Series of Facial Cleft Number 2-12: Strategies for Treatment Séries De Fissuras Faciais Número 2-12: Estratégias Para Tratamento

Facial Cleft #2-12

ARTIGO ORIGINAL

Series of facial cleft number 2-12: strategies for treatment Séries de fissuras faciais número 2-12: estratégias para tratamento

Renato da Silva Freitas1, Nivaldo Alonso2, Luciano Busato3, Alessandra dos Santos Bitencourt4, Alessandro Zorzi4, Brunno Gomes Rocha4, Larissa Lopes Rocha4, Thais de Freitas Azzolini5

RESUMO abstract

Introdução: A fissura facial número 2 é uma malformação Introduction: The facial cleft number 2 is an extremely rare extremamente rara, de etiologia desconhecida. O acometimento malformation of unknown etiology. The soft tissue defect begins de tecidos moles começa com uma fissura comum de lábio supe- with a common cleft of the upper lip and an involvement of the rior e de terço medial de borda alar. A cartilagem alar pode ser middle third of the alar rim. The nasal alar cartilage may be hypo- hipoplásica e deslocada superiormente na sua porção média. Sua plasic and drown upward in the midportion. Its northbound is the correspondente craniana é a fissura 12. Objetivo: O objetivo cranial cleft number 12. Objective: The aim was to review the deste estudo foi revisar os resultados funcionais e estéticos de functional outcome and aesthetic results of the different techniques diferentes técnicas aplicadas a cada caso. Método: O presente applied for each case. Methods: The present study is based on estudo foi baseado na observação de 10 pacientes com fissura observations of 10 patients with Tessier number 2 cleft seen at número 2-12 de Tessier, atendidos no CAIF- Centro de Atendi- Assistance Center for Cleft Lip and Palate during the period from mento Integral ao Fissurado Lábio Palatal, durante o período 1996 to 2008. We reviewed the clinical databases and radiologic de 1996 e 2008. Foram também revisados os dados clínicos e studies. Results: The treatment was begun in the first year of life radiológicos de cada caso. Resultados: O tratamento iniciou- in majority of our patients. Seven of them were submitted to lip se no primeiro ano de vida da maioria de nossos pacientes. Sete repair using Millard technique (4 cases) and simple lip adhesion (3 deles foram submetidos a reconstrução labial, sendo quatro pela cases), and three patients did not have any surgery over the upper técnica de Millard, e três pacientes à adesão labial. Três pacientes lip. Nasal cartilage reposition was performed in three cases, repair não apresentavam acometimento labial. O reposicionamento da of the alar coloboma using local flaps in one case, two half-nose cartilagem alar foi realizado em três casos, a correção do colobo- reconstructions using a forehead flap, and two local V-Y flaps. The ma alar usando retalhos locais em um, reconstrução de ausência eyebrow was corrected repositioning the hair when it was disloca- de heminariz com retalho frontal em dois, e uso de retalho em V-Y ted or using a micrograft associated to tattooing in cases without em 2 pacientes. A sobrancelha foi corrigida pelo reposicionamento hairs. Conclusion: The treatment of the cleft number 2 demands dos cabelos, quando estes estavam deslocados, ou utilizando o a planned, staged, sequential approach to produce the ideal end- microimplante de cabelo associado à tatuagem, em casos sem result. Skin, soft tissue, and bone must be addressed to achieve an sobrancelha remanescente. Conclusão: O tratamento da fissura ideal result with optimize outcome. Lip, nose, medial canthus and 2-12 necessita de abordagem planejada, estagiada e sequencial, cranial involvement were treated in the same stage, and we have para produzir um resultado final adequado. Pele, tecidos moles e utilized local flaps in all possible situation. Hypertelorism correction osso devem ser abordados para conseguir um bom seguimento. O should be delayed to eight years of age. acometimento do lábio, nariz, canto palpebral medial e craniano foram tratados no mesmo ato operatório, e utilizamos retalhos locais sempre que possível. Pacientes com hiperteleorbitismo deveriam ser tratados após oito anos de idade.

