CHAPTER 49 N OTOPLASTY
Total Page:16
File Type:pdf, Size:1020Kb
Load more
Recommended publications
-
Hearing Aid Uptake in Children with Unilateral Microtia and Canal Atresia: a Comparison Between a Tertiary Center and Peripheral Centers
J Int Adv Otol 2020; 16(1): 73-6 • DOI: 10.5152/iao.2020.5509 Original Article Hearing Aid Uptake in Children with Unilateral Microtia and Canal Atresia: A Comparison between a Tertiary Center and Peripheral Centers Todd Kanzara , Alasdair Ford , Elizabeth Fleming , Su De Department of Otolaryngology, Arrowe Park Hospital, Birkenhead, United Kingdom (TK) Department of Otolaryngology, Alder Hey Children's Hospital, Liverpool, United Kingdom (AF, EF, SD) ORCID iDs of the authors: T.K. 0000-0002-8407-3818; A.F. 0000-0002-2467-3547; E.F. 0000-0002-9519-2687; S.D. 0000-0003-0442-6781. Cite this article as: Kanzara T, Ford A, Fleming E, De S. Hearing Aid Uptake in Children with Unilateral Microtia and Canal Atresia: A Comparison between a Tertiary Center and Peripheral Centers. J Int Adv Otol 2020; 16(1): 73-6. OBJECTIVES: To review the trialing and uptake of hearing aids in children with unilateral microtia or canal atresia, known collectively as congenital unilateral conductive hearing loss (CUCHL), observed in a tertiary hospital and local peripheral services. MATERIALS and METHODS: A retrospective review of medical records for patients with CUCHL was conducted using data from a shared audiol- ogy database at a tertiary children’s hospital. RESULTS: We identified 45 patients with CUCHL and excluded seven of them due to missing data. Of the 38 patients, 16 (16/38, 42%) did not have any subjective hearing complaints. Furthermore, 32% (12/38) of patients attended audiology at a tertiary centre and 83% (10/12) from this group trialled a hearing aid. In comparison, 46% (12/46) whose audiology care was delivered peripherally trialled aiding. -
Syndromic Ear Anomalies and Renal Ultrasounds
Syndromic Ear Anomalies and Renal Ultrasounds Raymond Y. Wang, MD*; Dawn L. Earl, RN, CPNP‡; Robert O. Ruder, MD§; and John M. Graham, Jr, MD, ScD‡ ABSTRACT. Objective. Although many pediatricians cific MCA syndromes that have high incidences of renal pursue renal ultrasonography when patients are noted to anomalies. These include CHARGE association, Townes- have external ear malformations, there is much confusion Brocks syndrome, branchio-oto-renal syndrome, Nager over which specific ear malformations do and do not syndrome, Miller syndrome, and diabetic embryopathy. require imaging. The objective of this study was to de- Patients with auricular anomalies should be assessed lineate characteristics of a child with external ear malfor- carefully for accompanying dysmorphic features, includ- mations that suggest a greater risk of renal anomalies. We ing facial asymmetry; colobomas of the lid, iris, and highlight several multiple congenital anomaly (MCA) retina; choanal atresia; jaw hypoplasia; branchial cysts or syndromes that should be considered in a patient who sinuses; cardiac murmurs; distal limb anomalies; and has both ear and renal anomalies. imperforate or anteriorly placed anus. If any of these Methods. Charts of patients who had ear anomalies features are present, then a renal ultrasound is useful not and were seen for clinical genetics evaluations between only in discovering renal anomalies but also in the diag- 1981 and 2000 at Cedars-Sinai Medical Center in Los nosis and management of MCA syndromes themselves. Angeles and Dartmouth-Hitchcock Medical Center in A renal ultrasound should be performed in patients with New Hampshire were reviewed retrospectively. Only pa- isolated preauricular pits, cup ears, or any other ear tients who underwent renal ultrasound were included in anomaly accompanied by 1 or more of the following: the chart review. -
Evaluation of Fetal Orbits and Ears
