postnatal counselling. Most of the parents went ahead with the pregnancy and since they were well aware of the options for management, they had never thought of terminating the pregnancy. 450 10th World Cleft Lip, Palate & Craniofacial Congress

10 Rey-Bellet et al evaluated 29 parents who were prenatally diagnosed to have cleft lip/palate babies. Parental reactions at the time of diagnosis varied among them but most of them had severe psychological shock. Feelings of guilt, anxiety, fear and sadness were also noticed among the parents. All the parents were explained in detail regarding the deformity and had full satisfaction about the prenatal counselling and diagnosis. Counselling during the antenatal period timewas considered to warn and essential prepare by family all the as parents. well. and Prenatal friends diagnosis gave the parents enough time tobalance their feelings and accept the child at birth. They had

11 Nusbaum et al conducted interviews with 20 parents of children with cleft lip with or without cleft palate to explore parental experiences of a diagnosis. The main discussions were regarding the advantages and disadvantages of prenatal diagnosis, preparedness for birth of child and most of the parents preferred receiving the diagnosis prenatally, and the awareness that the deformity is treatable brought about great satisfaction among the parents. The early diagnosis also helped the parents to have interactions with similar parents and have a better understanding of the condition. 12 Kuttenberger et al conducted a questionnaire survey to evaluate parents’ experiences of the first counselling at the cleft center concerning timing and content of the consultation and the quality of the information provided. In order to reduce anxiety, confusions and uncertainty, clear and consistent information about the condition, its possible treatments17 and prognosis has to be given during the initial counselling at the cleft center 13 Hsieh et al performed a purposive sampling study to identify the psychosocial factors that affect expecting mothers who continue their Cleft lip/ palate pregnancy to term. Most of the mothers initially experienced a sort of psychological stress and sorrow and had received blames from other relatives. This again concentrate on the necessity of a proper counselling which enables management.a better care of the child. Else if diagnosed after birth, the initial hickups among the parents and relatives might lead to the neglect of child, resulting in a poor

14, 15 Zeytinoglu, and Greives also conducted similar studies and were of the similar opinion that a prenatal diagnosis experience provided an opportunity to work as a team with the cleft surgeons and to be supportive of each other. Most of the parents had concerns regarding the wellbeing of the child and especially regarding the feeding techniques which can be adopted. A thorough prenatal counselling helped to alleviate such concerns and led to a more successful parenting. 451 10th World Cleft Lip, Palate & Craniofacial Congress DISCUSSION

The time period immediately after the diagnosis and the first year after the birth is most challenging for parents. Receiving a prenatal diagnosis of cleft lip and palate is challenging for parents, and there are high levels of psychological distress after diagnosis. A systematically planned counselling will assist the parents to adjust during this difficult time. Primary goal of prenatal counselling is to educate parents about their child’s congenital anomaly. The intense emotional waves experienced during counselling hinders parents’ ability to understand the information provided. Hence repeated counselling by the cleft palate surgeons, gynecologists,18 maternal fetal medicine specialists and psychologists are mandatory. The early diagnosis of the condition also helped the parents19 to undergo a genetic counselling to identify underlying genetic problems. The counselling should be tailored to individual parents and expert psychologist20 opinion has to be taken for improving the effectiveness of the counselling. Another important concern for the parents is the use of feeding devices specially manufactured for cleft babies. The initial exposure to these devices is bound to create confusion and apparent anxiety among the parents. With the prenatal diagnosis and the training in the use of these bottles the problems usually faced after delivery can be minimized.15 Another concern that could be addressed by a proper prenatal counselling is the parents apprehension about the appearance of child and the speech problems that the child may develop. With structured21 counselling it is possible to give the parents an insight about these factors. The early counselling of parents also enabled them to have a good backup of internet sources for child care especially related LIMITATIONSto feeding of child.

This study was performed by a literature review of PubMed, google scholar and Cochrane databases only. Articles in English only where considered. Articles where an electronic edition was not there was not considered for the study. All the studies together evaluated 391 parents only which is a small sample. Out of them only 50 percent attended the prenatal clinics. So a broader study which involves a larger sample size is mandatory to evaluate the efficacy of prenatal counselling. Similarly, most studies used a retrospective questionnaire oftechnique results is which not possible again has with limitations the existing because data. only few questions were raised. The difference in pattern of questions also exists and a proper standardization

452 10th World Cleft Lip, Palate & Craniofacial Congress CONCLUSION

Prenatal ultrasound imaging is an important diagnostic tool used in the evaluation and treatment of pregnant women. With advances in technology, the resolution and image quality has considerably improved and newer modalities like 3D/4D ultrasound and Magnetic resonance imaging of the child are gaining popularity in the field of prenatal diagnosis of congenital abnormalities. The presence of a congenital anomaly like cleft lip/ palate has a severe effect on the general psychological wellbeing of the parents. If the condition can be identified early in the prenatal period itself, the parents can take help from the cleft surgeons and get a very good idea regarding the possible methods of treatment and be well prepared to have a child with the deformity. The aim of prenatal counselling is to educate and create awareness among parents about the child’s congenital anomaly. The intense emotions experienced during counselling may hinder parents’ ability to understand the information provided and parents may want and benefit from additional information, this helps in the overall betterment of the parent as well as the child especially in a situation which demandsCompeting early interests intervention for good results.

REFERENCESNone declared., Funding, None., Ethical approval, Not required. 01. Prevalence at birth of cleft lip with or without cleft palate: data from the International Perinatal Database of Typical Oral Clefts (IPDTOC). The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association. 2011;48(1):66-81. 02. Heidrich SM, Cranley MS. Effect of fetal movement, ultrasound scans, and amniocentesis on maternal-fetal attachment. Nursing research. 1989;38(2):81-4. 03. Steinberg JP, Gosain AK. Thirty Years of Prenatal Cleft Diagnosis: What Have We Learned? Plast Reconstr Surg. 2015;136(3):550-7. 04. Baker SR, Owens J, Stern M, Willmot D. Coping strategies and social support in the family impact of cleft lip and palate and parents' adjustment and psychological distress. The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association. 2009;46(3):229-36. 05. Jones MC. Prenatal diagnosis of cleft lip and palate: detection rates, accuracy of ultrasonography, associated anomalies, and strategies for counseling. The Cleft palate- craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association. 2002;39(2):169-73.

453 10th World Cleft Lip, Palate & Craniofacial Congress 06. Evans MI, Hume RF, Jr., Johnson MP, Treadwell MC, Krivchenia EL, Zador IE, et al. Integration of genetics and ultrasonography in prenatal diagnosis: just looking is not enough. American journal of obstetrics and gynecology. 1996;174(6):1925-31; discussion 31-3. 07. Wang LM, Leung KY, Tang M. Prenatal evaluation of facial clefts by three-dimensional extended imaging. Prenat Diagn. 2007;27(8):722-9. 08. Matthews MS, Cohen M, Viglione M, Brown AS. Prenatal counseling for cleft lip and palate. Plastic and reconstructive surgery. 1998;101(1):1-5. 09. Davalbhakta A, Hall P. The impact of antenatal diagnosis on the effectiveness and timing of counselling for cleft lip and palate. British journal of plastic surgery. 2000;53(4):298- 301. 10. Rey-Bellet C, Hohlfeld J. Prenatal diagnosis of facial clefts: evaluation of a specialised counselling. Swiss medical weekly. 2004;134(43-44):640-4. 11. Nusbaum R, Grubs RE, Losee JE, Weidman C, Ford MD, Marazita ML. A qualitative description of receiving a diagnosis of clefting in the prenatal or postnatal period. Journal of genetic counseling. 2008;17(4):336-50. 12. Kuttenberger J, Ohmer JN, Polska E. Initial counselling for cleft lip and palate: parents' evaluation, needs and expectations. International journal of oral and maxillofacial surgery. 2010;39(3):214-20. 13. Hsieh YT, Chao YM, Shiao JS. A qualitative study of psychosocial factors affecting expecting mothers who choose to continue a cleft lip and/or palate pregnancy to term. The journal of nursing research : JNR. 2013;21(1):1-9. 14. Zeytinoglu S, Davey MP, Crerand C, Fisher K, Akyil Y. Experiences of Couples Caring for a Child Born with Cleft Lip and/or Palate: Impact of the Timing of Diagnosis. Journal of marital and family therapy. 2016:n/a-n/a. 15. Greives MR, Anderson CL, Dean RA, Scerbo ML, Doringo IL, Bebbington MW, et al. Survey of Parent Experiences in Prenatal Visits for Infants With Cleft Lip and Palate. The Cleft palate-craniofacial journal : official publication of the American Cleft Palate- Craniofacial Association. 2016. 16. Marokakis S, Kasparian NA, Kennedy SE. Prenatal counselling for congenital anomalies: a systematic review. Prenatal Diagnosis. 2016;36(7):662-71. 17. Hunfeld JA, Tempels A, Passchier J, Hazebroek FW, Tibboel D. Brief report: parental burden and grief one year after the birth of a child with a congenital anomaly. Journal of pediatric psychology. 1999;24(6):515-20.

454 10th World Cleft Lip, Palate & Craniofacial Congress 18. Aite L, Trucchi A, Nahom A, Casaccia G, Zaccara A, Giorlandino C, et al. Antenatal diagnosis of diaphragmatic hernia: parents' emotional and cognitive reactions. Journal of pediatric surgery. 2004;39(2):174-8; discussion -8. 19. Petrucelli N, Walker M, Schorry E. Continuation of Pregnancy Following the Diagnosis of a Fetal Sex Chromosome Abnormality: A Study of Parents' Counseling Needs and Experiences. Journal of genetic counseling. 1998;7(5):401-15. 20. Kolker A, Burke BM. Grieving the wanted child: ramifications of abortion after prenatal diagnosis of abnormality. Health care for women international. 1993;14(6):513-26. 21. Hasanzadeh N, Khoda MO, Jahanbin A, Vatankhah M. Coping strategies and psychological distress among mothers of patients with nonsyndromic cleft lip and palate and the family impact of this disorder. The Journal of craniofacial surgery. 2014;25(2):441-5.

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Computer assisted Morphometrics in complex cranial vault reconstructive surgery:

Harsh Pipalia, Paul C. Salins, Samarth Shetty, Praveen Ganesh Department of Craniofacial surgery, Mazumdar Shaw Medical Centre, Narayana health city, Bommasandra, Bangalore, Karnataka, India

Abstract Introduction:

Morphometrics refers to the quantitative analysis of form, a concept that encompasses size and shape. In craniofacial surgery, surgeons are often faced with reconstruction of complex deteriorated structures caused by tumors, trauma or congenital abnormalities Based on the type of defect or abnormality; reconstruction should be planned according to the individual anatomy and physiology in order to achieve the best functional and aesthetic outcome. Various techniques have been described in the literature for fabrication of patient specific titanium cranial implants. On this account, we present computer assisted planning and utilization of morphometric principles for fabrication of patient specific titanium implants in the reconstruction of complex cranial defects.Materials and Methods:

Ten Patients with complex cranial defects more than 5cm were included. CT scan was done for all the patients. Stereolithographic (STL) model was made .The DICOM images were fed into the 3D Dolphin Imaging software and reformatted contour were transferred over the defect on STL model using modeling wax. Titanium moulds were fabricated following surface scans of reconstructedResults: contour on STL

A successful anatomic and aesthetic outcome without any major complication were achieved in all the patients

456 10th World Cleft Lip, Palate & Craniofacial Congress Conclusion:

The principles of morphometry in combination with computer assisted surgical planning can provide an insight to the envelope of surgical correction andKeywords: provide psychological preparation and education to the patient.

Morphometrics, cranial defects

457 10th World Cleft Lip, Palate & Craniofacial Congress

New treatment modality for maxillary hypoplasia in cleft patients - Review

Sarika.K1, Rajasigamani2 1II year Post Graduate, 2Professor and Head, Department of Orthodontics, Annamalai University, Chidambaram, India

Abstract

Treatment of cleft lip and palate requires a multidisciplinary team. These people usually have Maxillary hypoplasia due to congenital reduction in midfacial growth and the effects of the surgical scar from cleft lip and palate repair.Protraction facemask were mainly used for the managemnt of maxillary hypoplasia However the loss of dental anchorage have been reported with the oruse mini of tooth implants borne anchorage devices such as lingual arches etc.This side effects can be minimized using temperary skeletal anchorage device such as miniplates

Even though this method showed promising results, they still relied on facemask wear, and thus patient compliance. So now we using intermaxillary elastics applied to miniplates to protract the maxilla in cleft patients. The elastic forces used in this method were lower than facemask therapy forces and continuous traction is more favorable than heavy intermittent forces. The effect of the intermaxillary elastic forces was throughout the nasomaxillary structures. patients showed improvements in the skeletal relationship, primarily through maxillary advancement with little effect on the dentoalveolar units or any change in mandibular position. This review paper highlights abouts the function , indications , surgical techniqueINTRODUCTION and placement of miniplates for maxillary protraction

Cleft lip and palate occurs in about 1 to 2 per 1000 births in the developed world.Most children who have their clefts repaired early enough are able to have a happy youth and social life. Research has shown that during the early preschool years children with cleft lip and/or cleft palate tend to have a self- concept that is similar to their peers without a cleft. However, as they grow older and458 their social interactions increase, children with clefts tend to report more 10th World Cleft Lip, Palate & Craniofacial Congress

dissatisfaction with peer relationships and higher levels of social anxiety . An adolescent with cleft lip and/or cleft palate will deal with the typical challenges faced by most of their peers including issues related to self-esteem and social acceptance. Children with unilateral or bilateral cleft lip and palate are usually at risk for poor facial growth. They are prone to developing midfacial retrusion related to maxillary hypoplasiad a ornarrow growth hard retardation palate. secondary to excessive palatal scarring. As a result, CLP patients usually present with class III malocclusion, a retruded midface, an Furthermore, because a significant proportion of patients with maxillary deficient Class 111 skeletal malocclusion ultimately require orthognathic surgery and any treatment approach that could eliminate the need for, or reduce the extent of, future surgery would be of great benefit to these patients. Depending on the age of the patient and the extent of their midfacial development, some of these early problems can be corrected using midfacial or orthopedic protraction forces. These forces increase growth at the circummaxillary sutures and the maxilla can be repositioned anteriorly. Previously young patients with maxillary hypoplasia are usually treated with a protraction facemask. A heavy force is applied on the maxilla to stimulate its growth in a forward and downward direction and to redirect mandibular growth . However, this can results in a posterior rotation of the mandible and increased vertical dimension of the force. Moreover, as the forces were applied on the teeth, dental compensations can also be observed Beak et al. reported that facemask attached to the skeletal anchorage can be an effective alternative treatment modality for maxillary hypoplasia with minimal unwanted side effects in cleft patients . However it still depend on the facemask wear and also we can give the force only for 14 hours per day .Over the past decade, orthodontic skeletal anchorage devices have gained significant traction in the orthodontic community. Class III elastics attached to titanium miniplates as an anchorage device, offer the possibility to apply full-time orthopedic forces between the maxilla and mandible, while reducing dentoalveolar compensation, and moreover wearing maxillomandibular elastics is without doubt socially less constraining than wearing a face-mask. 459 10th World Cleft Lip, Palate & Craniofacial Congress TEMPORARY SKELETAL ANCHORAGE DEVICES

Temporary anchorage devices (TADs) refer to implants, screws, pins and miniplates,on plants that and are palatal specifically implants. designed for the purpose of providing skeletal anchorage. There are three most commonly used TAD categories;microscrews,

Titanium miniplates are attractive as an orthodontic implant anchor for a variety of reasons. They have been proven safe and effective as fixation plates for fractures and osteotomies in oral and maxillofacial surgery and orthopedics for more than 30 years. Their design allows the surgeon and orthodontist to avoid tooth roots when placing the plates and orthodontically moving teeth. They are adaptable to a variety of conditions.One of the more exciting applications of miniplate anchorage has been in the treatment of maxillary hypoplasia which would normally require orthognathic surgery. the treatment Miniplates plan that designed come inby a the variety orthodontist of shapes and sizes. The surgeon must have an assortment of lengths and shapes to meet the needs of the patient and

Two L-shape miniplates were placed at the zygomatic buttress under local anesthesia by a maxillofacial surgeon and fixed with three miniscrews, the distal ends of the miniplates were exposed through the attached gingiva between the upper first permanent molars and second premolars to control vector of elastic traction. In the mandible, two L-shape miniplate were placed under local anesthesia and were fixed by three miniscrews. The terminal ends of the miniplates were exposed between the lower central and lateral incisors. teethThe ideal and mental position foramen. for the insertion of the miniplates was evaluated using a panoramic radiograph in order to avoid damage to the roots of the adjacent TREATMENT PROCEDURE

Oral and maxillofacial surgeons who receive referrals for surgical placement of orthodontic anchorage devices need to understand the principles underlying this concept and develop a customized surgical protocol for ideal placement of the implants. Improper placement will result in failure of implants, encroachment on vital anatomic structures and ultimately a failure to provide a stable anchorage for the planned orthodontic treatment. After local anesthesia and surgical disinfection a vertical incision around the canine area was performed. An atraumatic subperiosteal dissection was done in infrazygomatic crest area and surgical miniplates were fixed with three 460 self tapping screws on both sides of infrazygomatic crest area. The end of the 10th World Cleft Lip, Palate & Craniofacial Congress

miniplates was exposed between the canine and first premolar area and this was modified as a hook and 2 miniplates were inserted in the anterior mandible between and inferior to the left and right permanent lateral incisor and canine.. In all sites, the miniplates were placed with the attachment arm exiting through attached tissue. at or near the mucogingival junction. All mucoperiosteal flaps wellwere tolerated secured withby patients. 4/0 resorbable sutures. The surgical sites were allowed to heal for 2 to 3 weeks before orthopaedic loading. The surgical procedures are

Four weeks after the placement of the miniplates, their mobility should be checked by the surgeon and orthodontic latex elastics (3/16′′ heavy size) were attached to the hooks of the miniplates to generate approximately 100 g of force on each side. The patient has to wear a tightly fitting and well-retained lower removable appliance in order to disoclude the upper and lower jaws during maxillary traction. The patient was instructed to wear the removable appliance full-time except for eating, contact sports, and tooth brushing; he was also told to replace the elastics every day (Fig.2). The traction force can be doubled after 1 month, and the final 250g of force per side was reached after 2 months. After attainingDISCUSSION the required overjet fixed orthodontic therapy should be started.

The concept of skeletal anchorage was first introduced by Creekmore and Eklund in 1983, when they stated that “with screws, pins, or some other readily removable implant anchored to the jaws, forces might be applied to produce tooth movement in any direction without detrimental reciprocal forces.” Absolute skeletal anchorage is a viable alternative to conventional extraoral orthodontic appliances such as headgear , etc Before introducing TADs, orthodontists have tried growth modifications by applying orthopedic forces to the teeth Therefore, dentoalveolar compensations rather than alterations of the facial growth were mainly responsible for improvement of malocclusion . To avoid dental compensations, titanium miniplates can be used to apply the orthopedic forces. Ahn et al. showed that the midface can be pulled forward over a mean distance of 8 mm using bone-borne traction hooks in combination with an extraoral face bow . Lee et al. reported that facemask therapy in conjunction with miniplates (FM/MP) induced a greater advancement of the maxilla , less posterior repositioning and opening rotation of the mandible, and less proclination of maxillary incisors compared to routine facemask therapy associated with rapid maxillary expansion. Kayaa 461 et al. also reported that facemask with miniplates offer an advantage for 10th World Cleft Lip, Palate & Craniofacial Congress correcting mild/moderate maxillary retrusion in class III patients . Beak et al. used this technique (FM/MP) to treat maxillary hypoplasia in cleft patients and they concluded that FM/MP can be an effective alternative treatment modality for maxillary hypoplasia with minimal unwanted side effects in cleft patients. Although , the aforementioned studies showed promising results, the y still relied on facemask wear, and thus patient compliance. Intermaxillary elastics applied to miniplates to protract the maxilla in our cleft patients seems to be better . The elastic forces used in this method were lower than forces of facemask therapy . This moderate continuous traction may be more favorable than heavy intermittent forces. Forward displacement/ modeling of the maxilla was significantly greater. The continuous elastic traction may result in more bone formation than the intermittent forces generated by a face mask. Another difference compared to face mask therapy is the skeletal anchorage applying the forces directly on the bone surface of the jaws We are not sure that higher forces result in more growth changes. But, high forces may exceed the maximal resistance of the external cortical plate of the infrazygomatic crest and lead to bone loss and loosening of the screws. For this reason we don't use forces higher than 200 grams . Significant displacement of the maxilla associated with minimal mandibular growth will result in a clear reduction in facial concavity was observed . Dental analysis measurements showed minimal dentoalveolar compensations. The palatal plane rotated counterclockwise moderately . This finding was the result of the direction of force application between the maxillary and mandibular miniplates, which were located below the center of resistance of the maxilla. Pure bone-borne orthopedic forces applied with intermaxillary elastics on miniplates were shownCONCLUSION to enhance midfacial growth in maxillary-deficient patients.

Intermaxillary elastics applied to miniplates are a promising technique for maxillary protraction with minimal dentoalveolar compensation in cleft lip and palate patients.This method can be even used in older patients were response.more anchorage is needed due to interdigitation of maxillay sutures. Carefully controlled studies are needed to elucidate the factors that affected treatment References 1. Berkowitz S. Cleft lip and palate: diagnosis and management. 2nd ed. Berlin: Springer Science & Business Media; 2006:392-394.