Descritores: Fissura facial. Fissura labial. Malformação Key words: Facial cleft. Cleft lip. Facial malformation. facial. Fissura Nasal. Cleft nose.

1. Associate Professor, Section of Plastic and Reconstructive Surgery, Federal Correspondence: Renato da Silva Freitas University of Paraná; and Plastic Surgeon of the Assistance Center for Cleft Rua General Carneiro, 181, 9º andar – Curitiba, PR, Brazil – Zip Code: Lip and Palate (CAIF). 8000-000 2. Chief of the Craniofacial Surgery Unit, Section of Plastic Surgery, Sao E-mail: [email protected] Paulo University. 3. Plastic Surgeon of the CAIF. 4. Medical Student, School of Medicine, Federal University of Paraná. 5. Medical Student, School of Medicine, Faculdade Evangélica do Paraná.

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INTRODUction Five of these patients were male and five were female. The average patient age at time of surgery was 7,9 years. The clinical The Tessier classification system is one of the most useful findings associated with lip malformation were: alveolar cleft, in facial cleft because it makes the correlation between clinical cleft palate, nose and eyelid involvement, lacrimal obstruction, appearance and surgical anatomy on craniofacial clefts. Also, it dislocation of medial canthus, eyebrow involvement including integrates topographic clinical observations with the underlying cleft and dislocation, hypertelorism and encephalocele. The skeletal disturbance. The facial cleft number 2 as described by surgical procedures and tomographic studies were evaluated. Tessier is an extremely rare malformation of unknown etiology We have discussed our protocol of the treatment. with distinct soft tissue and bone characteristics1. At the facial cleft number 2, the soft tissue defect begins with a common cleft of the upper lip and an involvement of RESULTS the middle third of the alar rim. The nasal alar cartilage may be hypoplasic and drown upward in the mid portion. The dome is At Assistance Center for Cleft Lip and Palate (CAIF), intact. The lateral aspect of the nose is flattened and the dorsum 7900 patients with facial malformation have been treated in is broad. A shallow soft tissue groove extends superiorly to the 18 years of activity. Around 300 patients have had rare facial asymmetrically widened nasal root2,3. clefts, the majority of them with craniofacial microsomia. From The skeletal defect begins on the maxilla. The cleft crosses this group, 10 patients with a number 2 cleft were reviewed. the alveolus in the region of the lateral incisor, and extends into Four patients were evaluated in the first year of life, with an the pyriform aperture. A hard and soft palate cleft may occur. The average age of presentation of three years. Five were female nasal septum is intact but deviated to the opposite side. A notch and five male. One patient had amniotic bands in limbs with is frequently present near the junction of the nasal bone and the amputations of some fingers. frontal process of the maxilla, which is widened. Related to cleft Related to cleft lip, three patients were found to have bila- number 12, the northbound of facial cleft number 2, above this teral cleft lip and four patients unilateral cleft lip (Table 1). nasomaxillary notching, the ethmoid sinus is undeveloped and Three patients did not have any involvement of the upper lip. there is not pneumatization of the frontal sinus of this side. The All patients with normal upper lip did not have alveolar clefts lacrimal system, palpebral fissures and eyebrow may be intact1,4. either. The alveolar bridge was compromised in six patients, Few articles have been published about the cleft number with varying degree. One patient had a cleft lip without an 25-12. These articles have often described the challenge of the alveolar cleft. Four patients had cleft palate. treatment. We conducted a retrospective analysis of our series Two patients presented normal shape and position of the consisting of 10 cases of Tessier number 2 cleft. The objective nose. However, eight patients had some nasal malformation, was to review the functional outcome and aesthetic results of including three cases with half-nose, two cases with alar base the different techniques applied for each case. upward deviated, two cases with a “regular” unilateral cleft nose, and two alar coloboma. In the orbitopalpebral area, four cases of lacrimal duct METHODS obstruction were identified (Table 2). Two patients had medial eyelid coloboma and the medial canthus was dislocated Ten patients with Tessier number 2 cleft were admitted to downward in four cases. Seven cases of cleft number 12 were Assistance Center for Cleft Lip and Palate (CAIF) since 1996. observed. Encephalocele was present in three patients of these The age at treatment ranged from newborn to 30 years-old. patients. The eyebrow was clefted in four cases and superiorly

