Congenital Complete Arhinia: Report of a Rare Case.

Congenital absence of the nose (arhinia) is extremely rare. Arhinia leads to severe airway impairment and poor feeding in the neonate. Th e degree of nasal absence varies from case to case. Here, we present a case of congenital complete arhinia associated with other craniofacial anomalies. Arhinia leads to severe respiratory distress and feeding problems in newborn warranting emergency management, but our case diff ers as the child was adapted to mouth breathing and naso-gastric tube feeding, giving us the opportunity to delay the defi nitive management for a few weeks yet avoiding the risk of operating in a small child to create nasal airway or tracheostomy on an emergency basis.

Atresia de coanas, revisión y una mirada desde la evidencia / Choanal atresia, review and a look from the evidence

La atresia de coanas es la anomalía congénita nasal más común. Cuando es bilateral, se presenta con dificultad respiratoria desde el nacimiento. La atresia unilateral se manifiesta con insuficiencia ventilatoria y rinorrea unilateral, pudiendo pasar inadvertida. El diagnóstico se sospecha ante la ausencia de paso de aire en las fosas nasales y la imposibilidad de hacer progresar una sonda nasogástrica. Se confirma mediante examen endoscópico y tomografía computarizada. El tratamiento definitivo es quirúrgico, existiendo diferentes técnicas y vías de abordaje. Se presenta una revisión de la literatura, con especial énfasis en el tratamiento pos-quirúrgico con stents y mitomicina-C a través de una mirada desde la evidencia.

Choanal atresia is the most common congenital nasal anomaly. When bilateral, it presents with respiratory distress at birth. Unilateral atresia is manifested by respiratory failure and unilateral rhinorrhea, and may go along unnoticed. Diagnosis is suspected in the absence of airflow in the nasal cavity and for the inability to advance a nasogastric tube. Diagnosis is confirmed by endoscopic examination and computed tomography. The definitive treatment is surgical, and there are different techniques and surgical approaches. A review of the literatureis presented, with special emphasis onthepost-surgical treatmentwithstents and Mitomycin-Cview from the evidence.

Atresia de coanas, tratamiento quirúrgico endoscópico, 5 años de experiencia / Choanal atresia, endoscopic surgical repair, a 5 year experience

Atresia de coanas es la obstrucción congénita de la región posterior de las fosas nasales. Múltiples técnicas quirúrgicas han sido empleadas para su reparación. En el presente estudio se recopiló la información de 5 años de trabajo en el Hospital Universitario del Valle en la ciudad Santiago de Cali - Colombia, entre los años 2003 y 2008. Se obtuvo un total de 16 casos.La información demográfica documentada incluyó datos como edad, sexo, síntomas iniciales, método diagnóstico, lateralidad, tipo de atresia de coanas, comorbilidades, tratamiento inicial, edad de intervención quirúrgica, técnica quirúrgica usada. La técnica quirúrgica consistió en el fresado y apertura de la placa atrésica vía endoscópica transnasal utilizando taladro, previa realización de colgajos mucosos en cruz y resecando parcialmente paredes del vómer.La experiencia observada en estos 5 años de trabajo demuestra que el abordaje endoscópico transnasal presenta grandes ventajas, entre ellas la baja tasa de reestenosis.

Choanal atresia is a congenital disorder where the back of the nasal passage is blocked. Many surgical techniques have been used to repair this disorder. In this study we collected information from 5 years of work at the Hospital Universitario del Valle in the city of Santiago de Cali - Colombia, between 2003 and 2008. A total of 16 cases were obtained. The documented data included demographic information like age, sex, initial symptoms, diagnostic methods, laterality, type of atresia, comorbilities, initial treatment and surgical technique used. The surgical technique consisted in endoscopic opening of the atretic plate using drill. Mucosal flaps and resection of partial plate of vomer were made previously.The experience observed in these 5 years of work shows that the endoscopic transnasal approach has major advantages, including low restenosis rate.

Atresia coanal congênita bilateral: paciente de 35 anos bilateral / Congenital choanal atresia: 35 years old patient

Introdução: Atresia coanal congênita é a falha no desenvolvimento da comunicação entre a cavidade nasal posterior e a nasofaringe. A incidência é de 1:5.000 a 1:8.000 nascidos vivos, sendo mais comumente unilateral e no sexo feminino. Objetivo: descrever a epidemiologia, a sintomatologia e os principais acessos cirúrgicos para correção da atresia coanal, a partir do relato de um caso. Relato do Caso: Sexo feminino, 35 anos, apresentando atresia coanal bilateral óssea. O diagnóstico foi realizado por tomografia computadorizada de seios paranasais e endoscopia nasal. Foi realizada correção cirúrgica com acesso transeptal associado ao acesso transnasal via endoscópica, com confecção de retalho mucoperiosteal cobrindo a parede lateral da neocoana. Realizada aplicação tópica de mitomicina. Não foi colocado stent. Conclusão: Em nossa experiência, o acesso transnasal via endoscópica constitui via de escolha para correção cirúrgica da atresia coanal congênita. Nos casos em que houver espessamento importante da porção posterior do vômer, o acesso transeptal pode ser associado ao transnasal. Não há necessidade da colocação de stents no pós-operatório, caso seja confeccionado um flap mucoso recobrindo a área cruenta da neocoana.

Introduction: Congenital choanal atresia is an imperfection on the development of communication between the posterior nasal cavity and the nasopharynx. The incidence is 1:5,000 to 1:8,000 live births. It is usually unilateral and in females. Objective: To describe the epidemiology, the clinical symptoms and the main surgical accesses for correction of choanal atresia from the case report. Case Report: 35-year-old female patient, presenting bone bilateral choanal atresia. The diagnosis was performed through CT and nasal endoscopic. Patient was treated by transnasal endoscopic surgical technique associated with transeptal access. It was performed a mucoperiosteal flap rotation covering the sidewall of the new choana. Topic use of mytomicin was performed, but there was no use of stents. Conclusion: In our experience, the transnasal access endoscopic is the choice for surgical correction of the congenital choanal atresia. In the cases where there is important thickness of the posterior portion of the vomer, the transeptal access can be associated with the transnasal. There is no need of stents use after surgery, if a mucous flap re-covering the new choana is made.