462 10th World Cleft Lip, Palate & Craniofacial Congress 2. Pangrazio-Kulbersh V, Berger J, Kersten G. Effects of protraction mechanics on the midface. Am JOrthod Dentofac Orthop 1998;114(5):484-91. 3. Westwood PV, McNamara Jr JA., Baccetti T, Franchi L, Sarver DM. Long-term effects of Class III treatment with rapid maxillary expansion and facemask therapy followed by fixed appliances. Am JOrthod Dentofac Orthop 2003;123(3):306-20. 4. Dogan S. The effects of face mask therapy in cleft lip and palate patients. Ann Maxillofac Surg 2012; 2(2):116-20. 5. Heymann GC, Cevidanes L, Cornelis M, De Clerck HJ, Tulloch JF. Three-dimensional analysis of maxillary protraction with intermaxillary elastics to miniplates. Am J Orthod Dentofac Orthop 2010; 137(2): 274-84. 6. Kaya D, Kocadereli I, Kan B, Tasar F. Effects of facemask treatment anchored with miniplates after alternate rapid maxillary expansions and constrictions; a pilot study. Angle Orthod2011;81(4):639-46. 7. Nartallo-Turley PE, Turley PK. Cephalometric effects of combined palatal expansion and facemask therapy on Class III malocclusion. Angle Orthod. 1998;68:217–224 8 Cha KS. Skeletal changes of maxillary protraction in patients exhibiting skeletal class III malocclusion: A comparison of three skeletal maturation groups. Angle Orthod 2003;73:26. [PubMed: 12607852] 9 McNamara JA. An orthopedic approach to the treatment ofClass III malocclusion in young patients. J Clin Orthod.1987;21:598–608. 10 Hickham JH. Maxillary protraction therapy: diagnosis andtreatment. J Clin Orthod. 1991;25:102–113. 11 Tahmina K, Tanaka E, Tanne K. Craniofacial morphology in orthodontically treated patients of class IIImalocclusion with stable and unstable treatment outcomes. Am J Orthod Dentofacial Orthop 2000;117(6):681–690 12 Tollaro I, Baccetti T, Franchi L.Mandibular skeletal changes induced by early functional treatment of Class III malocclusion: a superimposition study. Am J Orthod Dentofacial Orthop 1995;108(5): 525–532 13 Tollaro I, Baccetti T, Franchi L.Mandibular skeletal changes inducedby early functional treatment of Class III malocclusion: a superimpositionstudy. Am J Orthod Dentofacial Orthop 1995;108(5): 525–532 14 Battagel JM. Predictors of relapse in orthodontically-treated Class III malocclusions. Br J Orthod 1994;21(1):1–13

463 10th World Cleft Lip, Palate & Craniofacial Congress 15. Franchi L, Baccetti T, Tollaro I. Predictive variables for the outcome of early functional treatment of Class IIImalocclusion. AmJ Orthod Dentofacial Orthop 1997;112(1):80–86 16. Jacobson A, Jacobson R. Radiographic cephalometry from basic to 3-D imaging. 2nd ed. London: Quintessence Publishing Co,Inc; 2006: 63-78. 17. Jacobson A, Jacobson R. Radiographic cephalometry from basic to 3-D imaging. 2nd ed. London:Quintessence Publishing Co, Inc; 2006: 205-17.

464 10th World Cleft Lip, Palate & Craniofacial Congress

The importance of using 3D cephalometry in planning reconstructive surgery with craniofacial deformities

1Rahimov Chingiz, Habil. 1Hajiyeva Gunel, 2Novruzov Zaur, 1Farzaliyev Ismayil 1Azerbaijan Medical University, Department of Oral and Maxillofacial surgery, 2Azerbaijan Medical University, Department of Children’s Dentistry, Azerbaijan

Introduction

Treatment of congenital and acquired craniofacial deformities is one of the most trending topics within reconstructive surgery. In order to correct shape of the skull and face, one should perform significant movements of osteotomized osseous segments in new position that requires clear navigation instructions. The application of quite reasonable 2D cephalometry in dento- facial abnormalities is not always effective in case of craniofacial deformities. Current progress of computer technologies allows us to create 3D models of craniofacial abnormalities and determine new navigation guidance to improve outcomes of reconstructive procedures and follow orthodontic treatment. The aim of this study is to identify the informative benchmarks on the 3D cephalometry and their application in preplanning of both surgical procedures andMaterials orthodontic and methods setup in treatment of congenital craniofacial deformities.

Treatment outcomes of patients with diagnosis of Crousone syndrome and frontonasal dysplasia have been analyzed. Virtual preplanning of treatment was based on data collected from CT examination by the means of Materialise Mimic Research software (Materialise, Belgium). In all cases the method of 3D cephalometry was used as the determining. All virtual data was carefully documented and used during surgical procedure. The postoperative results wereResults assessed clinically and radiologically.

In all cases no significant postoperative complications were noted. Reasonably functional and aesthetic results were achieved. Orthodontic465 10th World Cleft Lip, Palate & Craniofacial Congress setup was applied directly after surgery with no additional major surgical interventions.Conclusions

The application of 3D planning systems and 3D cephalometry can significantly improve functional and aesthetic results, as well as decrease total time of patient’s rehabilitation.

466 10th World Cleft Lip, Palate & Craniofacial Congress

Rare Craniofacial Clefts - Our Experience

Mahendra Maharjan, Juna Gurung, Basant Kumar Mathema Department of Plastic, Cosmetic and Maxillofacial Surgery, B and B Hospital, Nepal

Abstract:

The incidence of craniofacial clefts is between 1.43 and 4.85 per 100000 births. Treatment of these clefts is very challenging surgically as well as psychologically. We here aim to share our experience in treating the rare craniofacial clefts at the Department of Plastic, Cosmetic and Maxillofacial Surgery, B and B Hospital, Nepal.

467 10th World Cleft Lip, Palate & Craniofacial Congress

A journey of surgery in crouzon syndrome-3 to 30 years

R.MURUGAN Senior Lecturer, Division of Oral & Maxillofacial Surgery, RMDCH, Chidambaram, India

ABSTRACT

Crouzon syndrome is an autosomal dominant genetic disorder known as a branchial arch syndrome. Preliminary surgery is typically used to prevent the closure of sutures of the skull from damaging the brain's development. Without surgery, blindness and mental retardation are typical outcomes. Each child is different and managing this syndrome requires multimodal tasks involved in it . We in our centre performed a series of procedure for a patient at different age levels starting from 3 to 30 years of age for crouzon syndrome. At 3 years of age we did cranioplasty and correction of upper facial skeletol following that at 11 years of age we corrected middle facial skeletol with high level maxillary advancement. At the age of 24 we corrected lower facial skeleton by mandibular set back and also open bite correction. Following that we also performed various dental corrections and had a followup till date. My paper emphasis on the multimodal tasks behind the management of these type of syndrome.

468 10th World Cleft Lip, Palate & Craniofacial Congress

Checklist Manifesto for Craniosynostosis correction

V. Suresh1, K. Sridhar2, Vijay Sankaran2, Deepika Gandhi2, Shrishu Klamath2, K.Raghavendran2 1Junior Consultant, 2Institute of Craniofacial Aesthetic and Plastic Surgery, SRM Institutes for Medical Science, Chennai

Introduction

Craniosynostosis is a craniofacial malformation which involves the premature closure of either one or more sutures presenting itself in isolation or along with any of its associated syndromes. Although it presents itself in approximately 1 in every 2000 odd live births, it always comes with additional consequences like raised Intra Cranial Pressure cranial base deformities. The fact that these surgeries involve some of the most vital structures of the body, take a long time, involve patients of very young age groups and has a multidisciplinary effort makes it very important to have set protocols. The protocol involves criteria for case selection, pre-operative investigations, intra operativeMaterial and monitoring, Method post-operative care apart from the surgical procedures.

The current protocol for peri-operative management of craniosynostosis correction was prepared using the experience of all the cases done by the team in yester years. The aim was to prepare and implement the developed protocol for all the children undergoing the craniosynostosis correction surgery. The team which involved Plastic surgeons, Maxillo-facial surgeon, Neurosurgeon, Anaesthetist and Paediatric Intensivists applied the proposed protocol to successive cases of craniosynostosis who were operated in our hospital. (Table:1)PRE- OPERATIVE Case selection and Timing of surgery:

Children requiring fronto-orbital advancement were between 1yr and 20 days to 3yrs and 4 months and two cases of Hypertelorism correction were 16yrs. 469 10th World Cleft Lip, Palate & Craniofacial Congress Clinical history

if any wereA complete noted. clinical history including familial history was taken. Milestones were recorded and complaints of headaches, vomiting and visual disturbances Radiological Investigations

A CT with 3D reconstruction was done. A corresponding stereolithographic model was prepared from the CT. The model helped in visualising intra-cranially and also saved a lot of theatre time.

Haematological Investigations Blood grouping, Haemoglobin, Prothrombin time, Activated Partial Prothrombin time were evaluated. Specific actions are taken in case of any variationOther department in values.By opinions routine a nasal swab was taken and checked for MRSA.

The team was led by Senior Plastic surgeon and opinions were obtained from Maxillofacial and Neuro surgeons,Ophthalmologists, Paediatrician, Paediatric intensivists and Cardiologists. The patients were then sent to the Anaesthesiologist for a proper airway assessment after whose consent the bloodCounselling was reserved for the surgery (3 units of PRBC) obtained.A counselling with the parents was done to explain about the procedure along with its benefits and potential risks.An informed consent was then Part Preparation

470 The surgical site was either shaved in its entirety or a strip of hair was 10th World Cleft Lip, Palate & Craniofacial Congress

removed according to the patient’s preference and a thorough wash was given withINSIDE an THEantibacterial THEATRE solution.

As it is a major surgery of long duration involving vital organs in children it is always better to have everything in place prior to the surgery for careful and continuous monitoring during and after the surgery. An appropriate size ET tube, either flexo -metallic or south ray tube was placed in the midline of the mouth and secured well with tegaderm and tapes. ET cuff pressure monitoring was mandatory. ECG, pulse oximeter,a precordial stethoscope were placedand BP and end tidal carbon Core dioxidetemperature monitoring monitoring were done was asdone routine. with temperature probe and the peripheries were kept warm using an external warmer. The arterial line, peripheral line and central line apart from their usage in fluid management also aremuch more accurate and continuous method of monitoring and help to detect fluid over lead early. Fluid loss was monitored with the urine output, blood soaked gauze and suction canisters. Cefuroxime was given to all our patients for prophylaxis and also post- operatively. Mannitol was used intra operatively when brain laxity was to be improved. Blood loss was monitored and a haematocrit values along with ABG, electrolytes were checked every POST – OPERATIVE CARE second hour intra operatively.

Patients were shifted directly to the PICU after the surgery and after stabilization the first sample was sent for evaluation of HB, ABG, and electrolytes. These investigations were repeated every 6 hours for the first 24hrs and there after every 12 hrs once for the next 24hrs. Non opioid analgesics were used for the maintenance of pain control and sedation was given if needed. 471 10th World Cleft Lip, Palate & Craniofacial Congress

Drains were placed under positive pressure and monitored. Antibiotics were generally stopped after 3 doses post operatively but neededa change and extended administration upto 3days in case of a dural tear or elevated temperatures for a couple of cases. The central line, urinary catheter and arterial line were removed between 48 -72hrs as the patient were shifted to the room A wash after 3 days and smaller dressing made the patient feel comfortable and relaxed. Staples were removed alternatively after 5 days and completely after 7 days. Follow-The Up patients were discharged between 5th to 7th post-operative days. time. Patient is followed up once after a week, a month and then after 3 months’ CHECKLIST PROTOCOL

(Table:1)

472 10th World Cleft Lip, Palate & Craniofacial Congress Results

A total of 6 children were included in this pilot study. The average age was 7.36yrs (range: 1yr20days-16yrs). The male: female ratio was 2:3. The average weight was 22.36kgs (range: 7.5-43kgs). The cases included Brachycephaly(3/6), Plagiocephaly(1/6) and hypertelorism(2/5). The pre- operative work-up due to adherence of the protocol helped us find two issues in the six patients, these included presence of latex allergy in one patient and Factor IX deficiency in one patient. The surgery done included Box-osteotomy in 2 and Fronto-orbital advancement in 4 patients. The average duration of the surgery was 6.4hours (range: 5-8 hours). The average PICU stay was 3.8 days (range: 2-6days). The average hospital stay was 10.8days (range6-22days). The outcomeDiscussion in all the five cases was good and all children were discharged home. Complete 8

Most of the brain growth occurs within the 1st year of life. 13 ossification takes place when surgery is performed before 1yr of age. Results of surgeries done earlier in life are more appreciable than those who present late. One of the most common reasons for such surgeries has been cosmesis.A surgical correction for someone with average intellect and4 relatively normal behaviour greatly improves quality of life and employment. Cranial vault osteotomies along with facial advancements have for long been the mainstay in treating children with raised ICP. Cerebral compression is one of the major indications for a synostosis correction. Other factors2 like ophthalmicThe examination and respiratory of the disorders shape, size, also position play a roleand fullnessin case selection. of the fontanelles and the cephalic index are very important. The symmetry of the eyes, position of the nose and displacement of the ears should also be noted. A complete examination of the hands, feet, cervical spine, airway and cardiac anomaly is done especially in syndromic cases.

A 3D CT along with a 3D reconstructed model gives additional information9 in cases of hypertelorism, severe asymmetries and complex craniosynostosis. Nasal swab is recommended by some as for pre- operative antibiotic cover.

Blood volume is always estimated and blood should be reserved based11 on the estimated amount of blood loss. Usually 3 units of PRBC are reserved. Intra-operatively the ETCO2 is monitored carefully as the brain is very sensitive to CO2. The normal ETCO2 is around 33mm of Hg, an increase of which473 10th World Cleft Lip, Palate & Craniofacial Congress indicates cerebral edema and a drop many lead to cerebral hypo perfusion. A urine output of 0.5-1ml/kg/hr indicates a good output. Neuro muscular monitoringCore temperaturehelps, to monitor monitoring, recovery done form with muscle temperature relaxants. probe, is always essential as these are long surgeries in an air-conditioned environment. The maintainperipheries the are temperature. kept warm using an external warmer. An open cranium presents a large area for heat loss and10 warm IV fluids are used along with the warmers to

A part from all these surgical handling plays an important role. Excessive pressure on any vital structures should be avoided, especially the eyeballs which causes bradycardia. The dead spaces and the frontal sinus opened during the surgery should be packed with the3 pericranium as they are potential areas of infection from the respiratory tract. Investigations are repeated every 6 hours for the first 24hrs and then only the ABG and electrolytes are done 12hrly for the next 24hrs. A low Hbalong with elevated temperatures and Tachycardia prompts for blood transfusion. An elevated lactate levels are expected immediate post-operative due to the temperature variation during the surgery. Sodium levels need to be monitored carefully and compensated in case of discrepancies. Non opioid analgesics are used for the maintenance of pain control and the child may or may not need sedation for the first 24hrs. Urine output monitoring for the first 24- 48 hours is mandatory, a value of 0.5 to 1 ml/kg/hr is normal. Drain is placed under positive pressure and removed after 48 hrs or ifConclusion: it is less than 30ml.

A strict protocol helps in working up cases thoroughly not only prior to the surgery but also after the surgery. Pre-operative work-up helped in diagnosing significant co-morbid conditions and as a result appropriate measures could be taken and thus problems being avoided. A good post-operative team approach helps in early identification of life-threatening complications and thus prevent majorReferences: morbidity and mortality. 01. Amelie Von Saint Andre-Von Arnim, Mda, Reid Farris, Mda, Joan S. Roberts, Mdb, OferYanay, Mda, Thomas V. Brogan, Mda, Jerry J. Zimmerman, MD, Phd, Fccmc,* Common Endocrine Issues In The Pediatric Intensive Care Unit Crit Care Clin 29 (2013) 335–358

474 10th World Cleft Lip, Palate & Craniofacial Congress 02. Burokas L Craniosynostosis: Caring For Infants And Their Families. Crit Care Nurse. 2013 Aug;33(4):39-50; Quiz 51. Doi: 10.4037/Ccn2013678. 03. David J. David, Rodney J Cooter Craniofacial Infections in 10 Years of Transcranial Surgery PlastReconstr Surg. 1987 Aug; 80(2):213-25. 04. David Matthews, C.B.E., M.D., F.R.C.S Craniofacial surgery—Indications, assessment and complications British Journal of Plastic Surgery Volume 32, Issue 2, April 1979, Pages 96-105 05. Hemangi K Anesthesia Considerations for Craniofacial Surgery Short Notes in Plastic Surgery June 28, 2012 06. Joseph E Losee, A. Corde Mason Deformational Plagiocephaly: Diagnosis, Prevention and Treatment. Clinics in Plastic Surgery 32 (2005) 53-64 07. Levine JP, Stelnicki E, Weiner HL, Bradley JP, Mccarthy JG. Hyponatremia In The Postoperative Craniofacial Pediatric Patient Population: A Connection To Cerebral Salt Wasting Syndrome And Management Of The Disorder. PlastReconstr Surg. 2001 Nov;108 (6):1501-8. 08. Mccarthy, Joseph G. M.D.; Glasberg, Scot B. M.D.; Cutting, Court B. M.D.; Epstein, Fred J. M.D.; Grayson, Barry H. D.D.S.; Ruff, Gregg M.D.; Thorne, Charles H. M.D.; Wisoff, Jeffrey M.D.; Zide, Barry M. M.D. Twenty-Year Experience With Early Surgery For Craniosynostosis: Isolated Craniofacial Synostosis-Results And Unsolved Problems. Plastic & Reconstructive Surgery: August 1995 - Volume 96 - Issue 2 09. Sailer HF, Haers PE, Zollikofer CP, Warnke T, Carls FR, Stucki P. The value of stereolithographic models for preoperative diagnosis of craniofacial deformities and planning of surgical corrections. Int J Oral Maxillofac Surg. 1998 Oct;27(5):327-33 10. Stricker PA, Fiadjoe JE. Anesthesia for craniofacial surgery in infancy. AnesthesiolClin. 2014 Mar;32(1):215-35 11. StrickerPA(1), Shaw TL, Desouza DG, Hernandez SV, Bartlett SP, Friedman DF, Sesok- Pizzini DA, Jobes DR Blood loss, replacement, and associated morbidity in infants and children undergoing craniofacial surgery. PaediatrAnaesth. 2010 May;20(5):471. 12. Van Krugten RJ, Bos AP, Offringa M, Tibboel D, Molenaar JC Postoperative Care After Craniofacial Surgery: Evaluation Of Routine Laboratory Testing. PlastReconstr Surg.1993 Mar; 91(3):429-32. 13. Whitaker LA, Bartlett SP, Schut L, Bruce D. Craniosynostosis: An Analysis Of The Timing, Treatment, And Complications In 164 Consecutive Patients. PlastReconstr Surg. 1987 Aug; 80(2):195-212.

475 10th World Cleft Lip, Palate & Craniofacial Congress

Selective osteotomy during growth for apertognathia in a Crouzon’s child – A case report

KAVITHA DEVI FINAL YEAR POST GRADUATE, RMDCH, ANNAMALAI UNIVERSITY,

GUIDED BY:PROF.DR.A.THANGAVELU,, MDS,DNB, HEAD OF DEPT, DIVISION OF ORAL & MAXILLOFACIAL SURGERY, RMDCH, ANNAMALAI UNIVERSITY

ABSTRACT:

Crouzon's syndrome (CS) is a rare autosomal dominant condition with multiple mutations of the fibroblast growth factor receptor (FGFR2) gene, which accounts for 4.8% of all cases of craniosynostosis. It is characterized by premature closure of cranial sutures, cranial deformities, midface hypoplasia, relative mandibular prognathism, hypertelorism, proptosis, strabismus and short upper lip, crowding of teeth, pseudocleft or sometimes cleft palate and other associated abnormalities. This paper presents the case of a 13-year-old male patient who presented with the complaints of inability to close the mouth and speak properly with continuous drooling of saliva for past 5 years and was diagnosed as a case of Crouzon syndrome with apertognathia. Aproblem based surgical approach was planned and surgical correction of apertognathia was done. This is to be followed by further skeletal corrections on skeletal maturity.

476 10th World Cleft Lip, Palate & Craniofacial Congress

Surgical Management of Late Reporting Pruzansky Grade III Hemifacial Microsomia

Sam Raj G1, A. Thangavelu2, S.Thiruneelakandan3, R.Murugam4, Nithin Sylesh5 1Resident, Dept of Maxillofacial Surgery,Annamalai univ,2Head, Dept of Maxillofacial Surgery,Annamalai univ,3Reader,Dept of Maxillofacial Surgery,Annamalai univ,4Lecturer, Dept of Maxillofacial Surgery,Annamalai univ, 5Resident, Dept of Maxillofacial Surgery,Annamalai univ. Abstract

Patients with obvious craniofacial deformities often seek treatment for anatomical, physiological or esthetical concerns much earlier. In areas, notespecially report in in rural early areas, phase owing of life. to The misconception aim of this orpresentation lack of awareness is to present or non- a availability of treatment centers, patients with rare craniofacial deformities do successful management of a 22 year old male identified with Pruzansky Grade III Hemifacial Microsomia. The patient’s main concern was esthetics followed by restoration of physiological function. He refused for long term treatment, owing to impending marriage. Considering the patient’s requirement and clinical situation, we evolved a short term, multi-stage, surgical rehabilitative process. This included correction of occlusal cant, inverted L osteotomy to counteract the posterior open bite created, sliding midline genioplasty for managing discrepancy along the midline unit. The agenesis of the Ramus-Condyle- Body mandibular unit was corrected with large iliac graft, which was sculpted to the shape of defect and secured with plates and screw. The soft tissue defects were addressed with multisite autogenous injectable fat transfers. The patient is doing fine at the follow-up. We aim to discuss the situation, limitations, advantages and unique challenges posed by such late reporting of large scale craniofacial defects and evolve a patient centric, expectation based treatment algorithmINTRODUCTION to serve as a ready reference for such future situation.

