Characteristics and management of congenital esophageal stenosis: findings from a multicenter study

Abstract

BackgroundCongenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.MethodsMedical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.ResultsOver 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27).ConclusionsCS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

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%).…”

“…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.…”

“…10,13 Los tres niños con EEC del tercio superior tuvieron aspecto radiológico y endoscópico de membranas esofágicas y, coincidentemente, fueron el grupo etario más alto. Esto sugiere que este tipo de estenosis sería mejor tolerada haciendo que el diagnóstico se retrasara.…”

Management of congenital esophageal stenosis associated with esophageal atresia and its impact on postoperative esophageal stricture

“…According to four observational studies, the overall incidence of CES among patients with EA and/or tracheoesophageal fistula (TEF) was 9.6% [4]. In a study on 61 patients with CES, 29 had associated EA [5]. CES can affect the anastomotic site [3,6] or distal to it [2,7,8].…”