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. Rev Bras Cir Craniomaxilofac 2010; 13(4): 225-9 225 da Silva Freitas et al. 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).