Hemifacial microsomia is a congenital deformity in which there is a deficient amount of skeletal and soft tissues on one side of the face. It is a syndrome affecting mainly the first and second branchial arches, which include underdeveloped temperomandibualar joint, mandibular1 ramus, masticatory. muscles, ears and defects in facial nerve and muscles .HFM is the most common2 congenital craniofacial anomaly seen with the exclusion of cleft lip and palate477 10th World Cleft Lip, Palate & Craniofacial Congress

The occurrence of HFM is between 1 in 3000 and 1 in 5600 births. Males appear to be more frequently affected than females and the right side is affected more often than the left side3. Although “hemifacial” refers to one half of the face, the condition is bilateral in 31% cases, with one side being more affected than the other. In 48% of cases, the condition is part of a larger syndrome such as Goldenhar syndrome4-7. Here we discuss a case of Hemifacial Microsomia with treatment.significant deformity with an OMENS scale8 of O2M3E3N0S2 and Pruzanskys classification9 of Grade 3 and the dilemma we faced in planning and staging his CASE REPORT

A 22 year old male patient presented to the department of oral and maxillofacial surgery with the complaint of deformity of the left side of the face since birth. He gives an history of progressive nature of the deformity with growth. He also complains of associtated partial hearing loss on the left side. His prenatal history reveals that his mother sustained a fall during her first trimester.His parents and brother are apparently normal with no other family members with a similar condition.e patient was moderately built moderately On general examination th nourished and well oriented to time place and person. There was obvious facial asymmetry on left side which was short and flattened with hollowing of the cheek. Bilateral auricles absent. The remanant lobules placed anteriorly and inferiorly on both sides. On the right sideed were slightly two higher remanant on the lobules. left side. There The is deviation of the tip of the nose and the chin to the left side. The ala of the nose and the corner of the mouth are plac corner of the mouth is deviated to the left side.The patient had a normal vision with harmonious movement of eye balls in all angles. On palpation the left masseter and temporalis was deficient. The right condyle was palpable with normal lateral and protrusive movements. The duringleft condyle mouth was opening. not palpable. Patient had adequate mouth opening with an interincisal distance of 33mm. there is notable deviation towards the left side

478 10th World Cleft Lip, Palate & Craniofacial Congress

He had a normal dentition upto the second molars in all quadrants with no significant dental caries or periodontal disease. The patient had bilateral posterior open bite and cross bite. The patient was subjected to investigations-orthopantamogram, frontal cephalogram, PNS without cone and 3D CT Scan of the face. Radiological examination revealed absence of ramus and condyle on the left side with significant left sided maxillary hypoplasia and a severely flattened and rudimentary zygomatic arch.

Based on the clinical and radiologic findings patient was diagnosed as hemifacial microsomia of the left side with OMENS scale of O2M3E3N0S3 and Pruzanskys grading of grade III - the most severe form of hemifacial microsomia. Considering the patient’s requirement and clinical situation, we evolved a short term, multi-stage, surgical rehabilitative process. Stage I was planned to exclusively correct the skeletal deformity. This was done by a lefort I osteotomy and superior impaction of the uninvolved right side by 8 mm to acheive cant correction, followed by an inverted L ramus osteotomy on the right side to counteract the posterior open bite created. A midline sliding genioplasty was performed to address the chin deviation. The agenesis of the Ramus-Condyle- Body mandibular unit was corrected with large iliac graft, which was sculpted to the shape of defect and secured with plates and screw.The patient was placed in IMF was two weeks to facilitate adaption of the musculature to the new position. 479 10th World Cleft Lip, Palate & Craniofacial Congress

on normal side Incision marking and Lefort I osteotomy with 8mm superior repositioning

Cant correction achieved

Genioplasty and Maxillary augmentation with iliac graft

Harvesting of Illiac bone graft and reshaping to augment the left Maxilla 480 and Mandible. 10th World Cleft Lip, Palate & Craniofacial Congress

Stage II surgery was done to address the soft tissue deficiency by autologus fat grafting of the malar and post auricular region. The patient was given a reasonable improvement in the facial profile and symmetry and an overall improvement in the occlusal function.

DISCUSSION Autogenous fat grafting Post op profile

Hemifacial microsomia (HFM) is a craniofacial malformation resulting in a variable degree of hypoplasia of the structures within the first and second branchial arches1. There are many theories given for the HFM based on embryologic, clinical and laboratory studies, but the exact etiology is yet not known, but pathogenically it is heterogeneous. The laboratory studies suggest that there are certain factors that are responsible for the clinical presentation of HFM, which can be due to early loss of neural crest cells10. The term was first used by Gorlin and Pindborg in 1964, l although the first recorded cases may have been those of Canton’ in 1861 and Von Arlt in 1881. Many terms have been used for this malformation, indicating the wide spectrum of anomalies observed and emphasized by authors from varied disciplines. The malformation has been known as hemifacial microsomia,sia, craniofacial microsomia, oculoariculovertebral dysplasia, golden har syndrome, first and second branchial arch syndrome, lateral facial dyspla unilateral otomandibular dysostosis, and facioauriculovertebml malformation complex11. The surgical treatment of facial asymmetry in Hemifacial microsomia can be dealt with by Distraction Osteogenesis, Orthognathic surgery, free tissue transfer, and or lipofilling. The application of Mirror imaging and rapid prototyping technique has been successfully employed by some surgeons to achieve optimal results11. Whenever required Lipofilling can be considered to augment the soft tissue defects within certain limits12. 481 10th World Cleft Lip, Palate & Craniofacial Congress

In our case the patient reported very late much after skeletal maturity and his social and financial circumstances warranted a cost effective and early solution. . He refused for long term treatment, owing to distance from his native town(over 500Kms) and other personal reasons. Considering the patient’s requirement and clinical situation, we evolved a short term, multi-stage, surgical rehabilitative process. This included correction of occlusal cant, inverted L osteotomy to counteract the posterior open bite created, sliding midline genioplasty for managing discrepancy along the midline unit. The agenesis of the Ramus- Condyle- Body mandibular unit was corrected with large iliac graft, which was sculpted to the shape of defect and securedp. with plates and screw. The soft tissue defects were addressed with multisite autogenous injectable fat transfers. The patientCONCLUSION is doing fine at the follow-u The treatment

of patients with hemifacial microsomia is complicated and long-lasting, therefore it requires co-operation between many specialists from medical and dental fields. In designing a course of treatment, the dental toocclusion address mustthe multi be considered in conjunction with the underlying. skeletal condition3. It is very important to integrate the timing of surgical intervention2 BIBLIOGRAPHY ple tissue deficiencies in Hemifacial microsomia 01. Rashmi Saddiwal, Manjula Hebbale, Shams Ul Nisa, Vikrant Sane. Hemifacial Microsomia – A Case Report and Review of Literature. International Journal of Advanced Health Sciences • Vol 1 Issue 8 • December 2014 02. B.Neirzwicki, C.Meyer, Hemifacial Microsomia – review of Literature and case report.Int. J. Oral Maxillofac. Surg. 2005;34(Supplement 1) 03. Maria Mielnik-Blaszczak, Katarzyna Olszewska.Hemifacial Microsomia – Review of literature. Dent. Med. Probl. 2011, 48, 1, 80–85 04. Monahan R., Seder K., Patel P., Alder M., Grud S., O’Gara M.: Hemifacial microsomia: etiology, diagnosis and treatment. J. Am. Dent. Assoc. 2001, 132, 1402– 1408. 05. Kapur R., Sheikh S., Jindal S., Kulkarni S.: Hemifacial microsomia: a case report. J. Indian Soc. Pedod. Prevent.Dent. 2008, 26, 34–40. 06. Wang R.R., Andres C.J.: Hemifacial microsomia and treatment options for auricular replacement: a review of the literature. J. Prosthet. Dent. 1999, 82, 197–204. 07. S.L., Haan E., Slee J., Goldblatt J.: Familial hemifacial microsomia due to autosomal dominant inheritance. Aust. Dent. J. 1994, 39, 287–291.

482 10th World Cleft Lip, Palate & Craniofacial Congress 08. Vento et al. The O.M.E.N.S. Classification of Hemifacial Microsomia. Paper presented at the 45th meeting of the American cleft palate association,Williamsburg, Virginia, April 1988. 09. Jose Rolando Prada Madrid, Giovanni Montealegre, and Viviana Gomez.. A New Classification Based on the Kaban’s Modification for Surgical Management of Craniofacial Microsomia. Craniomaxillofac Trauma Reconstruction 2010;3:1–7. 10. Madi M, Shetty SR, Babu SG, Achalli S. Hemifacial microsomia: A case report an d overview. Cukurova Med J 2014;39:625-35. 11. Jeffrey C. Posnick, Surgical correction of mandibular hypoplasia in hemifacial microsomia: A personal perspective. J Oral Maxillofac Surg 56:639-650, 1998. 12. Libin Zhou, Lisheng He, Hongtao Shang, Guicai Liu, Jinlong Zhao, Yanpu Liu. Correction of hemifacial microsomia with the help of mirror imaging and a rapid prototyping technique: case report. Br J Oral Maxillofac Surg 47 (2009) 486–488 13. Hoehnke C, Eder M, Papadopulos NA, et al. Minimal invasive reconstructio of posttraumatic hemi facial atrophy by 3-D computer-assisted lipofilling. J Plast Reconstr Aesthetic Surg 2007;60:1138–44.

483 10th World Cleft Lip, Palate & Craniofacial Congress

TRANSMOGRIFICATION - AN ONGOING CASE OF HEMIFACIAL HYPERTROPHY

YUNUS AMIN POST GRADUATE, DEPARTMENT OF OTHODONTICS AND DENTOFACIAL ORTHOPEDICS, MEENAKSHIAMMAL DENTAL COLLEGE AND HOSPITAL, ALAPAKKAM MAIN ROAD, MADURAVOYAL, CHENNAI – 95, INDIA,

INTRODUCTION :

In general, children with CRANIOFACIAL DEFORMITY should be evaluated for other associated physical anomalies and developmental delays that may require treatment and/or help identify specific syndromes and causes Children who are affected with such deformities are affected by the surrounding peer pressure and psychologically. Treating such patients should be done as early as possible because of the growth that in present at young age. Meazzini et al has showed good amount of improvement in hemifacial micrsomina treated with modified functional appliance 1.Taking growth as a plus point we can reduce the severity of the craniofacial abnormality while directlyAIM : or indirectly reduces the complexity of surgery post growth completion

MATERIAL To present AND METHODS a ongoing :case report of a child with hemifacial hypertrophy.

A young child of age 14yrs is undergoing treatment for the craniofacial abnormality for the past 4yrs using a HYBRID MYOFUNCTIONAL APPLIANCE. Hypertrophy was present on the right side of the face with chin deviated towardsRESULTS the : opposite side because of the excessive growth on right side.

Good amount of changes has been achieved using hybrid appliance. Deformations in archform of the patient was corrected considerably. Chin deviation showed moderate changes which shifted from left to right towards the centre of the facial midline indicating that growth has occurred on the left side leading to partial correction of the asymmetry. 484 10th World Cleft Lip, Palate & Craniofacial Congress CONCLUSION :

Achieving a appreciable balance in the alvelolar remodelling and thereby causing change in skeletal parameters using myofunctional appliance can considerably reduce the deformity which will reduce the complexity of orthognathicREFERENCES surgery : performed at later age.

1.M.C. Meazzini, A. Caprioglio, G. Garattini, L. Lenatti,C.E. Poggio. Hemandibular Hypoplasia Successfully Treated With Functional Appliances: Is It Truly Hemifacial Microsomia? Cleft Palate–Craniofacial Journal, 2008, Vol. 45 No. 1

485 10th World Cleft Lip, Palate & Craniofacial Congress

Efficacy and success of structured collagen as an Interpositional graft in the treatment Temperomandibular joint

Sathish Vashista NGO, Bangalore, Karnataka, India

Purpose:

A variety of materials have been used to prevent recurrence after interposition arthroplasty in the treatment of temporomandibular (TM) joint ankylosis. Purpose of this study is to assess the efficacy and success of structured collagenMaterials as and an interpositionalMethods: graft in the treatment of TM Joint ankylosis.

Using prospective study design, the investigators enrolled a total of 30 patients with TM Joint ankylosis. Structured collagen membrane was used as an interpositional graft. Measurement of mouth opening, 3D CT scans and radiographs were done for all patients pre-operatively and post-operatively interpositional graft material. to adequately assess the efficacy of structured collagen membrane as an Results:

Sample was composed of 30 patients whose age ranged between 1 to 35 years and the follow-up period ranged from 12 to 36 months. Average mouth opening was increased from 6.39 mm pre-operatively to 40.25 mm post operatively this was maintained for up to 3 years postoperatively. CT scans segment and gleniod fossa in any of the patients. showed no physical evidence of bone formation between proximal condylar

486 10th World Cleft Lip, Palate & Craniofacial Congress Conclusion:

The results of the present study conclude that structured collagen can be effectively used as an interpostional graft. Post-operative physiotherapy also playsKey words: major role in preventing re-ankylosis.

TM Joint Ankylosis; Interpositional Arthroplasty; Structured Collagen membrane;

487 10th World Cleft Lip, Palate & Craniofacial Congress

Topic – Post Ankylotic Deformity Correction – Report of 2 Cases with Different Surgical Approach

Ravi Kumar M P, David P Tauro Vikram Hospital Private Limited , No.46, Vivekananda Road, Yadavagiri, Mysore, Karnataka (State), India

Abstract

TMJ ankylosis with secondary dentofacial deformities in adult patients is a severely disfiguring condition and surgical treatment of this disease remains a great clinical challenge. Treatment goals are to restore the joint function, to improve facial appearances and to correct malocclusion, as well as to re- establishing harmony among them. Currently, various surgical techniques, such as arthroplasty with or without interpositional material, orthognathic surgery, distraction osteogenesis, autologous bone or bone replacement materials graft have been described in the literature. In most cases these techniques should be used in combination to achieve satisfactory outcomes. The biggest difficulty for most clinicians is to determine the proper sequence of these procedures because no uniform treatment protocol has been established. We encountered 2 cases in the last 6 years which had different clinical and functional problems. Both cases were treated differently by using DO and Orthognathic Surgery. Comprehensive management was planned along with the use of orthodontic treatment in order to obtain satisfactory results with respect with occlusion and facial esthetics. In Case 1, both DO and orthognathic surgical technique was used. In Case 2, reankylosis release was done followed by orthognathic surgical procedure. Patient has been on follow up for nearly 3 years post-surgical phase till date. We would like to present and discuss the details of the management and the result that we obtained at the end of treatment. Management of these cases required tailored made approach in order to cater the patients’ needs and also the functional problems.

488 10th World Cleft Lip, Palate & Craniofacial Congress

Current approaches in the orthodontic management of cleft lip and palate

Dr.R.K.Sathishkumar IInd year PG Guided by :Dr.Rajasigamani Professor & HOD, Department of orthodontics, Rajah muthiah Dental college, Chidambaram, India

ABSTRACT Cleft

lip and palate are considered to be one of the most common birth defects involving craniofacial suture and again gives an opportunity for all our finest hands to work together. Individuals with cleft lip and palate are ideally treated in a multidisciplinary approach where the role of orthodontists and surgeons is extensive, starting from birth and continuing through infancy, mixed dentition period, till adoloscence and even beyond. The various specialities involved are shrewdly focusing on the functional balance and structural stability whereas the goal of an orthodontist is aimed at the third factor to create an esthetic harmony in cleft lip and palate patients. The inevitable truth is that the cleft lip and palate patients are finally handed over to the orthodontic franertity to create an esthetic profile and pleasing smile. A mutual cooperation is expected at the very beginning to go hand in paperhand for highlights a successful th treatment outcome. The role of an orthodontist in timing and sequence of treatment is important in terms of overall management. This e update of evolving concepts and recent advances in the orthodonticIntroduction techniques in treating a cleft lip and palate patients.

The management of patients with cleft lip and cleft palate requires prolonged orthodontic and surgical treatment and an interdisciplinary approach in providing them with optimal esthetics,function and stability.1 Sequencing and timing of orthodontic/orthopedic and surgical treatment in infancy, early mixed dentition, early permanent dentition and after the completion of facial growth will be discussed. 489 10th World Cleft Lip, Palate & Craniofacial Congress

The most promising diagnostic tool by which congenital and developmental anomalies are more clearly detected before birth is with ultrasonography and its refinements. Once a prenatal diagnosis of a craniofacial anomaly is made, counseling is provided to parents before delivery by the members of the cleft palate-craniofacial team. The team’s pediatric dentist or orthodontist may discuss with parents the dentoskeletal manifestations associated with clefts and offer an overview of the dentalNeonatal care period: required 2 weeks at the toappropriate 6 months developmental stage. The goa

l of neonatal maxillary orthodontics was to eliminate the need for orthodontic treatment after the dentition erupted and did not include primary bone grafting. It was predicated on active and passive ideal alignment of the cleft segments so that the teeth would erupt normally in their respective aligned segments. Even treatment begins at an early stage, It does’t eliminate the later orthodontic treatment. Modifications of appliances and primary bone grafting continued, but by the 1990s nasoalveolar molding (NAM) was gaining attraction with the profession and the intervention was typically performed by the orthodontist serving on the cleft palate and craniofacial team. With the use of an intraoral molding appliance and extraoral taping, the alveolar ridges are approximated within 1 to 2 mm of each other. The addition of nasal stents achieves lengthening of the columella in bilateral cleft patients. Ultimately, NAM prepares the infant for a 1-stage primary lip-nose repair between 3 and 5 months of age combined withDeciduous gingivoperiosteoplasty dentition: 2 to 7 yearsto close the alveolar defect.

Although treatment in the deciduous dentition was popular in the 1930s and 1940s, most interventions were duplicated in the mixed dentition when the permanent teeth erupted. It therefore became prudent to delay most orthodontic interventions such as expansion and alignment of the incisors until the mixed dentition. With the development of the phonetic component of speech and language, articulation in the toddler is challenged by structural impairment. Lack of velopharyngeal competency and palatal mobility of the repaired cleft palate may result in adaptations by the child to reduce the hypernasality of speech and the development of glottal stops. To reduce the nasal air escape, a surgical pharyngeal flap may be suggested in which the soft palate is connected permanently to the superior anterior pharyngeal wall. Lip or nose revision surgeries are occasionally recommended for children before they490 start grade school. 10th World Cleft Lip, Palate & Craniofacial Congress Mixed dentition: 7 to 12 years

One major advantage for an orthodontist is having a successful alveolar bone graft in the cleft site into which the permanent canine erupts and brings additional alveolar bone. This requires close collaboration by the surgeon and orthodontist. Presurgical maxillary expansion is typically indicated; after the alveolar bone graft, the teeth adjacent to the cleft may be repositioned and moved into the sites without compromising their periodontal health. The controversies surrounding primary alveolar bone grafting resulted in some skepticism when secondary alveolar bone grafting was introduced in the oral surgery literature by Boyne and Sands34 and adopted by cleft palate teams. It took 15 years before a landmark publication from the team in Oslo, Norway, provided strong evidence for the benefits of autologous secondary bone grafting using cancellous bone fromthe iliac crest; this remains the gold standard in the 21st century. As the permanent teeth erupt, an association between cleft type and dental abnormalities of size, shape, and number becomes apparent, with the maxillary lateral incisor being the most vulnerable. The prevalence of “hypodontia” has been reported to increase with the severity of the cleft in both the deciduous and permanent dentitions. The circummaxillary sutures respond during the mixed dentition by modification and redirection of the nasomaxillary complex with protraction headgear. This became popular with orthodontists in the 1970s to correct the midface deficiency after its introduction by Delaire et al, who applied the protraction force to prevent relapse after surgical maxillary advancement. Children with unilateral CLP typically have a midface deficiency, and their response to the protraction facemask has been studied for skeletal and dental outcomes after treatment. More recently, the introduction of bone- borne anchorage with miniplates and miniscrews provides an orthopedic force that Permanent can be applied dentition: to the 12 maxilla.years to adulthood

Once the growth gets terminated, skeletal correction without surgical technique is not possible and surgical corrections of the skeletal and nasolabial soft tissue revisions are planned. Treatment of orofacial clefts in adolescent patients is difficult to correct.This special group of patients has already experienced multiple surgical procedures and nose and lip revisions. Speech may have deteriorated with velopharyngeal incompetency and the quality of life and social interactions impacted. After definitive jaw surgeries and growth stabilizes, dental implants are an option for restoration of missing maxillary incisors at the cleft site with 491 10th World Cleft Lip, Palate & Craniofacial Congress high success rates if sufficient bone is provided by autogenous grafts and with implant lengths of at least 13 mm in the grafted alveolar sites.42 More prospective clinical studies of dental implants in cleft sites are needed to improve the quality of evidence on their success rates. Mesializing the maxillary canine into the lateral incisor space (canine substitution) is a common alternative to implants,TECHNICAL yielding ADVANCES similar IN esthetic THE 21ST results.44 CENTURY45,46,47 Cone-beam computed tomography

Cone-beam computed tomography (CBCT) technology is now available in orthodontic educational and private practice settings. This 3-dimensional imaging of anatomic structures is especially valuable in evaluating cleft palate and other craniofacial skeletal anomalies. Various journals reported on the use of CBCT for the clinical assessment ofvalveolar bone grafting, demonstrating vertical and buccopalatal width measurements of the bone bridge. W€ortche et al used CBCT to evaluate axial and coronal sections through the cleft defect before alveolar bone grafting. Using volume-rendering software to analyze CBCT images, Oberoi et al47 objectively measured the amount of bone at the cleftDistraction site before osteogenesis: and after alveolar bone grafting.