Evaluation of Fetal Orbits and Ears Maria A. Calvo-Garcia, MD. Associate Professor of Radiology Cincinnati Children’s Hospital Medical Center Disclosure • I have no disclosures Goals & Objectives • Review basic US anatomic views for the evaluation of the orbits and ears • Describe some of the major malformations involving the orbits and ears Background on Facial Abnormalities • Important themselves • May also indicate an underlying problem – Chromosome abnormality/ Syndromic conditions Background on Facial Abnormalities • Assessment of the face is included in all standard fetal anatomic surveys • Recheck the face if you found other anomalies • And conversely, if you see facial anomalies look for other systemic defects Background on Facial Abnormalities • Fetal chromosomal analysis is often indicated • Fetal MRI frequently requested in search for additional malformations • US / Fetal MRI, as complementary techniques: information for planning delivery / neonatal treatment • Anatomic evaluation • Malformations (orbits, ears) Orbits Axial View • Bony orbits: IOD Orbits Axial View • Bony orbits: IOD and BOD, which correlates with GA, will allow detection of hypo-/ hypertelorism Orbits Axial View • Axial – Bony orbits – Intraorbital anatomy: • Globe • Lens Orbits Axial View • Axial – Bony orbits – Intraorbital anatomy: • Globe • Lens Orbits Axial View • Hyaloid artery is seen as an echogenic line bisecting the vitreous • By the 8th month the hyaloid system involutes – If this fails: persistent hyperplastic primary vitreous Malformations of -
Otoplasty and External Ear Reconstruction
Medical Coverage Policy Effective Date ............................................. 4/15/2021 Next Review Date ....................................... 4/15/2022 Coverage Policy Number .................................. 0335 Otoplasty and External Ear Reconstruction Table of Contents Related Coverage Resources Overview .............................................................. 1 Cochlear and Auditory Brainstem Implants Coverage Policy ................................................... 1 Prosthetic Devices General Background ............................................ 2 Hearing Aids Medicare Coverage Determinations .................... 5 Scar Revision Coding/Billing Information .................................... 5 References .......................................................... 6 INSTRUCTIONS FOR USE The following Coverage Policy applies to health benefit plans administered by Cigna Companies. Certain Cigna Companies and/or lines of business only provide utilization review services to clients and do not make coverage determinations. References to standard benefit plan language and coverage determinations do not apply to those clients. Coverage Policies are intended to provide guidance in interpreting certain standard benefit plans administered by Cigna Companies. Please note, the terms of a customer’s particular benefit plan document [Group Service Agreement, Evidence of Coverage, Certificate of Coverage, Summary Plan Description (SPD) or similar plan document] may differ significantly from the standard benefit plans upon which -
Review of Microtia: a Focus on Current Surgical Approaches Nujaim H
The Egyptian Journal of Hospital Medicine (October 2017) Vol.69(1), Page 1698-1705 Review of Microtia: A Focus on Current Surgical Approaches Nujaim H. Alnujaim1, Mohammed H. Alnujaim2 1Division of Plastic and reconstructive surgery, Department of Surgery, King Saud University, Riyadh, Saudi Arabia 2College of Medicine, King Saud University, Riyadh, Saudi Arabia Corresponding author: Dr. Nujaim Hamad Alnujaim, Tel: +966506688244, Email: [email protected] ABSTRACT A wide spectrum of anomalies may involve the auditory system. As a visible structure, auricular malformations constitute a great burden. A wide set of anomalies may affect the ear including the microtia spectrum, protruding ears (bat ear), constricted ear (Lop and Cup ears), Stahl ear, and cryptotia. In plastic surgery practice protruding ears and microtia are common presentations. Microtia literally means small ears. Microtia is a spectrum of anomalies of the auricle that range from disorganized remnant of cartilage attached to soft tissue lobule to complete absence of the ear (anotia). Ear reconstructive procedures has made in impact in the lives of these patients. The early attempts to surgically restore the ear in microtia was in 1920 using a rib cartilage. Up to 49% of microtia cases are associated with other anomalies or a known syndrome. The most common syndromic associations are hemifacial microsomia, Towens Brocks syndrome, Treacher Collins, Goldenhar and Nager syndrome. Oculo-auriculo-vertebral spectrum (OAVS). Generally, the ear can be retrieved by two possible methods: Surgical reconstruction using autologous or alloplastic cartilage and the use of prosthesis which could be adhesive or implant retained. Surgical reconstruction proved to be superior to other methods due to its longevity and less complications. -
