Congenital esophageal stenosis associated with esophageal atresia

Abstract

Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES-EA patients from Lille University Hospital, Sainte-Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non-specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1-3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1-7 dilations/patient). Esophageal perforation was encountered in three patients (18%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.

How to Care for Patients with EA-TEF: The Known and the Unknown

“…[137] In about half of cases, congenital esophageal stenosis is associated with EA. [137, 138] In these cases, diagnosis of the congenital stenosis is often delayed.…”

Estenosis esofágica congénita: diagnóstico y tratamiento. Serie de casos

“…En concordancia con otros autores, se estima que la infrecuencia de la patología no induce su pesquisa y el diagnóstico definitivo puede demorarse, incluso, hasta la segunda década de la vida. [9][10][11][12][13] L a m a y o r í a d e n u e s t r o s p a c i e n t e s s e presentaron con disfagia desde la incorporación de sólidos a la dieta. Un lactante con disfagia al iniciar la alimentación con sólidos requiere un estudio contrastado para confirmar el diagnóstico.…”

“…6,10,14 Estas medidas podrían evitar demoras diagnósticas con consecuencias como retardo del crecimiento y enfermedad respiratoria. 1,9 Se ha informado que la EEC se presenta en un 3-14% de los pacientes con atresia de esófago 6,12 y esta asociación se halló en tres de nuestros pacientes (27%). Esta relación obliga a evaluar cuidadosamente los estudios contrastados para lograr un diagnóstico precoz.…”

“…Esta relación obliga a evaluar cuidadosamente los estudios contrastados para lograr un diagnóstico precoz. 12,13 No hay un tratamiento estandarizado para esta patología y se han reportado buenos resultados con cirugía, dilataciones y resección endoscópica con láser. De acuerdo con la mayoría de los autores, probablemente, ningún tratamiento individual sea adecuado para todos los casos.…”

“…2,3,[6][7][8]10 Debido al riesgo reportado de perforación con la dilatación en los casos con restos traqueobronquiales, dos de nuestros pacientes, al principio de la serie, se sometieron a cirugía en primera instancia debido a la sospecha radiológica de este tipo de estenosis. 2,6,[10][11][12] En uno, se confirmó histológicamente y, en el segundo, se diagnosticó estenosis fibromuscular.…”