Recently, the induction of new bone formation and soft tissue migration in the area has gained considerable attention. This aim can be achieved by methods such as distraction osteogenesis. The usefulness of distraction osteogenesis in both endochondral and membranous bones is well demonstrated. The main superiority of distraction osteogenesis is the lengthening of the soft tissue over the bone. The application of distraction osteogenesis in infants to advance the severely underdeveloped mandible has improved the airway and feeding, eliminating the long-term need for a tracheostomy or feeding tube. Orthodontists have played an integral role in collaborations to develop craniofacial applications of distraction osteogenesis in the mandible and in surgical maxillary advancement for severe cleft maxillary hypoplasia. The distraction osteogenesis technique gradually protracts the surgically mobilized maxilla by stretching the scarred palatal tissues in small increments and allowing adaptation of the soft tissues including the velopharyngeal mechanism. The technique of rigid external distraction initially involves cementation of an intraoral tooth-borne splint in the maxilla with extraoral extensions (hooks) for traction. Contemporary bone-anchored methods are becoming increasingly popular with preadapted miniplates attached to the anterior surface of the 492 10th World Cleft Lip, Palate & Craniofacial Congress

maxilla beside the piriform aperture for traction. The use of internal distractors in the cleft maxilla has the added benefit of long consolidation periods. More recent applications of the principles of distraction osteogenesis are directed specifically at closing or reducing the size of large alveolar clefts. Alveolar bone transport has been used to move osteotomized dentoalveolar posterior segments anteriorly, in both bilateral and unilateral cleft patients. Vertically directed distraction of an edentulous bone segment on a previously grafted site can improve the esthetic outcome resulting from dental Intra-oral tooth borne distractor implant placement. Temporary anchorage devices or miniscrews

Temporary anchorage devices or miniscrews allow the orthodontist to move selected teeth without causing unwanted reactive movements of adjacent teeth when inserted into strategic areas on the maxilla or mandible. Their small simplesize (most implantation, commonly lessimmediate than 2.5 loading, mm in diameter,stability, andand 4-10 easy mm explantation. in length), mechanical retention in cortical bone, and lack of osseointegration allow for

Miniscrews commonly used in orthodontic practices have multiple applications in patients with clefts and other craniofacial anomalies. The finding of various studies correlating the findings in patients treated with Le Fort I internal distraction for severe cleft maxillary deficiency with internal tooth borne distractor and both the procedures shows better dental occlusion, less relapse and better speech results. Tooth borne distraction has several advantages compared to bone anchored distractors. These include: a. Ease of application b. Less morbidity such as cheek and lip ulcers seen with bone anchored appliances c. Removal of appliance at the end of consolidation period is very simple and can be done without anaesthesia d. Economical e.The Lessprocedure chances however of infection does since have there a few are disadvantages no fixation screws such as: a. Vector control unidirectional only b. Need for more diligent oral hygiene maintenance 493 10th World Cleft Lip, Palate & Craniofacial Congress c.The Activationprocedure atis homenot without being difficult complications. These include:

(i) Root damage of permanent teeth adjacent to the osteotomy site (ii) Anterior open bite due to the segment moving upwards (iii) Bur breakage during surgery (iv) Appliance fracture (v) Appliance dislodgement Tooth borne segmental distraction of the anterior maxilla can thus be considered as a viable treatment option for the management of patients with cleft lip and palate. There was an opening of the mandibular plane, anteinclination of the palatal plane and increase in lower anterior facial height. Extrusion of the maxillary anterior and posterior segments was also observed5. TADs can support the mandibular dentition while intermaxillary elastics are usedCONCLUSION to protract the maxilla.

The successful management of a patient with cleft lip and palate requires careful coordination of all members of the Cleft Palate Team. Introduction of nasoalveolar molding has significantly changed the outcome of cleft treatment. The shape, form and nasal aesthetics of patients with clefts are significantly better in those who have had the benefits of NAM and . The recent advances in the management of cleft lip and palate has been evolving since 1915 and treatment outcome of cleft lip and palate with advancing clinical techniques in each speciality constantly will be improved to enable the clinician to provide the best possible care while striving to reach the goal of excellent facial aesthetics inREFERENCES: patients born with clefts. 01. Eby JD. Maximum improvements in congenital orofacial clefts. Am J Orthod 1956;42:867- 72. 02. Kiskadden WS, Tholen EF. Primary and secondary repair of cleft lip. Int J Orthod Oral Surg Radiogr 1932;18:863-73. 03. Davis AL. Cleft lip and palatal surgical correction. Am J Orthod Oral Surg 1932;18:282- 90.

494 10th World Cleft Lip, Palate & Craniofacial Congress 04. Millard DR Jr. Uranoplasty by maxillary compression, Cleft craft: the evolution of its surgery. Volume III: alveolar and palatal deformities. Boston: Little, Brown and Company; 1980. p. 223-30. 05. Ivy RH. Review of recent literature on cleft lip and palate surgery. Am J Orthod Oral Surg 1937;23:844-9. 06. H€antzschel K. The eugenic significance of congenital clefts of the lip, jaw and palate [German] [dissertation]. Leipzig, Germany: Universit€at Leipzig; 1935. 07. Pena W, Vargervik K, Sharma A, Oberoi S. The role of endosseous implants in the management of alveolar clefts. Pediatr Dent 2009; 37:329-33. 08. Wermker K, Jung S, Joos U, Kleinheinz J. Dental implants in cleft lip, alveolus, and palate patients: a systematic review. Int J Oral Maxillofac Implants 2014;29:384-90. 09. Oosterkamp BC, Dijkstra PU, Remmelink HJ, van Oort RP, Sandham A. Orthodontic space closure versus prosthetic replacement of missing upper lateral incisors in patients with bilateral cleft lip and palate. Cleft Palate Craniofac J 2010;47:591-6. 10. Hamada Y, Kondoh T, Noguchi K, Iino M, Isono H, Ishii H, et al. Application of limited cone beam computed tomography to clinical assessment of alveolar bone grafting: a preliminary report. Cleft Palate Craniofac J 2005;42:128-37. 11. W€ortche R, Hassfeld S, Lux CJ, M€ussig E, Hensley FW, Krempien R, et al. Clinical application of cone beam digital volume tomography in children with cleft lip and palate. Dentomaxillofac Radiol 2006; 35:88-94. 12. Oberoi S, Chigurupati R, Gill P, Hoffman WY, Vargervik K. Volumetric assessment of secondary alveolar bone grafting using cone beam computed tomography. Cleft Palate Craniofac J 2009;46:503-11. 13. Mulliken JB, Benacerraf BR. Prenatal diagnosis of cleft lip: what the sonologist needs to tell the surgeon. J Ultrasound Med 2001;20:1159-64. 14. Yen, 201173- Provide skeletal anchorage during orthopedic maxillary protraction. 15. Ward JA, Vig KW, Firestone AR, Mercado A, da Fonseca M, Johnston W. Oral health- related quality of life in children with oro-facial clefts. Cleft Palate Craniofac J 2013;50:174- 81. 16. Grayson BH, Cutting C, Wood R. Preoperative columella lengthening in bilateral cleft lip and palate. Plast Reconstr Surg. 1993;7:1422-3.

495 10th World Cleft Lip, Palate & Craniofacial Congress 17. Sischo L, Broder HL, Phillips C Coping With Cleft: A Conceptual Framework of Caregiver Responses to Nasoalveolar Molding. Cleft Palate Craniofac J. 2014 Sep 18. Rubin MS, Clouston S, Ahmed MM, M Lowe K, Shetye PR, Broder HL, Warren SM, Grayson BH. Assessment of presurgical clefts and predicted surgical outcome in patients treated with and without nasoalveolar molding. J Craniofac Surg. 2015 Jan;1:71-5 19. Maull DJ, Grayson BH, Cutting CB, Brecht LL, Bookstein FL, Khorrambadi D, et al. Long-term effects of nasoalveolar molding on three-dimensional nasal shape in unilateral clefts. Cleft Palate Craniofac J. 1999;5:391-7. 20. Cutting C, Grayson B, Brecht L, Santiago P, Wood R, Kwon S. Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair. Plast Reconstr Surg. 1998;3:630-9. 21. Pfeifer TM, Grayson BH, Cutting CB. Nasoalveolar molding and gingivoperiosteoplasty versus alveolar bone graft: an outcome analysis of costs in the treatment of unilateral cleft alveolus. Cleft Palate Craniofac J. 2002;1:26-9.

496 10th World Cleft Lip, Palate & Craniofacial Congress

Evaluation of variation in dimensions of sella turcica in patients with cleft lip and palate as compared to Class I controls: A pilot study.

Himija Karia Orthodontics, Sr. Lecturer VSPM Dental College, Nagpur, India

INTRODUCTION:

The cleft of the lip and palate is the most common congenital anomaly affecting the cranio-facial region. The etiology of CLP is said to be multi- factorial having both genetic and environmental causes. Clefts may be localized defects or may be associated with widespread structural changes in other parts of the skull. Several studies have shown that the growth and displacement of the maxilla is directly linked to the growth and angulation of the cranial base. Hence it may be assumed that any anomaly in the cranial base may have impact on the growth as well as spatial position of the naso-maxillary complex. Sella turcica is an important anatomical landmark in the base of the skull. An anatomically normal sella turcica lies on the body of sphenoid bone; bounded anteriorly by tuberculum sellae and posteriorly by dorsum sellae. Two anterior and two posterior clinoid processes project over the pituitary fossa. During embryological development, sella turcica area is the key point for the migration of the neural crest cells to the fronto nasal and maxillary developmental fields. Hence it may be assumed that abnormalities in the size and shape of sella turcica may be directly or indirectly linked to the congenital deformities of the naso-maxillary complex such as CLP. Numerous studies have reported aberration in the size of sella turcica in relation to genetic disorders. Nielson et al found morphological deviations and malformations in the walls of the sella turcica in subjects with cleft lip and palate. Alkofied conducted a cephalometric study and reported that 63.2% subjects with CLP had an abnormal shape of the sella turcica. Therefore it may be assumed that there might be a positive correlation between the morphology of the sella turcica and CLP. Hence the purpose497 of 10th World Cleft Lip, Palate & Craniofacial Congress this study was to measure the length, width, depth and angulation of the sella turcicaMATERIALS in patients AND withMETHOD: CLP and to compare them with subjects without CLP.

A case control study was carried out at Sharad Pawar dental college Sawangi, Wardha. Ethical clearance was obtained from the institutional ethical committee and none of the subject was exposed for the purpose of study. Pre treatment DVT scans of 60 cleft lip and palate patients and 40 Angle Class I individuals who met our inclusion criteria were selected from the records available at the department. The Digital Volume Tomographic images for each subject was obtained using Phillips Allura Xper FD20 3D RA, unit with exposure parameters of 80 kVp, 10 mA and 4-5 sec. The images were obtained with the patients positioned supine and the head positioned such that the Frankfort horizontal (FH) plane was perpendicular to the floor. The acquired three dimensional data was converted to DICOM format and the multiplanar reconstruction (MPR) images were sectioned at the computer work station. These sections were then evaluated using Intruis Suite R2 software. Following measurements were carried1. LENGTH out: OF SELLA AT OPENING: 2. WIDTH OF THE SELLA: 3. DEPTH OF THE SELLA : 4. ANGULATION OF SELLA TURCICA: STATISTICAL ANALYSIS:

The data was analyzed using SPSS version 16.0 software. Descriptive statistics was carried out to calculate mean & standard deviation. Unpaired T test was applied to draw comparison between readings obtained from the cleft group and the control group. Chi square test was used to check any correlation of the dimensions of the sella with age or sex. The confidence interval was set atRESULT: 95% ( p value < 0.05).

The statistical analysis revealed that the length, width and depth of the sella turcica were found to be significantly reduced (p < 0.05) in the Cleft group as compared to the control group. However the angulation of the sella turcica was found to be significantly increased in the cleft subject as compared to the subjects498 from the control group. 10th World Cleft Lip, Palate & Craniofacial Congress

When comparison was drawn between males and females in the control as well as cleft group; all linear measurements showed slightly higher value for male subjects in both the groups. However, no significant difference was found in the dimensions of the sella turcica between males and females. Only the angulation of the sella was slightly increased in the females of the cleft group as compared to the males of cleft group. These findings indicate that the development of the sella turcica, pituitary gland and the naso-maxillary complex may be interlinked. Further studies in this direction may be able to establish a relationship between clefting and development of the cranial base structures such as sella turcica. The result of this study indicates that there is indeed statistically significant variation in the dimensions of the sella turcica in individuals with and without cleft. This study also paves way for further endocrinological research to determine if there is any abnormality in the pituitary function in subjects with smaller sella cavity and CLP. The findings of these studies will not only help in better understanding of the development of the cranio-facial structures at the genetic level, it can also widenCONCLUSION: the scope of management of CLP patients. 1. The dimensions of the sella turcica (length, width and depth) were found to be smaller in the cleft group as compared to the control group. 2. The angulation of the sella was greater in the cleft sample as compared to the control. 3. The angulation of sella was significantly greater in females in cleft group as compared to males. 4. All linear measurements were slightly larger in males though not statistically significant.

499 10th World Cleft Lip, Palate & Craniofacial Congress

Screw Design For Cleft Cases

Umarevathi gopalakrishnan Reader, dept of Orthodontics, Sri venkateswara Dental college and hospital, Thalambur, Chennai. Premkumar Sridhar Professor, dept of Orthodontics, Tamilnadu Government dental college and hospital, Chennai.

ABSTRACT

The study of the palatal width in cleft cases will render the fact that width between the pterygoid hamuli is greater in cleft cases than in normal patients. Consequently whenever expansion is desired in cleft cases a screw which can perform a selective expansion without expanding the already increased posterior palatal width would be desired. Such a screw will be discussed in this paper and the screw’s efficiency in causing a selective expansion is compared with that of a conventionally used expansion screw. The reason for the palatal widthINTRODUCTION increase in cleft cases is discussed.

Narrow maxillary arches have been associated with mouth breathing, digit sucking habits and cleft cases [1-6]. The imbalance in the equilibrium between the intraoral and extraoral muscles is cited as the cause in these cases. In cleft patients there is a typical collapse of the anterior segment with a normal to increased posterior width. A kind of selective expansion of the anterior segment alone is needed in these cleft palate cases [7]. Schellino et al [8] designed a Fan type Rapid maxillary expansion (RME) screw named “ragno” stating that it will work asymmetrically and produces selective expansion. The objective of this study is to evaluate the dental and skeletal effects of tooth borne Fan type RME andSUBJECTS Hyrax RME AND on METHODS craniofacial structures.

The sample of the study consisted of 12 patients who reported to the patientsDepartment in the of late Orthodontics, mixed or early Tamilnadu permanent Government dentition with Dental tapered College maxillary and Hospital with complaint of malocclusion. The criteria for case selection included arch and unilateral or bilateral cross bites requiring palatal expansion as part of500 their treatment. No consideration was given to the gender of the subject and 10th World Cleft Lip, Palate & Craniofacial Congress

patients with palatal clefts and premature synostosis of the palate were not included in the study. Midpalatal suture patency was assessed with occlusal radiographs (Fig 1a, 1b). The study was proposed at the Institutional Ethical Committee, Tamilnadu Government dental college and Hospital, and the approval obtained. The subjects were divided into two groups by Concealed randomization using opaque envelope method. Group 1 had six patients who were treated with fan type expansion screw. Group 2 had 6 patients who were treated with Hyrax expansion screw. Both the screws were adapted parallel to the occlusal plane of the upper teeth. The hinge point of the Fan RME screw was positioned in line with the distal surface of the upper first permanent molar. The anterior arms were adapted to the lingual surface of the canines and premolars. Posterior arms were bent perpendicular to the screw body and adapted to the molars. The anterior and posterior arms along with the corresponding teeth from canines to molars were enclosed in the clear acrylic material. The incisors were left free (Fig 2). primary molars. The anterior and posterior arms were adapted to the lingual The Hyrax screw was positioned parallel to the second premolars or surfaces of the canines, premolars and molars. The anterior and posterior arms along with the corresponding teeth were included in clear acrylic material. The incisors were left free. The thickness of the occlusal acrylic part was limited to the freeway space. Activation was started after 24 hrs of cementation. The appliances were activated one quarter turn twice per day, in the morning and evening. Both the groups were expanded equally for a period of 3 weeks. Suture opening was confirmed with occlusal radiographs (Fig 3a, 3b). After expansion, the screw was fixed with ligature wire and left for a week to minimize discomfort during removal. After removal of the screw, alginate impressions, photographs and radiographs were taken and findings recorded .The appliance was cemented back after taking impressions and radiographs, to be used as the retainer for a period of three months following which routine orthodontic treatment was carried out. Maxillary width and nasal width were measured on the frontal cephalogram. The maxillary width is measured between right and left jugale points (defined as the crossing of the outline of the tuberosity with that of the jugal process). Nasal width was measured between the widest points in the 501 nasal cavity. Stone models were used to measure intercanine and intermolar 10th World Cleft Lip, Palate & Craniofacial Congress width. Intercanine width was measured with a divider between the cusp tips of canines. Inter molar width was measured between the deepest points of the centralRESULTS sulci of the upper first molar teeth. Statistical analysis

The findings of the frontal radiographs and dental casts were analyzed statistically. The arithmetic mean and standard deviation between the pre and post treatment frontal cephalometric measurements of each group was analyzed using Wilcoxon signed rank test. Comparison between the groups was done using Mann Whitney U test. Differences between the pretreatment and post treatment findings are given in table I for Fan type RME (Group 1) and in table II for Hyrax (Group 2). Comparison between the two groups is given in table III. Since the data pertaining to intercanine width and intermolar width were normally distributed parametric test of significance (unpaired t test) was usedFrontal for cephalometric comparison between findings the groups (table IV).

Group 1(Fan type RME) showed significant increase for nasal cavity (NC- CN) width (P-0.026) and maxillary (JL-JR) width (P-0.026). Group 2(Hyrax) also showed significant increase for nasal cavity width (P-0.020) and maxillary width (P-0.046). When the two groups were compared using student‘t’ test, significant differences were observed. Group 2 showed significant increase for both nasal cavity width (P-0.007) and maxillary width (P-0.003) when compared to Group1.Transverse dental findings

Since the data pertaining to intercanine width and intermolar width were normally distributed parametric test of significance (unpaired t test) was used to compare between the groups (table IV). Group 1 showed significant increase for both upper intercanine width (P- 0.001) and intermolar width (P-0.000) Group 2 also showed significant increase for both upper intercanine width (P-0.000) and intermolar width (P-0.000) When the two groups were compared using student‘t’ test, group 2 showed significant increase for the intermolar width than Group 1 (P-0.000). 502 Insignificant difference was seen for intercanine width (P-0.235). 10th World Cleft Lip, Palate & Craniofacial Congress DISCUSSION

Patients with narrow maxilla sometimes require a selective anterior expansion especially when there is no posterior cross bite and the molar width is normal. This is most often the case in habit induced constriction of the arches and cleft palate cases. In cleft palate cases the width between the Pterygoid hamuli is slightly wider than in noncleft cases. The muscle intergrity in the soft palate region is lost if a cleft involves this region. The tensor veli palatini muscle ridgesafter originating on the horizontal from the portion a) scaphoid of the palate fossa afterb) spina winding angularis around and the c) hamular lateral wall of the auditory tube inserts into the palatine aponeurosis and transverse process. This muscle tenses the soft palate and opposes the outward pull on the pterygoid process exerted by the internal and external pterygoid muscles. With a lack of continuity of the tensor veli palatine muscle in cleft cases, there is unopposed outward pull of the hamular process by the pterygoid muscles palatewhich explainsmanifests the as increased a normal hamularlateral relationship width in these of thecases. most With posterior full pterygoid teeth, width and anterior collapse, the stereotype malocclusion associated with cleft the cross bite only becoming evident towards the anteriors. [7,9,10] When the regular RME screw is used in such cases a near parallel expansion of molars and cuspid region occurs. [11-15] The arch is expanded in the region where it is not required. To that end, Schellino et al [8] introduced the Fan type RME claiming that it works asymmetrically. Doruk et al [16] compared the effects of tissue borne Fan type RME with Hyrax and concluded that intercanine expansion was significantly greater in Fan type RME than Hyrax. Luca Levrini and Vittorio [17] reported a case of narrow maxilla treated with Fan RME that had greater intercanine expansion than intermolar expansion. In this study we used the tooth borne RME design of Mcnamara wherein the incisors were left uncovered. Occlusal radiographs revealed a more typical V opening for the Fan RME (Fig 3a) compared with the Hyrax (Fig 3b). Similar to the posterior resistance offered by the circummaxillary sutures which allows the two halves of the maxilla to open in a V pattern [18-21], the distal hinge in the Fan type screw offers a posterior resistance that allows a more ‘V’ shaped opening of the maxillary dental arch. The hinge therefore acts as an additional resistance to facilitate a more V shaped opening. Hyrax presented with significantly greater increments for both nasal cavity width and maxillary width when compared with Fan type RME. The limited parallel opening for the Fan RME attributed to the distal hinge could have caused the minimal nasal widening compared to Hyrax. Since the maxillary503 10th World Cleft Lip, Palate & Craniofacial Congress width was assessed at the level of jugale points, the restricted posterior expansion by the hinge in the Fan RME led to this maxillary width difference between Fan RME and Hyrax. The increase in the intercanine width was almost similar in both the groups. Fan type RME caused only minimal expansion of the intermolar width when compared to the Hyrax attributed to the distal hinge. The ratio between the intercanine and intermolar width was nearly 4:1 in the Fan type RME and 0.75:1 in Hyrax. This is in accordance with the findings of Doruk et al [16] and LucaConclusion Levrini and Vittorio. [17]

The results suggest that the fan-type RME screw can expand the maxilla asymmetrically with less posterior expansion both dentally and skeletally. These findings may be of great benefit in the treatment of patients exhibiting anterior maxillary narrowness with normal intermolar width and in cleft palate patients. More studies involving an increased sample size and long term follow up are needed to have a better knowledge about the clinical efficiency of the FanReferences: type screw. 01. Gross A M, Kellum G D, Franz D, Michas K, Walker M, Foster M, Bishop F W. A longitudinal evaluation of open mouth posture and maxillary arch width in children. The Angle Orthodontist 1994; 64: 419-424. 02. Marvin L. Hanson, Melvin S. Cohen Effects of form and function on swallowing and the developing dentition. Am J of Orthod and Oral Surgery 1973; 64: 63–82. 03. Felice S. O'Ryan, Dale M. Gallagher, John P. LaBanc, Bruce N. Epker. The relation between nasorespiratory function and dentofacial morphology: A review. Am J of Orthod and Oral Surgery 1982; 82: 403–410. 04. H.C. Metz. Pernicious habit retarding lateral development of anterior teeth. Am J of Orthod and Oral Surgery 1930; 16: 968–970. 05. Lewis Krams Beck. Chronic mouth-breathing. Am J of Orthod and Oral Surgery 1923; 9: 263–264. 06. Linder Aronson. Respiratory function in relation to facial morphology and dentition. Br J orthod 1979; 6:59-71. 07. Subtelny J, Rochester N Y. Width of the nasopharynx and related anatomic structures in normal and unoperated cleft palate children. Am J of Orthod 1955; 41: 889-909.