BIRTH DEFECTS COMPENDIUM Second Edition BIRTH DEFECTS COMPENDIUM Second Edition
BIRTH DEFECTS COMPENDIUM Second Edition BIRTH DEFECTS COMPENDIUM Second Edition Editor Daniel Bergsma, MD, MPH Clinical Professor of Pediatrics Tufts University, School of Medicine Boston, Massachusetts * * * M Palgrave Macmillan ©The National Foundation 1973,1979 Softcover reprint of the hardcover 1st edition 1979 978-0-333-27876-5 All rights reserved. No part of this publication may be reproduced or transmitted, in any form or by any means, without permission. First published in the U.S.A. 1973, as Birth Defects Atlas and Compendium, by The Williams and Wilkins Company. Reprinted 1973,1974. Second Edition, published by Alan R. Liss, Inc., 1979. First published in the United Kingdom 1979 by THE MACMILLAN PRESS LTD London and Basingstoke Associated companies in Delhi Dublin Hong Kong Johannesburg Lagos Melbourne New York Singapore and Tokyo ISBN 978-1-349-05133-5 ISBN 978-1-349-05131-1 (eBook) DOI 10.1007/978-1-349-05131-1 Views expressed in articles published are the authors', and are not to be attributed to The National Foundation or its editors unless expressly so stated. To enhance medical communication in the birth defects field, The National Foundation has published the Birth Defects Atlas and Compendium, Syndrome ldentification, Original Article Series and developed a series of films and related brochures. Further information can be obtained from: The National Foundation- March of Dimes 1275 Mamaroneck Avenue White Plains, New York 10605 This book is sold subject to the standard conditions of the Net Book Agreement. DEDICATED To each dear little child who is in need of special help and care: to each eager parent who is desperately, hopefully seeking help: to each professional who brings understanding, knowledge and skillful care: to each generous friend who assists The National Foundation to help. -
A Novel De Novo Mutation in MYT1, the Unique OAVS Gene Identified So
European Journal of Human Genetics (2017) 25, 1083–1086 & 2017 Macmillan Publishers Limited, part of Springer Nature. All rights reserved 1018-4813/17 www.nature.com/ejhg SHORT REPORT Anovelde novo mutation in MYT1, the unique OAVS gene identified so far Marie Berenguer1, Angele Tingaud-Sequeira1, Mileny Colovati2, Maria I Melaragno2, Silvia Bragagnolo2, Ana BA Perez2, Benoit Arveiler1,3, Didier Lacombe1,3 and Caroline Rooryck*,1,3 Oculo-auriculo-vertebral spectrum (OAVS) is a developmental disorder characterized by hemifacial microsomia associated with ear, eyes and vertebrae malformations showing highly variable expressivity. Recently, MYT1, encoding the myelin transcription factor 1, was reported as the first gene involved in OAVS, within the retinoic acid (RA) pathway. Fifty-seven OAVS patients originating from Brazil were screened for MYT1 variants. A novel de novo missense variant affecting function, c.323C4T (p. (Ser108Leu)), was identified in MYT1, in a patient presenting with a severe form of OAVS. Functional studies showed that MYT1 overexpression downregulated all RA receptors genes (RARA, RARB, RARG), involved in RA-mediated transcription, whereas no effect was observed on CYP26A1 expression, the major enzyme involved in RA degradation, Moreover, MYT1 variants impacted significantly the expression of these genes, further supporting their pathogenicity. In conclusion, a third variant affecting function in MYT1 was identified as a cause of OAVS. Furthermore, we confirmed MYT1 connection to RA signaling pathway. European Journal of Human Genetics (2017) 25, 1083–1086; doi:10.1038/ejhg.2017.101; published online 14 June 2017 INTRODUCTION ID 00095945 and 00095942, 00095943/00095955 for variants previously Oculo-auriculo-vertebral spectrum (OAVS) is a developmental dis- described2). -