Table 1 - Tessier number 2 cleft. Clinical findings. Gender Age Lip Alveolar Nose Palate Associated Others upperward + amniotic bands 1 female 2 BCL clefted clefted 12 + encephalocele cleft in limbs R upperward + 12 + xeroderma pig- 2 male 0 lUCL clefted clefted no cleft mentosum 3 male 18 BCL clefted normal clefted no no 4 female 13 BCL normal normal normal microtia no 5 male 0 rUCL clefted UCN normal 12 + encephalocele no 6 male 0 normal normal halfnose normal 12 no halfnose + 7 female 3 normal normal normal no no proboscis 8 female 13 normal normal halfnose normal 12 no 9 male 30 rUCL clefted UCN normal 12 no 10 female 0 rUCL clefted UCN clefted 12 + encephalocele no 7,9

Rev Bras Cir Craniomaxilofac 2010; 13(4): 225-9 226 Facial Cleft #2-12 dislocated in two cases. Six cases of hypertelorism, one case of nose. All the cleft palate was closed using a von Langenback iris coloboma and one of keratitis were observed. technique and intravelar veloplasty. Only two patients were The treatment was begun in the first year in majority of necessary to perform the reposition of medial canthus using a our patients (Table 3). Seven of our patients were submitted flap from the upper eyelid with medial pedicle. The eyebrow to lip repair, using Millard technique (four cases) (Figure 1), was corrected repositioning the hair when it was dislocated or lip adhesion (three cases), and three patients did not have or using hair micrografts and tattoing in cases with absence any surgery over the upper lip. Nasal cartilage reposition was of eyebrow (Figure 2). Four cranioplasties were necessary, performed in three cases, repair of the alar coloboma using three using bone grafting and one using acrylic to reshape the local flaps in one case, two half-nose reconstructions using a forehead. Only one case of hypertelorism was submitted to forehead flap, and two local V-Y flaps. Two patients had normal orbital advancement with Tessier technique.

Table 2 - Tessier number 2 cleft. Orbitopalpebral findings.

Lacrimal Eyelid Medial Canthus Eyebrow Hypertelorism Eye Globus

1 normal normal normal normal yes normal

2 normal normal normal R clefted no iris coloboma

3 normal normal normal normal yes normal

4 normal normal normal normal no normal

5 normal normal dislocated clefted yes normal

6 obstruction normal normal dislocated no normal

7 obstruction clefted normal normal no normal

8 obstruction clefted dislocated dislocated yes keratitis

9 normal normal dislocated clefted yes normal

10 obstruction normal dislocated clefted yes normal

Table 3 - Surgical procedures. # Lip Repair Nose Palate Orbitopalpebral Eyebrow Others Surgeries 1 5 adhesion V-Y flap von Langhenbeck no no hypertelorism cranioplasty with 2 4 Millard local flap von Langhenbeck no no acrylic local flap with cranioplasty with 3 3 Millard skoog no no hairs bone graft 4 1 PR* PR* PR* no no maxilla osteotomy 5 1 Lip review no no no no no von micrograft and cranioplasty with 6 5 Millard McComb no Langhenbeck tattoo bone graft half-nose 7 2 no no no no no reconstruction half-nose medial cartilage graft to 8 3 Lip review no no reconstruction canthopexy nose 9 0 no no no no no no von medial cranioplasty with 10 4 Millard McComb micrograft Langhenbeck canthopexy bone graft * PR: Previous repair