504 10th World Cleft Lip, Palate & Craniofacial Congress 08. Schellino E, Modica R, Benech A, Modaro E. REM: la vite ragno secondo Schellino e Modica. Boll Interm Orthod 1996; 55: 36–39. 09. Graber T M. Craniofacial morphology in cleft palate and cleft lip deformities. Surg.Gynec. Obstct 1949; 88:359-367. 10. Graber T M. The congenital cleft palate deformity. J.Am.Dent.Ass 1954; 48:375-395. 11. Suzuki A, Takahama Y. A Jointed Fan Type expander: A newly Designed expansion appliance for the upper dental arch of patients with cleft lip and/or palate. Cleft Palate Journal 1989; 26: 239-241. 12. Müge Sandikçiolu, Serpil Hazar. Skeletal and dental changes after maxillary expansion in the mixed dentition. Am J of Orthod and Dentofacial Orthop 1997; 111: 321–327. 13. Renée G. Geran, James A. McNamara Jr, Tiziano Baccetti, Lorenzo Franchi, Lainie M. Shapiro. Prospective long-term study on the effects of rapid maxillary expansion in the early mixed dentition. Am J of Orthod and Dentofacial Orthop 2006; 129: 631–640. 14. André Weissheimer, Luciane Macedo de Menezes, Mauricio Mezomo, Daniela Marchiori Dias, Eduardo Martinelli Santayana de Lima, Susana Maria Deon Rizzatto. Immediate effects of rapid maxillary expansion with Haas-type and hyrax-type expanders: A randomized clinical trial. Am J of Orthod and Dentofacial Orthop 2011 ; 140: 366–376. 15. Miray Gunyuz Toklu, Derya Germec-Cakan, Murat Tozlu. Periodontal, dentoalveolar, and skeletal effects of tooth-borne and tooth-bone-borne expansion appliances. Am J of Orthod and Dentofacial Orthop 2015; 148:97–109. 16. Cenk Doruk, Ali Altug Bicakci, Faruk Ayhan Basciftci, Ugur Agar, Hasan Babacan. A Comparison of the Effects of Rapid Maxillary Expansion and Fan-Type Rapid Maxillary Expansion on Dentofacial Structures. The Angle Orthodontist 2004; 74: 184–194. 17. Luca Levrini,Vittorio Filippi. A Fan Shaped expander. J of Clin Orthod 1999; 11: 642-643. 18. Donald J. Timms. A study of basal movement with rapid maxillary expansion. Am J of Orthod and Oral Surgery 1980; 77: 500–507. 19. Müge Sandikçiolu, Serpil Hazar. Skeletal and dental changes after maxillary expansion in the mixed dentition. Am J of Orthod and Dentofacial Orthop 1997;111:321-327. 20. John L. Hayes. Rapid maxillary expansion. Am J of Orthod and Dentofacial Orthop 2006;130:432-433. 21. Brett J. Garrett, Joseph M. Caruso, Kitichai Rungcharassaeng, James R. Farrage, Jay S. Kim, Guy D. Taylor. Skeletal effects to the maxilla after rapid maxillary expansion assessed with cone-beam computed tomography. Am J of Orthod and Dentofacial Orthop 2008;134: 8.e1–8.e11. 505 10th World Cleft Lip, Palate & Craniofacial Congress

TABLE 1: Difference between pretreatment and post treatment findings of Fan RME

Table II: Difference between pretreatment and post treatment findings of Hyrax

Table III: Comparison between FAN type RME and Hyrax

Table IV: comparison for transverse dental findings

506 10th World Cleft Lip, Palate & Craniofacial Congress

Figure 1a: Pretreatment occlusal radiograph of Fan RME Figure 1b: Pretreatment occlusal radiograph of Hyrax

Figure 2: Fan type RME

Figure 3a: Post treatment occlusal radiograph of Fan RME Figure 3b: Post treatment occlusal radiograph of Hyrax

507 10th World Cleft Lip, Palate & Craniofacial Congress

Evaluation of skeletal and dental changes followed by maxillary expansion using NITI palatal expander in patients with unilateral Cleft lip and palate.

Sangeetha Duraisamy Reader, Department of orthodontics & Dentofacial orthopaedics , SRM Dental College, Ramapuram, , Chennai, TamilNadu, India. Co Author: K. Ravi HOD, Department of orthodontics & Dentofacial orthopaedics, Dean, SRM Dental College, Ramapuram, Chennai. Abstract:

Introduction: In CLCP patients surgical repair of the palate often results in constriction of the maxillary arch due to the contraction of the scar tissue. NITI palatal expanders are commonly used for maxillary expansion in CLCP owingAim: to its advantages.

The aim of the study is to evaluate the skeletal and dental changes followed by maxillary expansion using NITI palatal expander in patients with unilateralMaterials: CLCP.

Pre and post expansion study models, maxillary occlusal photographs, lateral cephalograms and frontal cephalograms of eighteen Unilateral CLCP patients treated with NiTi palatal expander were included in the study. The study models were analysed for changes in intermolar, inter premolar and intercanine width. PA cephalograms were analyzed for changes in bizygomatic width, nasal cavity width, maxillary width and intermolar width. The lateral cephalograms were compared for changes in facial height, maxillary first molar tipping,Results mandibular& Discussion: plane angle and occlusal plane inclination.

Expansion was produced at a rate 0f 2.1± 0.04 mm/ month with Skeletal expansion contributing to only 23% of the total changes. Significant changes occurred508 in the maxillary first molar region in inter molar width with distal 10th World Cleft Lip, Palate & Craniofacial Congress

tipping and derotation of the molar. Significant opening of the mandibular plane angle, tipping of the occlusal plane and increase in anterior facial height wasConclusion: noted inspite of the bite blocks placed in lower molars.

The expansion produced by the NiTi palatal expander is predominantly dental in patients with treated unilateral cleft lip and cleft palate patients.

509 10th World Cleft Lip, Palate & Craniofacial Congress

COMPUTER ASSISTED MORPHOMETRICS IN COMPLEX CRANIAL VAULT RECONSTRUCTIVE SURGERY

Harsh Pipalia1, Praveen G2, Samarth Shetty 3, Paul C. Salins4, Rohan Raut5, Hrishikesh Bhowmick 6 1Fellow, Department of Craniofacial Surgery, Mazumdar Shaw Cancer Centre, Bangalore, 2Consultant, Department of Craniofacial Surgery, Mazumdar Shaw Cancer Centre, Bangalore, 3Consultant , Department of Craniofacial Surgery, Mazumdar Shaw Cancer Centre, Bangalore, 4Head, Department of Craniofacial Surgery, Mazumdar Shaw Cancer Centre, Bangalore, 5Fellow, Department of Craniofacial Surgery, Mazumdar Shaw Cancer Centre, Bangalore, 6Fellow, Department of Craniofacial Surgery, Mazumdar Shaw Cancer Centre, Bangalore

Abstract Introduction

Morphometrics refers to the quantitative analysis of form, a concept that encompasses size and shape. In craniofacial surgery, surgeons are often faced with reconstruction of complex deteriorated structures caused by tumors, trauma or congenital abnormalities Based on the type of defect or abnormality; reconstruction should be planned according to the individual anatomy and physiology in order to achieve the best functional and aesthetic outcome. Various techniques have been described in the literature for fabrication of patient specific titanium cranial implants. On this account, we present computer assisted planning and utilization of morphometric principles for fabrication of patient specific titanium implants in the reconstruction of complex cranial defects.Materials and Methods

Ten Patients with complex cranial defects more than 5cm were included. CT scan was done for all the patients. Stereolithographic (STL) model was made .The DICOM images were fed into the 3D Dolphin Imaging software and modeling wax. Titanium moul reformatted contour were transferred over the defect on STL model using ds were fabricated following surface scans of reconstructed510 contour on STL 10th World Cleft Lip, Palate & Craniofacial Congress Results

A successful anatomic and aesthetic outcome without any major complicationConclusion were achieved in all the patients

The principles of morphometry in combination with computer assisted surgical planning can provide an insight to the envelope of surgical correction andKeywords provide psychological preparation and education to the patient.

INTRODUCTIONMorphometrics, cranial defects, titanium mould, STL model.

Reconstruction of the craniofacial skeleton is extremely challenging even to the most experienced surgeon. Some of the critical factors that contribute to the complexity include anatomy, presence of vital structures adjacent to the affected part, uniqueness of each defect and chances of infection. In any craniofacial reconstruction whether secondary to trauma, ablative tumor resection, infection and congenital/developmental deformities, restoration of aesthetics and function is the primary goal and calls for precise pre-surgical planning and execution of the plan1 Decompressive craniectomy is a lifesaving surgical procedure in acute brain swelling. For those who survive, the cranial defect after decompressive craniectomy is usually reconstructed at a later point of time. Cranioplasty is performed for the following reasons: protection of the brain, restoration of the brain's hydrodynamic conditions and cosmetics.2 Traditionally, cranioplasty implants were fabricated in the operating dependsroom by strongly manual moldingon the manual of a pliable ability biocompatible of the surgeon, material often that resulting sets over in time to assume a solid shape. The good outcome of such procedure, however, unsightly repairs3 Computer-designed alloplastic implants have revolutionized the conceptualization and approach to complex cranioplasty and have become a reliable alternative. These implants have the advantage of being preoperatively tailored to the exact size of the cranial defect, thus allowing a shorter operating time, improved postoperative stability, and incomparable cosmetic results4 We describe a technique of fabrication of titanium patient specific moulds by combining the principles of direct and computer assisted indirect methods511 10th World Cleft Lip, Palate & Craniofacial Congress for reconstruction of cranial defects. The aim of this article is to introduce a new engineering strategy in manufacturing cranial implants, which can possibly be exploredMATERIALS further AND and METHODS applied in other fields of medical implant fabrication. Steps In Implant Fabrication

Computed tomography (CT) from vertex to midface was used for acquisition of patient data. The CT data in DICOM format were sent to manufacturer for fabrication of Stereolithographic model (Jayon Surgicals,Kerala, India). The DICOM files were fed in Dolphin 3D imaging software 11.8(Dolphin Imaging and Management Solutions, Chatsworth, CA). morphometrics from uninvolved side were superimposed on defect at every interval of 1cm (Fig.1). superimposedModeling wax ofimages 1 cm widthand were contouredtransferred based on to on the

Stereolithographic model. The additional 5% fullness was added with modeling wax in temperoparietal defects for compensating the temporalis detachment (Fig 2, 3 4). The Wax model is sent back to manufacturer for fabrication of titanium mould. The wax model was scanned using blue light scanner and data is obtained in STL format. This data is transferred to CNC milling machine for fabrication

of titanium mould of 0.6mm thickness. Mould is perforated at multiple places to facilitate drainage and prevent collection. Holes were drilled in Extension wings for screw fixation over existing bone (Fig.5). 512 10th World Cleft Lip, Palate & Craniofacial Congress PATIENT POPULATION Ten patient

s with post resection or Post traumatic cranial defects were treated using this technique. inferiorAll 10 patientsmargins wereof the havingimplant defect were in temporo-parietal area. The stripping of temporalis kept short of around1 cm to avoid excessive muscle. Surgical site was accessed through previous incision made for craniectomy procedure. Titanium moulds were secured using 4mm self RESULTdrilling screws.

No intraoperative or postoperative complication were observed (mean postoperative period 9 months). Modification of implant was not required intra-operatively in any of the patients. The mean duration of procedure was 50DISCUSSION minutes. All patients were satisfied with surgical outcome.

Cranioplasty is an operative procedure aimed to fill a hole in the cranial theca or to replace bone removed either as a result of trauma, infection or tumor, by means of an artificial prosthesis13. The main indications for cranioplasty are cerebral protection, cosmesis, and as a therapy of the so-called ‘‘syndrome of the trephined’14.Various alloplastic materials are available for cranioplasty such as stainless steel mesh-acrylic 5, titanium 6, 7, methyl methachrylate 8, and customized prefabricated prostheses 9, and autologous cryopreserved bone 10. The use of computers in the analysis of cephalometric data has a long history11. The three dimensional preoperative planning of the surgical procedures with computer assistance has recently been described. The virtual planning helps surgeons better understand the steps of the reconstructive procedure and focus on achieving optimal shaping of the bony segments. Many investigators have reported that, when applying 3D medical models to clinical cases, model accuracy is a major concern12. The increase in the accuracy of the bio-model allows an improvement in the medical applications based on image diagnosis, more accurate results in computational modeling, and improvements in surgical planning in situations in which the required accuracy directly affects the results of the procedure. The three approaches to three-dimensional surgical513 10th World Cleft Lip, Palate & Craniofacial Congress simulation have been reported by Marsh et al15. In the first approach, mirror- image techniques project to the normal side over the mid-sagittal plane to the abnormal side, allowing precise measurements to be taken of the deformity. A second approach involves model making using computer controlled milling machines based on CT data. And the third approach involves preoperative planning of procedures directly on the computer terminal using commercially available computer assisted design (CAD) software. So in our study we have used all the three approaches described above. First a STL model was prepared and then the DICOM files were uploaded in software and morphometrics were applied and modeling wax of 1 cm width were contoured based on the superimposed images and were transferred on to the Stereolithographic model. In our study titanium has been a material of choice for cranioplasty due to its biocompatibility, strength to weight ratio and osseo-integrative property. Titanium in various forms as sheets, mesh have been in use for sometime more recently with the advent of EBM or direct metal laser sintering (DMLS) 3D printed cranial implants has come into vogue. Titanium mesh reconstruction is a popular method among the surgeons due to the ability to use the preformed mesh as a template for resection. However, the strength of a thin dynamic mesh that can be molded intra-operatively at times requires to be enhanced with PMMA. So to make is more cost effective we have used titanium plate which has good strength and additional bone graft or PMMA is not required. In recent years the model of the cranium with the defect is fabricated using 3D printing technologies and used as a replica or template of the actual region of interest depicting the precise defect. A secondary processing method as forming is used to produce the actual implant16. This process delivers a well-fitting prosthesis and is very useful in treating large cranial defects with advantages of reduced, operating time, healing time and hospitalization period, eventually leading to reduced cost to the patient. A novel method is being used at our center. In the current method, the implant is designed by applying morphometrics from uninvolved side which were superimposed on defect at every interval of 1cm and the wax up was created on the STL model. The translation between designing and fabrication of the implant is brought about with the help of blue light scanning. Finally the titanium implant is fabricated basedREFERENCES on CNC milling machine. 1. Parthasarathy J. 3D modeling, custom implants and its future perspectives in craniofacial surgery. Annals of maxillofacial surgery. 2014 Jan; 4(1):9. 514 10th World Cleft Lip, Palate & Craniofacial Congress 2. Sundseth J, Sundseth A, Berg-Johnsen J, Sorteberg W, Lindegaard KF. Cranioplasty with autologous cryopreserved bone after decompressive craniectomy. Complications and risk factors for developing surgical site infection. Acta neurochirurgica. 2014 Apr 1; 156(4):805-811. 3. Solaro P, Pierangeli E, Pizzoni C, et al. From computerized tomography data processing to rapid manufacturing of custom-made prostheses for cranioplasty. J Neurosurg Sci 2008;52:113–116 4. Gerbino G, Bianchi FA, Zavattero E, et al. Single step resection and reconstruction using patient specific implants in the treatment of benign cranio-orbital tumors. J Oral Maxillofac Surg 2013;71:1969–1982 5. Galicich JH, Hovind KH (1967) Stainless steel mesh-acrylic cranioplasty. Tech Note J Neurosurg 27:376–378 6. Joffe J, Harris M, Kahugu F, Nicoll S, Linney A, Richards R (1999) A prospective study of computer-aided design and manufacture of titanium plate for cranioplasty and its clinical outcome. Br J Neurosurg 13:576–580 7. Stoodley MA, Abbott JR, Simpson DA (1996) Titanium cranioplasty using 3-D computer modelling of skull defects. J Clin Neurosci 3:149–155. 8. Benzel EC, Thammavvaram K, Kesterson L (1990) The diagnosis of infections associated with acrylic cranioplasties. Neuroradiology 32:151–153 9. Couldwell WT, Chen TC, Weiss MH, Fukushima T, Dougherty W (1994) Cranioplasty with the Medpor porous polyethylene flexblock implant. Tech Note J Neurosurg 81:483– 486. 10. Cranioplasty with autologous cryopreserved bone after decompressive craniectomy. Complications and risk factors for developing surgical site infection. Acta Neurochirurgica. April 2014, Volume 156, Issue 4, pp 805–811. 11. Burke P H, Banks P, Beard I, Tee J F and Hughes C. Stereophotographic measurement of change in facial soft tissue morphology following surgery. Br. J. Oral Surg 1983; 21:237. 12. J.Y. Choi, J.H. Choi, N.K. Kim, et al. Analysis of errors in medical rapid prototyping models. Int J Oral Maxillofac Surg, 31 (2002), p. 23. 13. Solaro P, Pierangeli E, Pizzoni C, et al. From computerized tomography data processing to rapid manufacturing of custom-made prostheses for cranioplasty. J Neurosurg Sci 2008; 52:113–116 14. Joseph V, Reily P. Syndrome of the trephined. J Neurosurg 2009; 111:650–652.

515 10th World Cleft Lip, Palate & Craniofacial Congress 15. Marsh J. L, Vannier M W, Stevens W G, Warren, j O, Gayou D and Dye D M. Computerized imaging for soft tissue and osseous reconstruction in the head and neck. Clin. Plast. Surg. 12:279, 1985. 16. Chen JJ, Liu W, Li MZ, Wang CT. Digital manufacture of titanium prosthesis for cranioplasty. Int J Adv Manuf Technol. 2006; 27:1148–52. FIGURE LEGENDS:

Fig. 1: Morphometric analysis using 3D dolphin imaging software Fig. 2: Template for contour transfer Fig. 3: Contour transfer on STL model Fig. 4: Contoured wax up model Fig. 5: patient specific cranial reconstruction plate

516 10th World Cleft Lip, Palate & Craniofacial Congress

Maxillary distraction osteogenesis for cleft lip and palate patients -Influence on stability and speech of maxillary anterior segmental distraction osteogenesis (MASDO) with rigid external distraction (RED) system

Yoshihide Mori1, Tomohiro Yamada1, Hiroyuki Nakano1, Tomoki Sumida1, Naoto Haruyama2, Ichiro Takahashi2 1Section of Oral and Maxillofacial Surgery, Faculty of Dental Science, Kyushu University 2Section of Orthodontics, Faculty of Dental Science, Kyushu University

The Le Fort I maxillary distraction osteogenesis (DO) is effective for the orthognathic treatment in cleft lip and palate patients with severe maxillary retrusion. The amount of maxillary advancement by the one stage Le Fort I osteotomy is limited within 5 mm, but the Le Fort I DO can advance more than 10 mm. Less exacerbation of velopharyngeal function is observed compare with one stage operation. However, exacerbation of velopharyngeal function arose in borderline cases even in the cases of Le Fort I DO. For such borderline cases, the maxillary anterior segmental distraction osteogenesis (MASDO) was developed to avoid the problem. We use rigid external distraction (RED) system with MASDO, and the profile and technique was already presented at the CLEFT 2014 held in Hanoi, Vietnam. The stability and speech in 3 cases adopted MASDO with RED system were examined. The amounts of antero-inferior movement in maxillary segments were 15 to 20mm. The maxillary segments were advanced 1 mm/day, after 1 week from the operation. The internal fixation with 2 or 3 mini plates for each side was given after the distraction, and the RED system was removed, simultaneously. The ratio of relapse one year later was about 20%. Exacerbation of velopharyngeal function in a case was observed but recovered as time passed.

517 10th World Cleft Lip, Palate & Craniofacial Congress

Morphometric distraction for mandible in asymmetry corrections

Manikandhan R Dept. of Oral & Maxillofacial Surgery, Meenakshi Ammal Dental College & Hospital, Chennai, India

Hemifacial microsomia or any facial asymmetry have been treated by different methods depend on the age, type of severity and nature of pathology of the case presentation. Starting from simple distraction to complex Craniofacial correction by osteotomies produces excellent results. Almost all the procedures involving mandibular and maxillary component involves occlusal derangement necessitating continuos and or sometimes temporary orthodontic treatment to settle the occlusion. Morphometric distraction of mandible is an indigenous technique developed by the author for correction of asymmetry if a clinician does not want to change the occlusion but at same correct the facial symmetry to acceptable levels. This complete intraoral technique necessitates a special osteotomies.osteotomy design and indigenous distractor eliminating need for bone grafting and multiple plate fixation with negligible relapse after drastic movements for

518 10th World Cleft Lip, Palate & Craniofacial Congress

Nasoalveolarmoulding - Contemporary approach for cleft infants

J.A.Nathan Professor, Dept of OMFS , Ragas dental college and hospital, Chennai, India

Introduction:

Although there have been great advances in the field ofcleft surgery, the surgical repair alone cannot solve the multiple problems encountered. The success of treating cleft lip and palate is to achieve esthetically invisible scar, good nasal tip projection, precisely defined nasolabial complex and functionally good speech. The severity of deformity will directly increase the difficulty in surgical procedure and also affects the functional outcome of surgery. In a patient with a large cleft deformity, they have more hard and soft tissue deficiency .The objective of presurgical Nasoalveolar moulding (NAM) is to reduce the severity of initial cleft deformity and the achieve better esthetics and function.

519 10th World Cleft Lip, Palate & Craniofacial Congress

PREVENTION OF OROFACIAL CLEFT IN THE DEVELOPING WORLD

A.PRASZANTH Post-Graduate Resident, Dept. of Oral & Maxillofacial Surgery, Thai Moogambikai Dental College and Hospital, Chennai, Tamil Nadu, India

Orofacial clefts remain a prominent issue in the developing world, it is a common craniofacial birth defect in humans.Evidence suggest that the defect is polygenic and multifactorial in origin. Primary prevention of orofacial cleft is based on the recognition of etiologic and risk factors.while a number of preventive strategies are in place for the facial clefts in the developed world, the majority of developing countries are distant from achieving this goal for a number of reasons notable among these are discrepancy in the knowledge of genetics. In addition to it improper coordination of antenatal care contributed greatly to this setback. With ongoing effects aimed at determining the genetics of no syndromic orofacial clefts patients in developing countries researches directed at identifying the environmental factors should also play a role.

520 10th World Cleft Lip, Palate & Craniofacial Congress

DENTOFACIAL CHARACTERISTICS OF REPAIRED CLEFT LIP AND PALATE PATIENTS IN MALAYSIA

N Abdulla¹ and MB Alam² College of Dental Medicine University of Sharjah¹ and School of Dental Sciences, Universiti Sains Malaysia² Introduction

Facial morphology in individuals with cleft lip and palate deviates from the norm (Friede, H, 1977; Her-mann et al., 1999). The aim of this study is to evaluate the dentofacial characteristics of repaired cleft lip and palate patients when compared with non-cleft patients in the pediatricMaterials and and adult Methods age group.