EUROCAT Syndrome Guide
JRC - Central Registry european surveillance of congenital anomalies EUROCAT Syndrome Guide Definition and Coding of Syndromes Version July 2017 Revised in 2016 by Ingeborg Barisic, approved by the Coding & Classification Committee in 2017: Ester Garne, Diana Wellesley, David Tucker, Jorieke Bergman and Ingeborg Barisic Revised 2008 by Ingeborg Barisic, Helen Dolk and Ester Garne and discussed and approved by the Coding & Classification Committee 2008: Elisa Calzolari, Diana Wellesley, David Tucker, Ingeborg Barisic, Ester Garne The list of syndromes contained in the previous EUROCAT “Guide to the Coding of Eponyms and Syndromes” (Josephine Weatherall, 1979) was revised by Ingeborg Barisic, Helen Dolk, Ester Garne, Claude Stoll and Diana Wellesley at a meeting in London in November 2003. Approved by the members EUROCAT Coding & Classification Committee 2004: Ingeborg Barisic, Elisa Calzolari, Ester Garne, Annukka Ritvanen, Claude Stoll, Diana Wellesley 1 TABLE OF CONTENTS Introduction and Definitions 6 Coding Notes and Explanation of Guide 10 List of conditions to be coded in the syndrome field 13 List of conditions which should not be coded as syndromes 14 Syndromes – monogenic or unknown etiology Aarskog syndrome 18 Acrocephalopolysyndactyly (all types) 19 Alagille syndrome 20 Alport syndrome 21 Angelman syndrome 22 Aniridia-Wilms tumor syndrome, WAGR 23 Apert syndrome 24 Bardet-Biedl syndrome 25 Beckwith-Wiedemann syndrome (EMG syndrome) 26 Blepharophimosis-ptosis syndrome 28 Branchiootorenal syndrome (Melnick-Fraser syndrome) 29 CHARGE -
Congenital and Acquired Ear Deformities; Treatment Modalities
Congenital and acquired ear deformities; treatment modalities Marieke Petra van Wijk Author: M.P. van Wijk Cover: Ilse Modder, www.ilsemodder.nl Lay-out: Ilse Modder, www.ilsemodder.nl Print by: Gildeprint – Enschede, www.gildeprint.nl ISBN: 978-94-6323-565-5 © M.P. van Wijk, Utrecht, the Netherlands, 2019. All rights reserved. No part of this thesis may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording or any information storage or retrieval system, without prior permission of the author. Congenital and acquired ear deformities; treatment modalities Aangeboren en verworven oorschelpafwijkingen; behandelwijzen (met een samenvatting in het Nederlands) Proefschrift ter verkrijging van de graad van doctor aan de Universiteit Utrecht op gezag van de rector magnificus, prof.dr. H.R.B.M. Kummeling, ingevolge het besluit van het college voor promoties in het openbaar te verdedigen op dinsdag 23 april 2019 des middags te 2.30 uur door Marieke Petra van Wijk geboren op zaterdag 15 mei 1976 te Groningen promotor: Prof. dr. M. Kon copromotor: Dr. C. C. Breugem Paranimfen: Mw. Dr. E.M.L Corten Mw. Dr. A.L van Rijssen Leescommissie: Prof. Dr. J.J.M. van Delden Prof. Dr. R.L.A.W Bleys Prof. Dr. C.M.A.M. van der Horst Prof. Dr. R.J. Stokroos Prof. Dr. E.E.S. Nieuwenhuis TABLE OF CONTENTS Chapter 1. 11 Introduction and aim of the thesis Chapter 2. 29 Non-surgical correction of congenital deformities of the auricle: a systematic review of the literature. van Wijk MP, Breugem CC, Kon M. -
Case Report Plastic and Aesthetic Research
Case Report Plastic and Aesthetic Research Craniofacial abnormalities in goldenhar syndrome: a case report with review of the literature Ramesh Kumaresan1, Balamanikanda Srinivasan1, Mohan Narayanan2, Navaneetha Cugati3, Priyadarshini Karthikeyan4 1Departments of Oral and Maxillofacial Surgery , Faculty of Dentistry, AIMST University, 08100 Bedong, Kedah Darul Aman, Malaysia. 2Department of Oral Medicine and Radiology, Vinayaka Mission’s Shankaracharyar Dental College, Salem 636308, Tamil Nadu, India. 3Department of Pedodontic and Preventive Dentistry, Faculty of Dentistry, AIMST University, 08100 Bedong, Kedah Darul Aman, Malaysia. 