Management of congenital esophageal stenosis

Abstract

Background/purpose: The authors report the incidence, diagnosis, and treatment methods of congenital esophageal stenosis (CES) at their institution.

Methods: A retrospective analysis of 123 patients with tracheoesophageal anomalies in a pediatric hospital between 1980 and 1999 was performed. Charts were reviewed for patient demographics, presence of true CES, associated congenital anomalies, method of diagnosis, methods of treatment, and histopathology.

Results: Six of the 123 patients (4.9%) had true CES (one patient had 2 separate stenoses). Two patients had isolated CES, one patient had CES with isolated tracheoesophageal fistula (TEF), one patient had CES with isolated esophageal atresia (EA), and 2 patients had CES with EA/TEF. Diagnoses were made with an upper gastrointestinal (GI) contrast study in 5 patients, and one patient had one of 2 stenoses diagnosed by prenatal ultrasound and the other diagnosed intraoperatively. Four of the 7 stenoses were treated with surgical resection, and the remainder was treated with esophageal dilatation. Histopathology from the 4 resected stenoses showed tracheobronchial remnants in 3 specimens and submucosal thickening in 1 specimen.

Conclusions: Although isolated CES is rare (2 of 123 = 1.6%), CES associated with other tracheoesophageal anomalies has a higher incidence (4 of 123 = 3.25%). Patients with this lesion should be treated first with dilatation. If ineffective, resection is required.