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DISCUSSION Figure 1 - 30 years-old man, with right cleft lip, associated to hypertelorism, absence of mid-eyebrow and frontal bone (cleft #12). A: Pre-operative photograph The treatment of these rare facial clefts is usually not (frontal view); B and C: Tomographic view; D: Post- directed at a single-stage surgical correction, but demands a operative of lip repair (Millard technique) and frontal bone planned, staged, sequential approach to produce the ideal end- grafting. E: Follow-up of 8 years. result in the end of facial growth. Therefore, our cases were submitted to three surgeries in average. Reconstruction in these cases must consider the restoration of all elements – skin, soft tissue and skeleton, in a complex interrelated fashion at an age-appropriate time to optimize outcome. The result, both functional and aesthetic, is however prin- cipally determined by the quantity, quality and redraping of the soft tissues. Reconstruction with soft tissues comparable in color and consistency, and free from distorting traditional A B C forces, idealizes restoration of facial features and permits tension-free sitting of vital soft tissue landmarks. The tissue expansion is a useful adjunct to provide a greater amount of better vascularized local tissue for reconstruction of the soft tissue, by cheek rotation and advancement flaps. We did not have any case that was necessary to indicate tissue expander. In general, cleft number 2 presenting only alar coloboma can be corrected by full-thickness rotation flaps taken from the lateral aspect of the nose. Satisfactory rim contour is gene- rally obtained, especially in minor alar clefts, no associated to hypertelorism or encephalocele. However, sometimes the asymmetrical nostrils may require secondary correction. More severe clefts extending along the whole length of the nose can D E be corrected by rotation of longer nasal flaps alone or combined with forehead flaps. Cases associated with orbital hypertelorism, the nasal correction may be carried out simultaneously with orbital mobilization. One of our cases was submitted to advancement of two V-Y flaps to create the nostrils. Specifically to this patient, she had hypertelorism and forehead encepha- Figure 2 - Patient with 5 months-old, with right cleft lip and palate, absence of mid-eyebrow. A: Pre-operative photograph; B: Tomo- locele, which was partially corrected at eight years of age. In graphic view, demonstrating alveolar cleft and frontal defect. C: the minor case of alar coloboma, a subcutaneous pedicled V-Y Final result after lip repair (Millard), Palatoplasty (veloplasty), frontal flaps from nasojugal area achieved great contour of the nostril bone grafting, eyebrow reconstruction with micrograft and tattoing. and good quality of the scar. Other 3 patients had nasal distortion similar to unilateral cleft nose, and they were submitted to primary rhinoplasty using McComb (2 cases) and Skoog (1 case) techniques. Cases of half-nose from out unit were already published and discussed13. In CAIF we had 3 cases of halfnose, one of them refused the treatment. Few papers were published describing the malformation1-4 and others few demonstrating the treatment5-12. Ozek published different surgical approaches to be utilized9. Facial bipartition may be indicated to treat orbital hypertelorism, as suggested by van der Meulen14. When the nasolacrimal duct was involved, A B treatment includes total excision, since repeated infection tends to develop in the distorted ductal system. In patients with alar coloboma, the authors corrected by full-thickness rotation flaps taken from the lateral aspect of the nose and the complete cleft lip can be repaired using technique to repair regular cleft lip, as Millard. In our series, we have used the same approach for cleft lip. Absence of eyebrow was present is some patients, and distortion of its position in others. Some of patients were managed using hair micrografts and tattooing15, others only micrografts, and a third group rotation of residual hair to C eyebrow line. All of these techniques demonstrated to be good options to treat this abnormality. Dystopia of medial canthus