4Department of Oral Medicine and Radiology, Shree Balaji Dental College, Chennai 600100, Tamil Nadu, India. Address for correspondence: Dr. Ramesh Kumaresan, Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, AIMST University, 08100 Bedong, Kedah Darul Aman, Malaysia. E-mail: [email protected] ABSTRACT Goldenhar syndrome (oculo-auriculo-vertebral spectrum) is a rare congenital anomaly of unclear etiology and characterized by craniofacial anomalies such as hemifacial microsomia, auricular, ocular and vertebral anomalies. In many cases, this syndrome goes unnoticed due to a lack of knowledge about its features and because of its associated wide range of overlapping anomalies. Herewith, we present a case of Goldenhar syndrome in a 21-year-old male, who presented all the classical signs of this rare condition. This article also summarizes the characteristic features of patients with Goldenhar syndrome. Key words: Congenital abnormalities, eye abnormalities, Goldenhar syndrome, oculo-auriculo-vertebral spectrum INTRODUCTION CASE REPORT Goldenhar syndrome is a rare developmental anomaly The patient is a 21-year-old male who reported to involving structures derived from first and second the Department of Oral Medicine and Radiology, with branchial arches of the first pharyngeal pouch, the first complaints of an unaesthetic facial and dental appearance. -
Goldenhar Syndrome
orphananesthesia Anaesthesia recommendations for patients suffering from Goldenhar syndrome Disease name: Goldenhar syndrome ICD 10: Q87.0 Synonyms: Oculo-Auriculo-Vertebral (OAV) syndrome / sequence, Facio-Auriculo-Vertebral syndrome, Goldenhar-Gorlin syndrome In 1952, Maurice Goldenhar published a case collection of congenital mandibulo-facial malformations with or without epibulbar dermoids, auricular appendages and auricular fistulas. With the attempt to systematically classify these malformations, he described for the first time what later became known as the Goldenhar syndrome. Goldenhar syndrome is a variant of the Oculo-Auriculo-Vertebral spectrum. It consists of hemifacial microsomia (HFM), epibulbar dermoids and vertebral anomalies. Major manifestations of HFM are orbital distortion, mandibular hypoplasia, ear anomalies, nerve involvement and soft tissue deficiency (OMENS classification). In addition, patients with Goldenhar syndrome can present with heart, kidney and lung malformations as well as limb deformities. Depending on the organs affected and the severity of the malformations the phenotype is highly variable (Table). The exact cause of Goldenhar syndrome is unknown but considered to be multifactorial, i.e. a combination of gene interactions and environmental factors that causes a maldevelopment of the first and second branchial arches during the first trimester of pregnancy. Males are affected more often than females (3:2). About 10-30% of patients have bilateral, usually asymmetric facial microsomia. There is no agreement -
UK Care Standards for the Management of Patients with Microtia and Atresia
UK Care Standards for the Management of Patients with Microtia and Atresia BAA – British Academy of Audiology ( baaudiology .org ) BAAP – British Association of Audiovestibular Physicians ( baap.org.uk) BAPA – British Association of Paediatricians in Audiology(bapa.uk.com) BAPRAS - British Association of Plastic, Reconstructive and Aesthetic Surgeons ( bapras.org.uk) Changing Faces ( changingfaces.org.uk) ENT UK- Ear, Nose and Throat- United Kingdom ( entuk.org) Microtia UK ( microtiauk.org) Microtia Mingle ( microtiamingle.co.uk ) NDCS – National Deaf Children’s Society ( ndcs.org.uk) PPN UK – Paediatric Psychology Network UK (www.ppnuk.org) 1 Contents Page Section 1 Executive summary 4 Key points 5 Section 2 – Introduction and background 7 2.1 definitions 8 2.2 history 8 Section 3 - Impact upon patients and families 11 3.1 evidence base for impact of microtia and atresia on day to day hearing 11 3.2 evidence base for psychological impact of microtia and atresia 14 Section 4 – Care pathway 17 4.1 care pathway flow chart 18 Section 5 - Assessment 20 5.1 the multidisciplinary team 20 5.2 initial assessment 21 5.3 follow up after initial assessment 23 Section 6 – Considering ear reconstruction 25 6.1 decision making process 25 6.2 reconstruction options 26 6.3 perioperative care 29 6.4 training in ear reconstruction 31 Section 7 – Intervention for hearing loss 33 7.1 audiology based management and intervention options 34 7.2 hearing devices 37 Section 8 – service models and care structures 43 8.1 current UK service model 43 8.2 recommended