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

Abstract

Aim The aim of this work was to study the incidence, management of congenital esophageal stenosis (CES) associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF), and its impact on esophageal stricture (ES) after primary repair.

Patients and methods From January 2006 to December 2014, 41 sequential cases of EA with distal TEF were retrospectively studied. Cases with significant ES after primary repair were included in the study. Associated CES was diagnosed in the neonatal period by taking histopathologic samples from the tips of the esophageal pouches, failure to pass a size-6 nasogastric tube distally during primary repair, and by initial esophagogram.

Results Significant ES developed in 19 patients (46.3%); of them, six had CES (32%). Three patients had tracheobronchial remnants at the anastomotic site. Two of them had refractory strictures requiring resections and one had mainly major esophageal dysmotility. Patients 4 and 5 had CES distal to the anastomotic site on initial esophagogram. Patient 4 responded well to dilatations, whereas the other one had refractory stricture. Patient 6 had distal CES due to fibromuscular stenosis diagnosed by failure to pass a size-6 nasogastric tube distally. The patient responded well to dilatation, myectomy, and Thal’s fundoplication.

Conclusion One-third of the patients with significant stricture had CES; half of them were refractory to dilatation. Failure to have histology specimens and a high index of suspicion will make the incidence of this association a rarity. Diagnosis and management of CES with EA/TEF in the neonatal period is possible. Esophageal dilatation is the initial management for all cases with a low threshold for gastric fundoplication and gastrostomy. Resection is reserved for refractory stenosis.

Keywords: congenital esophageal stenosis, esophageal atresia, esophageal stricture, tracheoesophageal fistula