Rev Bras Cir Craniomaxilofac 2010; 13(4): 225-9 228 Facial Cleft #2-12 was identified in four cases, and indicated the transposition REFERENCES of a flap from upper eyelid with medial pedicle associated to medial canthopexy. 1. Tessier P. Anatomical classification facial, cranio-facial, and latero-facial Osteotomy and bone grafting of the craniofacial skeleton clefts. J Maxillofac Surg. 1976;4(2):69-92. with stable internal fixation can be performed with an accep- 2. Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial clefts: order out of chaos (Tessier classification). Clin Plast Surg. 1976;3(4):529-72. tably predictable result, even in clefts. The timing of inter- 3. Thorne CH. Craniofacial clefts. Clin Plast Surg. 1993;20(4):803-14. vention and pattern of osteotomy is individualized for each 4. Alonso N, da Silva Freitas R. Craniofacial clefts and other related de- of the rare clefts deformities, but involves orbital osteotomy formities. In: Guyuron B, Ericksson E, Persing JA, eds. Plastic surgery: early before 5 years, with delay of midfacial and mandibular indications and practice. vol. I. Philadelphia:Saunders Elsevier;2009. osteotomy until the completion of facial growth16. One of our p.455-71. cases was submitted to osteotomy of maxilla and advancement 5. Ortiz-Monasterio F, Fuente del Campo A, Dimopulos A. Nasal clefts. Ann Plast Surg. 1987;18(5):377-97. of 5mm, after orthodontia. 6. van der Meulen JC, Vaandrager JM. Facial clefts. World J Surg. Surgical results are not as good in asymmetrical clefts such 1989;13(4):373-83. as 2-12, associated with orbital hypertelorism. The alterations of 7. Tiwari P, Bhatnagar SK, Kalra GS. Tessier number 2 cleft, a variation. Case the orbital and nasal skeleton, as well as the soft-tissue altera- report. J Craniomaxilofac Surg. 1991;19(8):346-7. tions on the cleft side, represent a hard problem to reconstruct. 8. Shewmake KB, Kawamoto HK Jr. Congenital clefts of the nose: principles In terms of symmetry, the center of the face is extremely critical of surgical management. Cleft Palate Craniofac J. 1992;29(6):531-9. and, unfortunately, it can rarely be achieved. With improved 9. Ozek C, Gundogan H, Bilkay U, Cankayali R, Guner U, Gurler T, et al. Rare craniofacial anomaly: Tessier No. 2 cleft. J Craniofac Surg. 2001;12(4):355- techniques it is now possible to obtain excellent results in many 61. nasal clefts, but challenging problems are still present. 10. Sigler MO, Stein J, Zuker R. A rare craniofacial cleft: numbers 7, 2, and 3 clefts accompanied by a single median lip pit. Cleft Palate Craniofac J. 2004;41(3):327-31. CONCLUSION 11. Losee JE, Kirschner RE, Whitaker LA, Bartlett SP. Congenital nasal anoma- lies: a classification scheme. Plast Reconstr Surg. 2004;113(2):676-89. 12. Ortiz-Monasterio F, Taylor JA. Major craniofacial clefts: case series and The treatment of the cleft number 2 demands a planned, treatment philosophy. Plast Reconstr Surg. 2008;122(2):534-43. staged, sequential approach to produce the ideal end-result in 13. da Silva Freitas R, Alonso N, de Freitas Azzolini T, Busato L, Dall’Oglio the end of facial growth. In general, our patients were submitted Tolazzi AR, Azor de Oliveira E Cruz G, et al. The surgical repair of half- to three surgical procedures. Skin, soft tissue, and bone must nose. J Plast Reconstr Aesthet Surg. 2010;63(1):15-21. be addressed to achieve an ideal result with optimize outcome. 14. van der Meulen JC. Medial faciotomy. Br J Plast Surg. 1979;32(4):339-42. Therefore, color and consistency of soft tissue are important 15. da Silva Freitas R, Bertolotte W, Shin J, Busato L, Alonso N, Grande CV, et al. Combination micrografting and tattooing in the reconstruction of during the decision about the surgical options. Lip, nose, medial eyebrows of patients with craniofacial clefts. Ophthal Plast Reconstr Surg. canthus and cranial involvement were treated in the same stage, 2008;24(4):340-2. and we have utilized local flaps in all possible situation. Hyper- 16. Tessier P, Rougier J, Hervouet F, Woilllez M, Lekieffre M, Demore P. Plastic telorism correction should be delayed to eight years of age. surgery of the orbits and eyelids. Chicago:Masson Publishing USA;1981.

Paper performed at Assistance Center for Cleft Lip and Palate (CAIF), Curitiba, PR, Brazil; Section of Plastic Surgery, Federal University of Paraná, Curitiba, PR, Brazil; Craniomaxillofacial Surgery Unit, Section of Plastic Surgery, Sao Paulo University, São Paulo, SP, Brazil. Article received: 12/6/2010 Article approved: 8/10/2010

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