Management of congenital esophageal stenosis.

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.