This is a comparative cross-sectional study on 75 repaired cleft lip and palate patients age from 8 to 40 years old attending Hospital University Sains Malaysia, and 75 non cleft subjects of the same age group who have given their consent and met the inclusion and exclusion criteria. Sampling of patients was done by systematic random sampling. Landmarks for measurements were identified on the face, lat-eral cephalometry tracings and dental study models. All measurements were done using a digital slid-ing caliper or a sliding caliper and an orthodonticResults ruler. Data were analysed using SPSS software version 20.0

The anterior-posterior and transverse dental arch dimensions of upper and lower incisors in relation to the respective planes were significantly higher for all non-cleft age groups. Angular measurement SNA were also found to be significantly higher for all non-cleft group. Oro-labial measurement sn-sto were also significantly higher in all non cleft group. However the oro-labial measurements cph-cph were sig- nificantly higher in all repaired cleft group. The mid-face measurements were significantly different at (n-sto) showing significantly higher measurement in non cleft adult group. 521 10th World Cleft Lip, Palate & Craniofacial Congress Discussion and Conclusions

study Therepopulation. were significant differences in dentofacial characteristics between repaired cleft and noncleft children, adolescent and adults in this

Orbital measurements did not show significant difference between the two groups. Linear measure-ments of structures in the mid face showed significant difference with the cleft group demonstrating shorter vertical and transverse dimensions, thus resulting in a class III dental malocclusion at the endKey ofwords the facial growth period.

Dentofacial characteristic, repaired cleft

522 10th World Cleft Lip, Palate & Craniofacial Congress

Niti Palatal Expander – When & How???

J.Abhishek III Yr P.G, SRM Dental College, Chennai, India

A major concern with cleft cases has been maxillary deficiency and this has numerous methods of approach. The correction of a transverse discrepancy occurs by a combination of both orthodontic and orthopedic tooth movement. The most common approach for the arch expansion is the rapid palatal expansion , but with the condition of the cleft as a main concern, slow and continuous forces are preferred for the expansion. Niti Slow Expansion Appliance has the ability to produce light , continuous pressure and the mid-palatal suture while simultaneously uprighting ,rotating and distalizing the maxillary first molar. A unique feature about this appliance is that it is temperature activated. This poster will show the use of a Niti Palatal Expander for expansion and when and how the appliance is chosen .

523 10th World Cleft Lip, Palate & Craniofacial Congress

Myofunctional Dental Preparation Before Bone Grafting

A.M.SHAKEEL Post-Graduate Resident, Dept. of Oral & Maxillofacial Surgery, Thai Moogambikai Dental College and Hospital, Chennai, Tamil Nadu, India

Secondary alveolar bone grafting is carried for patients with orofacial clefts to support the teeth on either side of the alveolar cleft. Orthodontic dental treatment aimed at improving the arch form is an essential criterion for successful bone grafting. The orthodontic procedure normally involves maxillary expansion align and level the alveolar bone on both sides of the cleft. Conventional palatal expansion technique such as Rapid Maxillary Expansion(RME) encounter bony resistance in cleft patients. In addition to this there are some disadvantages in clinical practise such as external root resorption, periodontal attachment recession and alveolar bone resorption.Expanding the arch while not opening the cleft toomuch will be a dilemma for orthodontic treatment. Myofunctional dental preparation was introduced to manage the palatal expansion.

524 10th World Cleft Lip, Palate & Craniofacial Congress

Clinicostatistical investigation on cleft lip and/or palate patients in the Kyushu University Hospital

Ami MAENO, Kaori MATSUMURA, Shinsaku ARAI, Takeshi MITSUYASU and Seiji NAKAMURA Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Japan.

Objectives

surgeons,The pedodontists, approach for orthodontists, the patient otolaryngologists, with cleft lip and/oranesthesiologists palate is multidisciplinary, and the cleft team should be ideally composed by craniofacial and speech-language pathologists. In our hospital, cleft patients are provided team medical care. Our cleft team actively conducts prenatal counseling and postpartum visits to obstetrics. We report about clinicostatistical investigation to grasp the actual circumstances of cleft lip and palate patients in Kyushu UniversityMaterials andHospital. Methods

A statistical investigation was carried out on patients with cleft lip and/ or palate within the Department of Oral and Maxillofacial Surgery, Kyushu University Hospital. This investigation was over the course of 16 years from JanuaryResults 1998 to December 2013.

The results were as follows: 1. Total number of primary cases was 418 (males, 197; females, 221). 2. These cases included 132 patients with cleft lip and palate, 143 patients with cleft lip (and alveolar), 122 patients with cleft palate, 20 patients with submucous cleft palate and 1 patient with facial cleft. 3. Cleft lip and palate cases were observed more frequently in males, while cleft palate cases were observed more frequently in females. 525 10th World Cleft Lip, Palate & Craniofacial Congress

areas. 4. Most of the patients were residents in Fukuoka city and the surrounding

5. The largest majority of patients were referred from other obstetric clinics. 6. Total number of prenatal counseling cases was 89, and the number of cases of postnatal rounds in obstetric clinics was 212. Both prenatal counseling Conclusionscases and postnatal round cases gradually increased within 16 years.

The number of patients has increased in recent years. Patient data grouped by cleft type shows an increase in the number of patients with cleft palate. This is likely due to the growing number of referrals from speech and language therapy facilities. Cleft lip and palate was found to be more common in males, while cleft palate was more common in females. This was consistent generalwith previous population. findings in Japan. There was a higher incidence of cleft lip and/ or palate patients born prematurely or with low birth weight, compared to the

When the number of prenatal counseling cases and postnatal round cases increased, it was suggested that it was because the needs from patients’ families and obstetricians also increased. The mental burden on the patient’s family is reduced by prenatal counseling and postnatal rounds. We want to continue to build good relations with local obstetric hospitals and encourage facilitation of the cleft lip and palate treatment.

526 10th World Cleft Lip, Palate & Craniofacial Congress

Hemi-facial hypertrophy with trismus

Amirthaa Varshini Post-Graduate Resident, Dept. of Oral & Maxillofacial Surgery, RMDCH, Tamil Nadu, India

Hemi facial hyperplasia is a rare developmental anomaly characterised by asymmetric overgrowth of one or more body parts, it usually represents a hyperplasia of the tissues rather than a hypertrophy. This is about ,a case report of an 14 year old female , who reported with complaints of restricted mouth opening , diffuse swelling of right side of face, on further investigations revealed muscle hypertrophy with fatty replacement noted in right masseter, both medial and lateral pterygoids, buccinator, mylohyoid, anterior and posterior belly of digastrics, zygomaticus major muscle. Mild osseous hyper trophy of right half of mandible was noted. The current literature states that imaging studies such as CT/MRI must be done as confirmation and surgery be performed mainly for cosmetic reasons, but this was a reported case with functional deformity and severe trismus. The patient was taken up for surgical correction by high condylectomy along with release of fibrotic bands and reconstruction with buccal pad of fat, therby improving function of the patient. This paper highlights on the rare presentation with clinical signs and symptoms, hence the outcome of surgery is presented with extensive review of literature.

527 10th World Cleft Lip, Palate & Craniofacial Congress

Furlows Palatoplasty

Ankush Sune PG Resident, Oral & Maxillofacial Surgery, RDVV Jabalpur , Madhya Pradesh, India

Cleft palate is considered to be most common facial birth defect worldwide. Attempts to repair these defects are made back to 16th century. Various palatoplasty techniques have been developed since then. Furlow’s double opposing palatoplasty (FDOP) was first introduced by Leonard furlow in 1978. Its distinct advantage over other techniques are lengthening of soft palate, restoring a normal velar anatomy and function. Here I present a poster on the technical details of the same.

528 10th World Cleft Lip, Palate & Craniofacial Congress

My acquaintance with cleft patients in one year of residency

Anuj Chauhan Post graduate student, Division of Orthodontics and Dentofacial Deformities, Centre for Dental Education and Research, All India Institute of Medical Sciences, New Delhi- 110029. INDIA

Abstract

About 30000 children are born in India with cleft lip and/or palate every year. Moreover incidence of cleft lip and palate in AIIMS hospital New Delhi was found to be 1.4per 1000 and 0.3 per 1000 for isolated cleft palate. Since 1972 All India Institute of medical sciences is providing care for cleft patients which usually start from infant life and continued till adulthood and involves multidisciplinary approach. Since post graduate students are also involved in providing care to such patients; so being a post graduate student at CDER, AIIMS for about 18 months, my acquaintance with cleft cases so far is highlighted in terms of challenges noticed and treatment plans formulated to meet the objectives and also the progress achieved in past one year. It includes 3 cases two of which reported at adolescent age. Case 1 presented with constricted maxillary arch and severity related to Goslon index 2 and was planned to be treated with expansion in upper arch followed by alignment and evaluation for bone graft with nose and lip revision. Case no 2 presented with constricted maxillary arch and missing right lateral incisor and second premolar with Goslon index 2 and was planned to be treated with expansion in upper arch with simultaneous distalisation of right first premolar followed by alignment and upper lip revision. Case 3 was an adult female presented with reverse overjet and vertical maxillary deficiency with impacted left maxillary canine with Goslon index 4 and was planned to be treated with expansion in upper arch followed by alignment and further Le Fort I osteotomy and BSSO.

529 10th World Cleft Lip, Palate & Craniofacial Congress

Modified Le Fort I osteotomy along with secondary Alveolar grafting for a Cleft facial deformity case

1M Arun , 2Prof. Dr. Muthushekhar 1Post Graduate, Oral & Maxillofacial Surgery, Saveetha Dental College, 2Head of the Department, Oral & Maxillofacial Surgery, Saveetha Dental College.

A cleft of the lip, gum (alveolus), and/or palate can produce a variety of dental and facial deformity problems. It needs multimodality intervention to produce a satisfactory functional and aesthetic outcome . Here we present a case report of a male patient age 23 years who presented to us with the history of cleft lip surgery when he was a year old and his current complaint was to correct his alveolar cleft and midface deficiency. Secondary alveolar grafting using autogenous iliac bone along with a modified Le fort I procedure was done to correct the underlying Alveolar Cleft and facial deformity.

530 10th World Cleft Lip, Palate & Craniofacial Congress

Orthodontic management of excessive incisor display of an adult bilateral cleft lip and palate patient

Ashish Prasad Senior Resident , Division of Orthodontics and Dentofacial Deformities,, Centre for Dental Education and Research, All India Institute of Medical Sciences, New Delhi- 110029., INDIA

This poster entails the successful orthodontic treatment of a case of postsurgical malocclusion, short upper lip and excessive incisor display in an adult female patient of 23 year with bilateral cleft lip and palate.The patient underwent cleft lip repair at an early age of 2.5 year followed by palatoplasty at the age of 21 years. She reported with irregular and supernumerary teeth, inability to close lips, excessive visibility of upper incisors associated with short upper lip and difficulty in speech. After the extraction of supernumerary teeth she was treated with arch alignment, intrusion and retraction of the maxillary central incisors followed by prosthetic replacement of the missing teeth. The intrusion and retraction of incisors in an adult bilateral cleft lip and palate female of 23 year gave her a remarkably good smile. Subsequently she underwent nose and lip revision surgery at the age of 27 year. The orthodontic procedures and prosthetic rehabilitation have resulted in a near normal dental surgery.occlusion with significant improvement in aesthetics and psychosocial benefits to the individual.These benefits were further enhanced by nose and lip revision

531 10th World Cleft Lip, Palate & Craniofacial Congress

Modified presurgical orthopaedics - Nam (Nasoalveolar Moulding)

Bhuvaneswari .M second year-PG, SRM dental college

Nasoalveolar molding (NAM) is an an adjunctive and modified presurgical neonatal therapy for the correction of cleft lip and palate. Nasoalveolar molding can be done effectively to reshape the nasal cartilage and mold the maxillary dentoalveolar arch before surgical cleft lip repair and primary rhinoplasty. It enhance the esthetic and functional outcome of the surgical procedures. The main purpose of the presurgical NAM is to reduce the severity of cleft deformity and for the effective surgical repair of the lip alveolus and nose. Other advantages includes retraction of the premaxilla, presurgical elongation of the columella, correction of the nasal cartilage deformity, alignment of the cleft alveolar segments, increase in the surface area of the nasal mucosal lining, up-righting of the columella, and achieving close approximation of the cleft lip segments at rest result from gentle application of forces through the NAM appliance. NAM technique has reduced and eliminated surgical scars associated with traditional columella reconstruction and has reduced the number and cost of revision surgical procedures, and has become the standard of care in this Cleft Palate Center. The purpose of this presentation is to discuss about the treatment technique and relavant advantages in detail.

532 10th World Cleft Lip, Palate & Craniofacial Congress

Application of Fisher method for cleft lip patients

1Hui Young Kima, 2Byoung Moo Seob 1Postgraduate student; 2Professor, Department of Oral and Maxillofacial Surgery, Seoul National University School of Dentistry & Dental Research Institute, Seoul 03080, Korea.

For the correction of cleft lip deformity, esthetics are one of the most important aspects in selecting surgical technique. Recently, Dr. David Fisher introduced his surgical technique for both unilateral and bilateral cleft lip patients with same surgical philosophy. Fisher method is unique and excellent surgical technique, which ensure esthetic outcomes in both unilateral and bilateral types of cleft lip regardless the extent of cleft severity. Most unique feature of his technique is medial incision line which runs parallel to the normal side of philtral ridge in unilateral cleft lip surgery. If the medial side of affected area is shorter than normal side, small triangular flap can be inserted from the lateral flap to increase the lip length in the medial side. Similar concept can be applied to the bilateral case with some modification. The authors applied Fisher technique with subtle modification on the vermilion border and additional adoption of orbicularis oris muscle overlapping nostril.suture for improving the philtral ridge prominence. Furthermore minimal invasive primary rhinoplasty was also utilized for correcting asymmetry on the

In this report, we present reasonable surgical outcomes of Fisher technique for the correction of unilateral and bilateral cleft lip patients.

533 10th World Cleft Lip, Palate & Craniofacial Congress

Saethre – Chotzen Syndrome: A rare congenital disease in a young South Indian Male

Catherine S. Chandran1, Saravanan Chandran2, Vivek Narayanan3 13rd Yr Post Graduate Student, 2Professor, 3 HOD & Dean - SRMKDC Department of Oral & Maxillo Facial Surgery, SRM Kattankulathur Dental College, Potheri, Chennai, Tamil Nadu, India

Saethre – Chotzen Syndrome, Dental Abnormalities Abstract:

Saethre – Chotzen Syndrome is an autosomal dominant genetic condition where there is premature fusion of the bones of the skull thereby affecting the shape of the skull and face. It is also associated with abnormalities of dentition, hands and feet. The signs and symp-toms of this condition may vary from person to person amongst the affected individuals. Genetic studies may reveal mutation of the TWIST I gene. Anodontia is the term attributed to the genetic disorder where there is absence of all teeth. Encountering the pure form is extremely rare. However, when it is present it is more of-ten than not associated with other abnormalities. Rare but more common are Hypodontia and Ol-igodontia. Hypodontia refers to the congenital absence of up to six teeth whereas Oligodotia is the term used to address a situation when more than six teeth are congenitally missing. The diag-nosis of the condition is primarily based on clinical findings. A combination of environmental and genetic factors can contribute to the occur-rence of dental agenesis. These include infection, trauma, drugs as well as genes associated with syndromes such as Cleft lip & Palate, Ectodermal Dysplasia, Down’s Syndrome etc.... We report a possible case with this rare syndrome affecting a young South Indian male in his early twenties. Complete and partial manifestations of thisKey syndromeWords have been re-ported in literature.

534 10th World Cleft Lip, Palate & Craniofacial Congress

TECHNIQUES IN CLEFT OSTEOTOMY

Nambinayaki.E.M 2nd year post-graduate,OMFS, Ragas Dental College & Hospital, Chennai

VEERABAHU.M MDS HEAD OF THE DEPARTMENT, Prof.Dr.J.A.NATHAN, Ragas Dental College and Hospital, Chennai, India

ABSTRACT

CONTENT:

Maxillary hypoplasia is often a consequence of labio-palatal repair. A lefort advancement is planned to mask these defects. This poster highlights on the techniques with which a patient is planned for a cleft osteotomy, presenting a case of HEMIFACIAL MICROSOMIA with previous repair of cleft lip and palate, treated for lefort 1 advancement.

535 10th World Cleft Lip, Palate & Craniofacial Congress

Alveolar graft before and after maxillary expansion in the cleft lip and palate patient

Evan .A. Clement Post-Graduate Resident, Department of orthodontics,, Ragas dental college & hospital,, Uthandi, Chennai-600119, Tamil nadu, India.

Abstract

Patients with cleft disorders present surgical and orthodontic challenges, treatment for these patients require co-ordinated efforts among many health professionals. The goal of the treatment is to provide normal function and esthetic appearance. Alveolar bone grafting is a vital part of the rehabilitation for cleft patients. The factors that have been most frequently associated with the success of the graft are the age of grafting and the pre-grafting orthodontic treatment. The operated lip and palate influence the maxillary growth frequently causing anteroposterior, vertical and transversal maxillary deficiency. The maxillary transverse deficiency specifically can be assigned to both the absence of the midpalatal suture and the restrictive effects of early lip and palate repair. In rehabilitation protocol maxillary expansion is necessary before secondary alveolar bone graft procedure; RME increases the maxillary dental arch, aligns the maxillary segment and provides room in the alveolar cleft for bone graft. So the poster will highlight the impact of alveolar bone graft before and after maxillary expansion among cleft lip and cleft palate patients.

536 10th World Cleft Lip, Palate & Craniofacial Congress

Management Whistle Deformity in bilateral Cleft lip

Dr.Geetha sridharan , Dr.Keerthana ponvel Guide: V.B.Krishna Kumar Raja, Sneha 1st year Post graduate,, Department of Oral and Maxillofacial Surgery,, SRM DENTAL COLLEGE,, Ramapuram, Chennai, India

ABSTRACT:

Whistle deformity is a common secondary deformity that is often seen in patients operated for bilateral cleft lip. This is primarily attributed to the muscle diastasis in the central segment of the lip. To achieve successful outcome, it is essential to achieve good muscle volume in the central segment without disrupting the adjacent facial sub units. Many techniques for the correction of whistle deformity have been followed in the past. However the surgeon always confronts with the problem of minimal quantum of tissue available for prolabial- columella correction. The aim of our presentation is to thereview same. the literature on the techniques for the correction of whistle deformity and describe the procedure that is performed at our unit for the correction of

537 10th World Cleft Lip, Palate & Craniofacial Congress

Project of Gene banking for Cleft Lip and/or palate patients in Mongolia

Hideto Imura1, Nagato Natsume1, Satoshi Suzuki1, Toko Hayakawa1, SHONKHUUZ ENKHTUR2, GONGORJAV AYANGA3 1Aichi-Gakuin University Hospital Cleft Lip Palate Center, Japan 2Mongolian National University of Medical Sciences, Ulaanbaatar, Mongolia 3Head of the Department of Maxillofacial surgery of National Center for Maternal and Child Health,Ulaanbaatar,Mongolia

The etiology of cleft lip and palate can be explained by a multifactorial threshold mechanism involving both genetic and environmental factors. However, there are still lots of uncertain points of details of its mechanism. We examine possibility of a clinical application by an animal model experiment under the hypothesis obtained for the results of the epidemiological investigation for 30 years. We are doing genetic analysis and epidemiological investigation from 15 years ago. At present, we are developing a system to perform storage of information not only blood but such as nails, cheek swab of saliva sample. There were reports of gene collection from saliva among them. We have 2166 blood samples in Mongolia. We think we would like to use for the gene screening. It's also reported that dairy products have the effect on prevention of a cleft lip and/or palate in Mongolia. I'd like to use for a genetic pleomorphy analysis for a screening of the patient who can't prevent a cleft lip and/or palate with dairy products in the future using the blood sample we'll possess from now on. This study was supported by a Grant-in-Aid for Scientific Research (Category A No. 24256006) from Japan Societ for the Promotion of science

538 10th World Cleft Lip, Palate & Craniofacial Congress

GIVE COMFORT FOR YOUR IMPLANTS ! - A COMPREHENSIVE CASE REPORT ON DENTAL IMPLANT REHABILITATION ON ILLIAC GRAFTED MAXILLA

Jahamgeer Badusha. S, A.Thangavelu Head, Dept of Maxillofacial Surgery, Rajah Muthiah Dental College & Hospital, Annamalai University, Tamil Nadu, India

Traumatic deformities of the jaws is increasingly become common due to rise in the incidence of high velocity motor vehicle accidents. The challenge of rehabilitating such patients involve restoring normal facial contour and esthetics and also giving them a functional occlusion for normal chewing. Here we present a case of 22 year old male patient with a traumatic secondary deformity of the left maxillary alveolus with loss of buccal cortical plate following Road Traffic accident. At the time of presentation he had a knife edged maxillary alveolus with severely compromised width that was not suitable for implant placement. In the first stage onlay bone grafting of Illiac bone to the Maxilla was done and nine months later implants were placed.The implants were loaded after another 6 months completing a comprehensive functional and esthetic rehabilitation.

539 10th World Cleft Lip, Palate & Craniofacial Congress

Velopharyngeal function following Mongolian method for primary palatoplasty

Ayanga.G, Bulgan.B, Davaanyam.L 1. Erdenetsogt Jargaldavaa 2. Bulgan Baasan 3. Ayanga Gongorjav 4. Davaanyam luvsandorj 5. Bayasgalan Rentsen 6. Tserendulam Dashnyam 7. Erdenesaikhan Magsarjav 8. Turbold Baatarsuren Head of the Department of Maxillofacial surgery, National centre for Maternal & Child health of Mongolia President of Mongolian Cleft Lip & Palate Association Vice president of Mongolian Association of Oral & Maxillofacial Surgeons

Huvisgalchdiin street, Bayangol district, Ulaanbaatar, Mongolia

ABSTRACT Introduction:

The timing and technique for palatoplasty is very controversial, recent literature advocates for early repair, between six and eighteen months of age, facilitating normal speech and language development, and avoiding hearing palatoplasty.loss. It is using three main kinds of techniques for palatoplasty in the world wide, two flap pushback, Furlow double opposing z-plasty, and two-step

In Mongolia patients often present in older than recommended age for palatoplasty, it provides surgeons with the challenge of managing wider defects, which have higher rates of fistula formation and wound dehiscence. There were 30% of ONF in our practice. So, in 2000 we established new modified Mongolian technique for primary palatoplasty for improved outcomes across allObjective: age groups. And ONF rate reduced from 30% to 5.9 % in our department.

To determine velopharyngeal function after primary palatoplasty by MongolianPatient and method methods: in connective with age at repair and cleft type.

In our study were included over four year’s old patients, who underwent primary540 palatoplasty by Mongolian method at the authors` centre. We excluded 10th World Cleft Lip, Palate & Craniofacial Congress

syndromic cases, and patients who had an oronasal fistula postoperatively, and no cooperation with speech therapist. This study, we use video records of nasopharyngeoscopy to evaluate for cleft lip and palate patient’s velopharyngeal function associated with type of clefts, the timing of palate repair and cooperation withResult: speech therapist.

There were included totally 50 patients who are 4-13 years old (mean age 7,8 years) at the time of study, were performed primary cleft palate repair by Mongolian technique at NCMCH. The mean age at palate repair was 25,8 months (14 months-69 months). Overall, 38% (19) of patients were performed palate repair at 18≥ months old and 62% (31) patients were operated palate repair at 18< months. In the first group, VPI was demonstrated 47%, and in method.the second group 51%. Also, there were demonstrated VPI for 80% of Veau 1 type, 85.7% of Veau 2 type and 33.4%-38.1% of Veau 3, 4 type, after Mongolian Conclusion:

There were not found statistical significant difference on the age of palatoplasty in relating with velopharyngeal function after Mongolian method. And better velopharyngeal result for Veau 3, 4 type and wide cleft palate after MongolianKey words: method than other types.

Mongolian method, palatoplasty, velopharyngeal function, type of clefts

541 10th World Cleft Lip, Palate & Craniofacial Congress

A systematic review on Rigid external distractors in Cleft patients:

Joji Alex1, Aravind Sivakumar2 1Post graduate student , 2Saveetha Dental College, Chennai, 2Senior Lecturer, Saveetha Dental College , CHennai, India

The aim of this study was to systematically review literature reporting on the use of external distraction osteogenesis (DO) and internal DO in the treatment of severe maxillary hypoplasia in cleft and palate patients. Literature research has been performed using the PubMed database of the National Library of Medicine and National Institutes of Health till September 2016. We used cleft lip and palate and distraction osteogenesis as key words. Of the 104 articles found , which reported on the correction of the maxillary hypoplasia with DO techniques. A total of 32 studies reported on anteroposterior external DO (27 studies on rigid external device and 5 on face mask), 17 studies reported on anteroposterior internal DO have been retained for this review. Despite the heterogeneity and methodological limitations of most of the studies, results showed that external DO with rigid external device and internal DO resulted to be a more reliable and accurate technique than the face mask in the management of severe maxillary hypoplasia in patients with cleft lip and palate. The current review demonstrated that external in the treatment of severe maxillary hypoplasia in cleft and palate patients (1) is a reproducible and valuable alternative to standard orthognathic surgery procedures, (2) allows for a global improvement in facial aesthetic, (3) allows a maxillary correction in patients during the period of mixed dentition, and (4) allows either for an unchanged or better velopharyngeal function.

542 10th World Cleft Lip, Palate & Craniofacial Congress

Distraction Osteogenesis: Treatment Option For Facial Cleft Repair

Adam KB, Hariri F, Rahman ZAA Oral & Maxillofacial Surgery Unit, Kulliyyah of Dentistry, Kuantan Campus, International Islamic University Malaysia, Indera Mahkota, 25200 Kuantan, Pahang, Malaysia

Abstract

Introduction: Oblique facial clefts are an extremely rare disorder, accounting for only 0.24% of facial clefts. The condition causes pronounced facial deformity which can lead to major cosmetic and functional problems. Due to its nature of complexity, it becomes one of the reconstructive challenges which require careful planning and multiple surgeries throughout life. In this case report, we would like to share our experience with orbital reconstruction byMethod: means of distraction osteogenesis in a 1 and a half-year-old girl.

A 3 months old female infant was referred to our department for the management of left facial cleft. Soft tissue cleft was repaired at 4 months old followed by left orbital reconstruction via left infraorbital rim osteotomy and vertical distraction osteogenesis at the age of 1 and a half years old using alveolar distractor, as a modification. The procedure was indicated to achieve adequateResult: left eyelid closure and symmetrical orbital level.

Adequate eyelid closure, satisfactory cosmetic outcome, stable result of symmetrical eye level and good orbital volume was observed at 12 months post-surgical review.

543 10th World Cleft Lip, Palate & Craniofacial Congress Discussion:

Hard tissue reconstruction is useful in the treatment of oblique facial cleft, but opinion remains divided with respect to timing and technique of the reconstruction. Distraction osteogenesis may offer a good treatment alternative thatKeywords: offers stable and predictable results.

Facial cleft, Distraction osteogenesis, maxillofacial surgery.

544 10th World Cleft Lip, Palate & Craniofacial Congress

Long-term Follow-up of Early Cleft Maxillary Distraction

Young-Wook Park1, Min-Keun Kim2 1Department of Oral and Maxillofacial Surgery, 2College of Dentistry, Gangneung-Wonju National University

Abstract Introduction

Most of cleft lip and palate patients have the esthetic and functional problems of midfacial deficiencies due to innate developmental tendency and scar tissues from repeated operations. In these cases, maxillary protraction is requiredMaterial forand the method harmonious facical esthetics and functional occlusion.

A seven-year old boy had been diagnosed as severe maxillary constriction due to unilateral complete cleft lip and palate. The author tried to correct the secondary deformity by early distraction osteogenesis with the aim of avoiding marked psychological impact from peers of elementary school. From 1999 to 2006, repeated treatments, which consisted of Le Fort I osteotomy and face mask distraction, and complementary maxillary protraction using miniplates were performed including orthodontics. But, final facial profile was not satisfactory,Result and Discussion which needs compromising surgery.

The result of this study suggest that if early distraction treatment is performed before facial skeletal growth is completed, a orthognathic surgery or additional distraction may be needed later. Maxillofacial plastic illary deformity. and reconstructive surgeons should notify this point when they plan early distraction treatment for cleft max 545 10th World Cleft Lip, Palate & Craniofacial Congress Key words:

Unilateral complete cleft lip and palate, Maxillary constriction, Early distraction treatment

546 10th World Cleft Lip, Palate & Craniofacial Congress

CORRECTION OF NASAL SEPTAL DEVIATION IN CLEFT LIP PATIENTS - A COMPARISON OF TWO TECHNIQUES

M.LAVANYAA; DHIVYAA.A. ACTA oTorhinolAryngologiCA iTAliCA 2013;33:146-153 The role of septal surgery in cosmetic rhinoplasty Il ruolo della chirurgia del setto nella rinosettoplastica C. PARRILLA, A. ARTUSO, R. GALLUS, J. GALLI, G. PALUDETTI Department of Head and Neck Surgery - Otorhinolaryngology, Catholic University of Sacred Heart, Rome, Italy

Abstract:

Nasal septal deviation is one of the key features of unilateral cleft nasal deformity. Correction of the caudal septum is essential to achieve good projection of the nasal tip complex and the nasal dorsum. Apart from this, septoplasty is essential to correct the airway obstruction in cleft patients. The quantum of septal kinking is extreme in patients with CLAP and conventional techniques of sub mucosal resection and repositioning may be inadequate, leading to relapse of the caudal septal deviation (secondary to warpage). The issue with the warpage of the septal cartilage can be overcome either by septal weakening or by enhancing the septal support with septal onlay graft. Only few studies have compared the post- surgical stability of the nasal septal complex by any of the conventional methods in cleft lip palate population. The aim is to compare the post- surgical stability of the nasal septum corrected and stabilized by the conventional technique versus septal support with a cartilage onlay graft in patients undergoing cheilo-rhinoplasty.

547 10th World Cleft Lip, Palate & Craniofacial Congress

TESSIER’S CLASSIFICATION OF CLEFTS AND SURGICAL TREATMENT PLAN FOR EACH TYPE

M.THENARUVI Post Graduate Resident, Department of Oral & Maxillofacial Surgery, Thai Moogambigai Dental College and Hospital, Chennai, India

ABSTRACT :

A cleft is a gap in the soft tissue, bone, or both.Clefts may involve the mouth, cheeks, eyes, ears and forehead and may continue into the hairline. These craniofacial clefts are often referred to as Tessier clefts. They are numbered from 0-14 to indicate the location and extent of the cleft using the mouth, nose and eye sockets as landmarks, with the midline designated 0. These more extensive conditions may also be described anatomically, such as “oro-occular cleft” and “fronto-nasal dysplasia.”This poster presents the 15 types of clefts with a concise surgical managment plan for each type.

548 10th World Cleft Lip, Palate & Craniofacial Congress

INNOVATIVE MATERIALS IN INTEGRITY OF CLEFT REPAIR

Manoj Kumar Musunuri Chennai, India

Abstract: with a gap in the upper lip and roof of the mouth. It originates from failure in the Cleft lip and palate are congenital deformities of the craniofacial region fusion of oronasal processes within the first 5-6 weeks of gestation. Numerous bone and soft tissue grafting techniques are followed to repair cleft lip and palate defects. In addition to the gold standard surgical interventions involving the use of various autogenous, allogenic and xenogeneic graft materials used for bone repair, there are minimally invasive treatments to repair cleft defects by using Innovative Biosynthetic Materials. This poster highlights the range of current materials available for cleft repair that are used, tested or explored for the repair of cleft defects.

549 10th World Cleft Lip, Palate & Craniofacial Congress

Orthomorphic Correction of mandibular Asymmetry with Reverse sagittal split Osteotomy- A Case Report

Manu Prasy 3rd Year Post graduate in Oral and Maxillofacial surgery , Saveetha Dental College M.R.Muthushekhar Head of the department, Dept of Oral and Maxillofacial surgery, Saveetha Dental College

A patient name Banu aged 21,f reported to Department of oral and maxillofacial surgery, Saveetha Dental College and Hospital complaining of asymmetrical facial appearance and increased upper teeth visibility. Patient gives a history of previous surgery of TMJ Ankylosis when she was 9 years old. On facial evaluation it was found that she had increased vertical midface height as well as deviation of mandible towards the right side. A treatment of Anterior maxillary osteotomy for maxilla and reverse sagittal split osteotomy on right side for mandible was planned. Distraction was not considered because patient wanted immediate results. Post operatively, satisfactory facial appearance was achieved.¬ Reverse sagittal split Osteotomy is a versatile technique with immediate results in the correction of mandibular asymmetry when other treatments are contraindicated.

550 10th World Cleft Lip, Palate & Craniofacial Congress

Characterization of common tissue events in cleft lip palate affected tissue in children

Mara Pilmane, Ilze Akota¹ Institute of Anatomy and Anthropology, Stradins University, Riga, Latvia, [email protected]; Dzirciema street 16, Riga, ¹Institute of Stomatology, Riga Stradins university, Riga, Latvia, [email protected], Dzirciema street 16, Riga,

Introduction

Cleft lip palate (CLP) is the second most common anomaly among alive born newborns. Growth factors and their receptors, genes and their proteins, also undifferentiated cells are mentioned to play a role in the morphopathogenesis of CLP especially during the embryogenesis. Despite tissue is given similar response for the different irritants, there are not clearly described tissue events regarding general structural changes in the expression of above mentioned factors. Thus, aim of this study was research for the common featuresMaterial characteristicand Methods for CLP affected soft and hard facial tissue in children.

Tissue obtained from plastical surgery in 40 children of different age with CLP was prepared for the routine histology and stained also for the immunohistochemistry with MSX1, PAX9, Ki67, HoxB3, Ryk, TGFbeta, TGFbetaR3,Result and Discussion CD34, nestin.

TGFbeta and TGFbeta3R almost was not seen into the soft tissue. However, the cartilage and bone richly expressed these factors. MSX1 was absent in the bone, but marked epithelial cells especially in the mixed dentition age, when also numerous nestin-containing cells were observed. CD34 cells were detected mainly in blood vessel of children before and in milk dentition age. PAX9 and Ryk positive cells were seen in epithelium and also in connective tissue in CLP affected tissue of both dentition age and different type of cleft. Part of children demonstrated long epithelial processes showing PAX9, MSX1, NGFR and HoxB3, but not Ki67 immunoreactivity. 551 10th World Cleft Lip, Palate & Craniofacial Congress Conclusions

CLP affected soft and hard tissue are able to compensate lack of some factor expression for each other. Appearance of neuronal and mesenchymal primordial cells in CLP affected tissue depends on the dentition age. Oral epithelium is the dominating expression place for PAX9 and Ryk in case of cleft. CLP originates development of epithelial outgrowths positive for cellular differentiation and migration, but not proliferation markers.

552 10th World Cleft Lip, Palate & Craniofacial Congress

EFFECT OF NASO ALVEOLAR MOULDING ON DENTO ALVEOLAR ARCH

Mary Sheloni Missier Chennai, India

Introduction

Nasoalveolar moulding is done a presurgical treatment which is started within Result: one to two weeks after birth to improve the final results of surgery.

There was a statistical reduction in width of the large cleft and increase in bilateralDiscussion: width and columellar length and width surgery it was found that it resulted in the improvement of the final results of

553 10th World Cleft Lip, Palate & Craniofacial Congress

Rare cases of congenital anomalies of the nose.

Nataliya Tetrueva National Children Specialized Hospital OHMATDYT, Kiev/Ukraine

We would like to share our experience with treatment rare cases of congenital anomalies of the nose. The presentation include 5 cases studies of unusual occurrences of nasal anomalies - proboscis lateralis (PL) and lateral nasal cleft. We would welcome a discussion on potential methods of operations on such patients. Proboscis lateralis(PS) is rare congenital anomaly , which is embryologically related to the medial facial cleft. The report insidence of PL is fusionless then line one between per 100.000 live births. A tube -like rudimentary nasal structure is located off center from the vertical midline of the face, along the embrionic

the anterior maxillary process and the frontonasal process. PL usually associated with heminasal hypoplasia or aplasia. Khoo -Boo-Chai collected 34cases from the literature, classified them into 4 groups: 1.PL with normal nose 2.PL with nasal defect only 3.PL with nasal defect, defect of eye and adnexa 4.PL with nasal defect ,abnormality of the eye,adnexa,cleft lip and palate. The lateral nasal cleft is very rare malformation. Kawamoto reported incidence of these atypical clefts as 1,43 to 4,85 per 100.000 births. Losee classification of congenital nasal anomalies: Type 1- hypoplasia and atrophy Type2-hyperplasia and duplication 554 Type 3 -clefts (include Tessier nasal cleft) 10th World Cleft Lip, Palate & Craniofacial Congress

Type 4- neoplasms and vascular anomalies. We present 2 cases of PL with defect of eye and adnexa - group 3 to the classification of Boo-chai. Surgical reconstruction of the left heminose was done using the PL.. abnormalien nostril 1 case of PL with nasal defect only . We plan to perform dilation of the

1 case with cleft left nostril - type 3 according Losee..Surgery using rotation flap and Z plastic. PL and lateral cleft of nose are rary anomalies.The treatment of this anomalies must be individualize , because there is some variability in this malformation and the degree of nasal hypoplasia. Reconstruction of this anomalies . must be multidisciplinary and include ophathalmologist , otorhinolaryngologist ,maxillofacial surgion , and plastic surgion.

555 10th World Cleft Lip, Palate & Craniofacial Congress

BILATERAL CLEFT LIP REPAIR WITH DELAIRE & BOB MULLIKEN TECHNIQUES

S.Nirmal Raj Post Graduate, OMFS, Ragas Dental College and Hospital, Chennai, India Guided by: M. Veerabahu, J. A. Nathan

Abstract

The objective for surgical correction of the bilateral cleft lip is to reconstruct a symmetrically balanced lip and nose with good columellar length. The most common approach is a two-stage correction with columella elongation as a secondary procedure at the age of 1 to 5 years. Simultaneous surgical correction of bilateral cleft lip and nasal deformity is becoming more common. This is a major change from the conventional strategy of secondary nasal correction. This poster discusses about the bilateral cleft lip repair with delaire & bob mulliken techniques

556 10th World Cleft Lip, Palate & Craniofacial Congress

Dermatoglyphics - a diagnostic tool

Nithya Jagdish Department of Orthodontics, Faculty of Dental Sciences,Sri Ramachandra University,Chennai, Tamilnadu, India

Dermatoglyphics (Greek derma= skin, glyph= carve) is the scientific study of fingerprints, lines, mounts and shapes of hands. The epidermal ridges of the finger and palms as well as structures like the lip, alveolus and palate are formed from the same embryonic tissues (ectoderm) during the same embryonic period (6-9 weeks). Thus the genetic and environmental factors which are responsible for causing cleft lip and palate may cause peculiarities in dermatoglyphic patterns. Dermatoglyphic analysis is an extremely useful tool for preliminary investigations into conditions with a suspected genetic basis. This poster will highlight on dermatoglyphics in relation to cleft lip and palate and the various findingsReferences: from the literature. 1. De Bei et al. Dermatoglyphic analysis of primary and secondary cleft palate patients. Cleft Palate Journal, July 1977, vol. 14, No.3, 222-225. 2. R.S.Balgir. Dermatoglyphic in cleft lip and cleft palate anomalies. Indian Journal of Paediatrics, March 1993,vol. 30, 341-346. 3. Lakshmi Prabha.J et al /J. Pharm. Sci. & Res. Vol. 6(4), 2014, 200-202. 4. Admala NR, Arjunan S Adusumilli G, Thirumala JR, Devanna R, Pichai S. Dermatoglyphics and Cheiloscopy in the Inheritance of Cleft Lip and Palate: Unraveling the Mystery. J Ind Orthod Soc 2014;48(3):175-179. 5. Saujanya K, Prasad M G, Sushma B, Kumar J R, Reddy Y, Niranjani K. Cheiloscopy and dermatoglyphics as genetic markers in the transmission of cleft lip and palate: A case- control study. J Indian Soc Pedod Prev Dent 2016;34:48-54.

557 10th World Cleft Lip, Palate & Craniofacial Congress

Morphologic variability in nonsyndromic operated patients affected by cleft lip & palate : Cephalometric study

Rajesh Murugan Post-Graduate Trainee, Dept. of Orthodontics, Shree Balaji Dental College and Hospital, Chennai, amil Nadu, India INTRODUCTION

Oral clefts are common congenital deformities occurring worldwide at about ratio of 1:500. Two main types of cleft are recognized and has been distinguished from each other, embryologically and genetically - A cleft lip with orMATERIAL without cleft AND palate METHODS and an isolated cleft palate .

The sample comprised of lateral cephalographs of 20 persons : 10 (female) patients with nonsyndromic, operated cleft lip and palate ( OpC Group ) ; and 10 (female) control subjects with class I dental occlusion and with an overall harmonious skeletal and soft tissue profile (C Group ). CephalometricRESULT landmarks traced to capture key anatomic features.

Morphological variability is significant in the Op C Group than in the C GroupDISCUSSION

In this study we compared the morphologic variability of Op C Group with C Group . Op C Group subjects had retrusive maxilla , anterior cross bite and reduced height of the posterior region of upper face ; whereas C Group had no remarkable variation .

558 10th World Cleft Lip, Palate & Craniofacial Congress

Facial profile and maxillary arch dimensions in unilateral cleft lip and palate children in the mixed dentition stage V K Gopinath a, AR Samsudin a, S.N. Fazliah Mohd Noor b, HY Sharab b a College of Dental Medicine, University of Sharjah, Sharjah (UAE),b School of Dental Sciences, Universiti Sains Malaysia, Malaysia. Abstract: Cleft lip and Palate (CLP) are the most common congenital malformation in the craniofacial region. Aim: The aim of this study is to evaluate the vertical and sagittal facial profile and maxillary arch width, depth and length of unilateral cleft lip and palate (UCLP) patients and to compare them with healthy non-cleft children in the mixed dentition stage (7-13 years). Material and Methods: This is a retrospective study conducted at Hospital University Science Malaysia (HUSM). UCLP group consisted of 48 non-syndromic UCLP patients who have had the lip and palate repaired while the control group consisted of 48 healthy non-cleft subjects. The lateral cephalometrics measurements were used to determine the vertical height, sagittal depth of the face and cranial base length and angle. Maxillary arch dimensions were measured on the study cast include arch width, depth and length. Results: Vertical facial height and sagittal depth measurements showed significant decrease (P< 0.05) in the mean growth pattern in UCLP group. The anterior cranial base length (S-N) was shorter in UCLP children (p<0.001) while Ba-N length had no significant difference (p = 0.639). Nasion-Sella Tursica- Basion angle was significantly higher in the UCLP group (P= 0.016). Dental arch width with reference to canine to canine and 1st premolar to 1st premolar distance was significantly larger in control (p=0.001). Conclusions: Mean mid- facial and lower facial dimensions in the UCLP children who does not undergo orthodontic treatment are significantly lesser in all directions of growth than healthy non-cleft children. The maxillary dental arch has a normal depth but constricted in width and arch length. Key words: Cleft lip and palate, Facial profile, Maxillary arch dimensions

559 10th World Cleft Lip, Palate & Craniofacial Congress

Alveolar bone grafting Reetesh Navani Post-Graduate Resident, Dept. of Oral and Maxillofacial Surgery, RDVV, Jabalpur, Madhya Pradesh, India

560 10th World Cleft Lip, Palate & Craniofacial Congress

Benefits And Limitations Of Nasoalveolar Moulding Appliance (Nam) : A Scientific Review

Remmiya Mary Varghese Post-Graduate Resident, Dept. of Orthodontics, Saveetha Dental College, Chennai, Tamil Nadu, India

Abstract : Introduction:

Cleft Lip And Palate Is The Most Common Congenital Defect Of The Oral Cavity Which Varies In Its Form And Severity. Presurgical Infant Orthopedics Has Been Used For Centuries For The Treatment Of Cleft Lip And Palate. The Recently Advocated Nasoalveolar Molding(Nam) Represents A Shift From The Traditional Methods Of Presurgical Infant Orthopedics And Has Gained More AttentionObjective: As It Improves Facial Appearance And Function.

To Ascertain The Fact That The Benefits Of Nam Outweigh The Limitations Of The Procedure And It Should Be Considered The Treatment Of Choice To IMethodology: m p r o v e T h e F u n c t i o n a l A n d E s t h e t i c N e e d s O f A C l e f t P a t i e n t .

The Nasoalveolar Molding (Nam) Technique Utilizes A Wire And Acrylic Nasal Stents Attached To An Intraoral Denture. This Appliance Is Used To Mold The Nasal Cartilages, Premaxilla And Alveolar Ridges Into Normal Form And PositionBenefits: During The Neonatal Period.

Nam Is Intended To Reduce Severity Of The Oronasal Deformity Prior To Surgery ,Non Surgical Lengthening Of The Columella. It Also Eliminates The Surgical Scars Associated With Traditional Columella Reconstruction And Has Reduced The Number And Cost Of Revision Surgical Procedures And Bone Grafts.

561 10th World Cleft Lip, Palate & Craniofacial Congress Limitations :

Irritation Of The Oral Mucosa, Inflammation Of The Intranasal Lining, Notching Occuring Along The Alar Rim, Chance Of Ulcerations, Premature EmergenceConclusion: Of The Labial Surface Of Maxillary Decidous Central Incisors.

Nam Has Evolved Over The Past Decade Into Its Present Form And There Has Been A Significant Difference In The Outcome Of The Primary Surgical Cleft Repair Compared With Other Techniques Of Presurgical Orthopedics. Studies Show That The Nasal Shape Is Stable With Better Lip And Nasal Form. Despite Its Limitations, Nam Appears To Be A Promising Technique For The Treatment Of Cleft Lip And Palate Patients.

562 10th World Cleft Lip, Palate & Craniofacial Congress

DENTAL AND SKELETAL ASYMMENTRIES FOR ORTHOGNATHIC SURGERY

S.I.P. SHEIK ARIFA Chennai, India

Asymmetry in the face and dentition is a naturally occuring phenomenon. In most cases facial asymmetry can only be detected by comparing homologous parts of the face. The etiology of asymmetry includes: a) Genetic or congenital malformations e.g. hemifacial microsomia and unilateral clefts of the lip and palate; b) Environmental factors, e.g. habits and trauma; c) Functional deviations, e.g. mandibular shifts as a result of tooth interferences. Dental asymmetries and a variety of functional deviations can be treated orthodontically or surgically. On the other hand, significant structural facial managementasymmetries areat a not later easily point amenable if mandatory to orthodontic . treatment. These problems may require orthopaedic correction during the growth period and/or surgical

Patient complaints and desires need to be addressed since they may vary from unrealistic expectations to a lack of concern even in the presence of large deviations. With mild dental, skeletal and soft tissue deviations the advisability of treatment should be carefully considered, planned and executed for better outcome.

563 10th World Cleft Lip, Palate & Craniofacial Congress

VERMILION FREE BORDER RECONSTRUCTION A NOVEL TECHNIQUE

Sivaiah Sarnu Post Graduate, OMFS, Ragas Dental College and Hospital, Chennai, India Guided by: M. Veerabahu, J. A. Nathan

Abstract:

Aesthetic outcome of repaired unilateral cleft lip is assessed by evaluating nose, body of the lip and vermilion free border. Vermilion-free border is evaluated for continuity of white line, red line, Cupid's bow symmetry, equal width of dry vermilion and absence of retraction notches in static and function. This paper discusses about a novel technique of vermilion free border reconstruction.

564 10th World Cleft Lip, Palate & Craniofacial Congress

OBSTRUCTIVE SLEEP APNOEA IN THE CLEFT POPULATION

Stephan Rajkumar

Guided by: M. Veerabahu, J. A. Nathan Post Graduate, OMFS, Ragas Dental College and Hospital

Abstract:

Cleft lip & palate are common congenital deformities which results from clefting or discontinuity in the normal embryological union of lip and palate occurring individually or together in some cases. These patients could suffer from various sleep disordered breathing owing to their compromised airway space. Obstructive sleep apnoea, which is the most common type of sleep apnoea results from partial or complete obstruction of the airway & can affect the overall well being. Changes in the sleeping pattern can have an adverse effect on the health of an individual including children. Various efforts have been taken to understand the disorder in a better way. This poster sheds light on the fact that many cleft patients suffer from obstructive sleep apnoea, the presenting problems and the efforts taken to overcome them.

565 10th World Cleft Lip, Palate & Craniofacial Congress

PRENATAL DETECTION OF CRANIOFACIAL ANOMALIES

S.V.HAYMENTH Post-Graduate Resident, Dept. of Oral & Maxillofacial Surgery, Thai Moogambikai Dental College and Hospital, Chennai, Tamil Nadu, India

Sophisticated prenatal diagnostic imaging has facilitated the progression of fetal surgery from the experimental possibility to a clinical therapy for several life-threatening congenital conditions. Guidelines for such fetal surgery include the expectation that the child will be reasonably healthy as a result and that the in utero approach will improve the outcome or is safer than postnatal intervention. Prenatal ultrasound detection of craniofacial anomalies is now common. Reports of experimental cleft repairs in animal fetuses suggest advantages including diminished scarring, improved health and nursing. While fetal craniofacial surgery has not been attempted in humans, speculations exist about the psychological and social benefits of being born with a repaired defect. Fetal surgery is an emerging technology that may alter experimentation, cleft treatment, and the allocation of scarce resources. This paper examines the social and ethical implications of prenatal diagnosis and fetal surgery, focusing on fetal/maternal rights, and clinical decision making.

566 10th World Cleft Lip, Palate & Craniofacial Congress

The GOSLON Yardstick in Patients With Unilateral Cleft Lip and Palate- A SYSTEMATIC REVIEW

Swetha Sridharan Post Graduate student,, Saveetha Dental College,, Chennai, Tamil Nadu.

ABSTRACT Background:

Several indices are now available to assess the severity of the malocclusion in cleft lip and/or palate (CLP) patients; and although it has been quite some time since the introduction of these indices, there is no consensus as to which indexObjective: should be used for CLP populations.

To systematically review the available literature on the Goslon index usedSearch to assessmethods: the occlusal schemes in dental models of CLP patients

Ten electronic databases, grey literature, and reference list searches wereSelection conducted. criteria:

The inclusion criteria consisted of studies that uses Goslon’s index to assess a particular malocclusion index on study models of patients with CLP. Data collection and analysis: Full articles were retrieved from abstracts/ titles that appeared to have met the inclusion -exclusion criteria which were subsequently reviewed using more detailed criteria for a final selection decision. The Quality Assessment of Diagnostic Accuracy Studies tool was used to appraise the methodological quality of the finally included studies. Due to the heterogeneity of the data, only a qualitative analysis was performed.

567 10th World Cleft Lip, Palate & Craniofacial Congress Results:

A total of 7 studies met the inclusion -exclusion criteria. These studies revealed seven utilized indices, namely the GOSLON Yardstick, The GOSLON Yardstick was the most commonly used index, and the Modified Huddart -Bodenham performed the best according to the WHO criteria. Conclusions: Current evidence suggests that the GOSLON Yardstick was the most commonly used index, possibly due to a longer time in use. It is also considered to be a reliable index

568 10th World Cleft Lip, Palate & Craniofacial Congress

The trial telepractice for cleft palate speech between Mongolia and Japan

Toko Hayakawa, Kyoko Ozaki, Nagato Natsume, Hideto Imura, Fuko Yamauchi, Chisato Sakuma, Maya Ono, Akihiro Mori, Shinichi Nakahara, Masahiko Yamamoto Aichi Gakuin University, Japan

Since 2007 we have been providing telepractice as part of the actual training session for patients with cleft speech in Japan. There were not many easy-accessible applications for telepractice in the earliest years; hence telepractice could not be widely used. On the other hand there are many free applications which are easy to access such as LINE, FaceTime and Skype in recent years. It should be clear enough to be able to assess patients’ speech through speaker and mike. We do have enough clinical experience of telepractice among Japan, and the experiences can conclude those widely used applications are usable for telepractice in Japan. We have been performing charitable work and bringing up speech therapists in Mongolia for a long time and we have built a speech center in Mongolia. As well known, Mongolian landscape is large and some patients with cleft speech have difficulty to access a speech therapist around their residence. To develop the speech assessment and therapy system through telepractice in Mongolia, we had the trial telepractice between Mongolia and Japan if the widely used application of LINE is enough assessable for cleft speech. We used two cleft speech samples with 1) glottal stops and very mild hypernasality and 2) distortion from nasal emission with moderate hypernasality. Those two samples could be properly assessed in Japan through LINE and the speaker. It may indicate that telepractice through widely used applications can be performed across countries.

569 10th World Cleft Lip, Palate & Craniofacial Congress

Naso – Alveolar Moulding

Veerasankar S Department of Orthodontics,, Ragas dental college & hospital,, Uthandi , Chennai – 600 119, Tamilnadu , India

Introduction:

The deformities of nasal cartilage in unilateral and bilateral cleft lip and palate patients leading to loss of nasolabial fold after accomplishment of treatment.Hence pre surgical infant orthopaedics has been employed as an adjunctive neonatal therapy for their correction. Nasoalveolar molding (NAM) technique has reduced the severity of initial cleft alveolar and nasal deformity. Long term studies on NAM therapy indicate better lip and nasal form, reduced oronasal fistula and labial deformities, 60% reduction in the need for secondary alveolarObjective: bone grafting. lip and palate patients. To ascertain the significance of nasoalveolar moulding technique in cleft Material & Methods:

A search was conducted through internet and articles about nasoalveolar moulding technique in the treatment of cleft lip and palate deformity.A proper alignment of the alveolus, lip and the nose helps the surgeon to achieve a better and more predictable surgical result.The cleft deformity is significantly reduced in size with the NAM therapy before surgery, making primary repair of the lip, alveolus and the nose an effortless procedure.Studies have also demonstrated that 60% of patients who underwent NAM and gingivoperiosteoplasty did not requireConclusion: secondary bone grafting.

Long-term studies of NAM therapy indicate that change in nasal shape is stable with less scar tissue and better lip and nasal form.With the alveolar segments in a better position and increased bony bridges across the cleft, the permanent teeth have a better chance of eruption in a good position with adequate periodontal support.Since the initiation of NAM, there has been a significant difference in the outcome of primary surgical cleft repair.NAM has 570 10th World Cleft Lip, Palate & Craniofacial Congress

demonstrated tremendous benefit to the cleft patients as well as to the surgeon performing the primary repair.This enables the surgeon and patient to enjoy the benefits associated with repair of a cleft deformity that is minimal in severity

571 10th World Cleft Lip, Palate & Craniofacial Congress

PRENATAL DIAGNOSTIC MARKERS OF CLEFT LIP AND PALATE

Venkateswaran Ananthanarayanan Professor of Orthodontics, Tamil Nadu Dr.MGR Medical University, Chennai, India

ABSTRACT

Facial clefts, pertaining to the lip and palate, are one of the most common congenital malformations with an incidence rate of around 35000 birth a year in India. They may be part of a syndromic condition or may occur in isolation. Due to its multifactorial etiology, the stakes are high to detect the condition as early as possible. Every opportunity to sensitize the parents of the child is a boon which can be followed by pre and post-natal counselling. Ultrasonography has been one of the routine procedures in every prenatal checkup and thus can be made use of for early detection of cleft lip and palate. Apart from this, genetic predispositions have also steered researchers to finding out the genetic abnormalities associated with cleft lip ad palate This poster will highlight the genetic, ultrasonographic and other markers which are associated with cleft lip and palate and thus can be useful in their early detection.

572 10th World Cleft Lip, Palate & Craniofacial Congress

A case of extremely low birth weight infant with cleft lip and palate (2nd report)

Yukiko Morita, Yuji Fujimura, Hitoshi Hotokezaka, Jun-ya Tominaga, Keira Arizono, Seiko Iijima, Noriaki Yoshida Department of Orthodontics and Dentofacial Orthopedics, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. Introduction:

Extremely low birth weight infants (ELBWI) are infants weighting less than 1,000 g. Because these infants often have suckling disorders, they need to acquire oral feeding ability before hospital discharge. Previously, we reported a case of ELBWI with cleft lip and palate (CLP) treated with Hotz plate. In this report,Case Description: we present the growing process of the patient. The male

patient was born at 22 weeks of gestation by emergency cesarean section due to non-reassuring fetal status. His birth weight was 844 g. Respiratory distress syndrome, incomplete cleft lip of right side and cleft palate were found. He took in nutrishment by oro-gastric tube feeding. At 47 days after birth, his body weight increased to 1,380 g. An impression for Hotz plate was taken and set on the same day at NICU, and he acquired oral feeding ability with Hotz plate. He was discharged from NICU at 98 days his weight reached 2,700 g. Cleft lip surgery was performed at 6 months old and the following cleft palate surgery was performed at 17 months old. At 5 years old, he was examined to diagnose for orthodontic treatment.His physical growth and development including speech ability were approximately normal. On the other hand, the anteroposterior skeletal relationship of maxilla and mandible was deviated from the standard, and unilateral crossbite was observed. Quad helix was used to correct maxillary constriction, and face mask was planned to useSummary: for maxillary protraction.

Hotz plate effectively improved the sucking behavior of ELBWI with CLP, which may have facilitated his weight gain and enabled the cleft palate surgery to be carried out at the ordinary age. After several years follow-up, the orthodontic treatment was started. His physical growth approximated to the average and the regular treatment plan for CLP patients was decided. 573 10th World Cleft Lip, Palate & Craniofacial Congress

Iliac bone graft and osseointegrated implants

Gauri Gupta I Post-Graduate Resident, Dept. of Oral & Maxillofacial Surgery, Saveetha Dental College, Chennai, Tamil Nadu, India

NTRODUCTION:

Cleidocranial Dysplasia is an uncommon,generalised skeletal disordercharacterised by the delayed ossification of skull,aplastic or hypo plastic clavicles and serious complex dental abnormalities. This case report ofdiscusses life. the treatment of 24 years old south Asian man with Cleidocranial Dysplasia who had significant dental problems that greatly affected the quality MATERIALS AND METHODS:

Pre-operative CBCT and OPG was taken,then procedure was done under General Anaesthesia to remove all impacted teeth(17 in number). It was an interdisciplinary approach;clear self cure acrylic stent was used to decide the width of iliac graft to be placed in maxilla and mandible.Ridge Split(maxilla,mandible) was done and iliac bone graft was placed.Review was doneRESULTS: after 6 months.Intraossous implants were placed in both the ridges.

DISCUSSION:Results were satisfactory in terms of function and esthetics.

Since early diagnosis of Cleidocranial Dysplasia is essential for choosing the appropriate treatment approach,clinicians should be aware of its characterstic features.

574 10th World Cleft Lip, Palate & Craniofacial Congress

Role of 3D Printed skull models in complex craniofacial surgeries -

Narendra Reddy Chittamuru, PG student, Dept. of Oral & Maxillofacial Surgery, HOD & Prof Dr. Krishna Shama Rao

Introduction:

In medical practice, the usage of 3D printed skull models has definitely proven its efficacy. Patient-specific 3D printed skull models are extremely useful for the pre-operative planning of complex craniofacial surgeries, as they can help you assess the situation and decide on a plan of action before entering the operating room. In addition, they felicitate communication with patients and fellow physicians. Because of 3D printing, it is possible to replicate any Craniomaxillofacial foranatomical surgery. structure and provide you with a physical 3D model, printed using qualified system, which can help evaluate the patient's anatomy and prepare Materials and Methods:

Data obtained from the computed tomography (CT) scans with 1-mm resolution were converted into a computer-aided design (CAD) using the CT Digital Imaging and Communications in Medicine (DICOM) data. Once a CAD model was constructed, it was converted into a Selective Laser Sintering(SLS) format and then processed by the rapid prototyping technology to produce the physicalResult: anatomical model using a resin.

By employing 3D skull models in complex Craniofacial Surgeries, we obtained accurate 3D Printed Skull models. These also reduced the time period ofDiscussions: Surgeries.

Outcome of surgery depends not only on Technical aspect of Surgery but also on surgical plan. perfect surgical plan involves detailed imaging and modeling that can accurately replicate these anatomical detailed areas starting575 10th World Cleft Lip, Palate & Craniofacial Congress with simple radiographs many modern acquisition systems such as CT and MRI which provide the surgeon with detailed reconstructions of patient anatomy in combination with advances in Imaging processing. 3D printing is an advanced technique which involves duplicating structures layer by layer which gives precision to the technique.

576 10th World Cleft Lip, Palate & Craniofacial Congress

“GEOMETRIC COMPENSATIONS”

Manay Roshini Srinivas, IInd year, Post Graduate trainee, Department of Oral and Maxillofacial Surgery, Saveetha Dental College, Chennai, Tamil Nadu.

ABSTRACT INTRODUCTION:

Orthognathic Surgery for the correction of facial deformity arising from discrepancy in spatial relationship is well established. However, when the osteotomycause of the is deformity done. includes a functional impairment, Orthognathic surgery alone will not suffice. In such cases, morphometric analysis for orthomorphic MATERIAL AND METHOD:

This poster highlights a case report on a patient diagnosed with obstructiveTREATMENT: sleep apnoea due to unilateral TMJ ankyloses and facial deformity. was done. Orthomorphic surgery (reverse sagittal split osteotomy) with iliac graft CONCLUSION:

Orthomorphic surgery is a novel technique which can be used for post TMJ ankyloses deformity and simultaneous treat obstructive sleep apnoea.

577 10th World Cleft Lip, Palate & Craniofacial Congress

Evaluation Of Bone Volume In Secondary Alveolar Bone Grafting In Cleft Lip & Palate Patients

Priya.B 1st year M.D.S,

CO-AUTHOR:DEPARTMENT:Department of Craniofacial Orthodontics, Saveetha dentalArvind Sivakumarcollege and hospitals, Chennai-600077.

ABSTRACT Background:

The purpose of this studies was to evaluate the bone volume of secondaryObjective; alveolar bone grafting.

omputed tomography (CT) scans were made at 2-mm slice sections immediately before operation and at 3 months and 1 year postoperatively. The volume of the alveolar clefts and bone bridges was calculated from stored imagesSearch bymethods: using an imagescanner and a personal computer.

Ten electronic databases, grey literature, and reference list searches wereSelection conducted. criteria:

The inclusion criteria consisted of study the bone volume of secondary alveolarData collection bone grafting and analysis: using CBCT \CT of patients with CLP.

Full articles were retrieved from abstracts/titles that appeared to have met the inclusion -exclusion criteria which were subsequently reviewed using more detailed criteria for a final selection decision. The Quality Assessment of578 Diagnostic Accuracy Studies tool was used to appraise the methodological 10th World Cleft Lip, Palate & Craniofacial Congress

quality of the finally included studies. Due to the heterogeneity of the data, onlyResults: a qualitative analysis was performed.

The value at 1 year was significantly decreased compared with that at 3 monthsConclusion: postoperatively.

Adequate bone bridging was maintained for 1 year. However, it is suggested that the grafted site should be restored with a functioning tooth or a dental implant to place it under physiologic stress, because the volume of the bone bridges tends to decrease from 3 months to 1 year.

579 10th World Cleft Lip, Palate & Craniofacial Congress

A case of a rib and costochondral complex graft

Misato Katayama Introduction

A rib and costochondral complex graft is commonly used to treat micrognathia classified into Pruzansky grade . However, for a Robin sequence case due to Goldenhar syndrome, we reconstructed a temporomandibular joint inCase a 8-year-old report girl with serious airway obstruction.

The patient undertook a tracheostomy to correct her airway obstruction at 2 months of age. No further surgical treatments were performed on the micrognathia. When she was 6 years old, and during consultation with our department, a Robin sequence due to Goldenhar syndrome was confirmed. A Head and neck CT showed the following: (1) hypoplasia and deficit of the mandible (Left ramus; Type B by Purzansky crasisffication and Right ramus; site.Type ) (2) severe glossoptosis, and (3) airway constriction resulting from marked hyperplasia of the granulation tissue surrounding the tracheostomy

First, a bilateral mandibular body distraction was performed, and 15 trainingmm elongation started. was obtained after the distraction. On postoperative day 23, the patient’s cannula was switched to a speech cannula, and the first language

Secondly, for the right ramus, an 8th rib and costochondral complex graft and reconstruction of the right temporomandibular joint were performed at the age of 8 years for the next goal of extubation. For the temporomandibular joint fossa, a costochondral graft was performed and a well vascularized flap containing a periosteum was inserted into the space between the implanted fossaResults and costochondral graft.

After operation, the open mouth range had increased, and a CT scan showed580 that the airway space narrowing had also improved. 10th World Cleft Lip, Palate & Craniofacial Congress Discussion

This method of temporomandibular joint reconstruction may become a new effective treatment for cases of micrograthia with a ramus classified into Pruzansky type.

581 10th World Cleft Lip, Palate & Craniofacial Congress DEBULKED SIMPLIFIED EXPANSION & ALIGNMENT APPLIANCE FOR CLEFT LIP & PALATE PATIENTS

Sruthi S 1st year M.D.S, Department of Craniofacial Orthodontics, Saveetha dental college and hospitals, Chennai-600077. CO-AUTHOR: Arvind Sivakumar

ABSTRACT Introduction:

In cleft lip and palate patients, Maxillary deficiency occurs in sagittal, transverse and vertical planes. The maxillary deficiency is addressed in all three planes by various methods. In Transverse plane maxillary expansion is achieved with either slow or rapid palatal maxillary expansion devices. These palatal maxillary expansion appliances use heavy force or they are more bulky or can lead to ulcerations and discomfort to the patients. Also the expansion is requiredMaterials more & Methodology: in the anterior region than in the posterior region.

In order to avoid the bulky palatal expansion devices, we had decided to use a Jockey wire on the buccal side to expand the maxillary arch. The jockey wire is made of 19 Gauge (1.1 mm) Stainless Steel wire. 0.014 Niti Arch wire was segmented near the cleft region. Arch wire & Jockey wire ligatedResults: together with ligature ties. The Jockey wire goes into the Head Gear slot. lip palateAdequate patient. expansion was achieved with the jockey wire expansion. Here we are presenting a case report of the Jockey wire expansion in Bilateral cleft Conclusion:

There are various options to treat maxillary constriction like Hyrax & Quad helix. Jockey wire expansion is an economic & viable alternative for arch expansion582 in cleft patients.