Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases.

Purpose: The aim of this study was to review 14 patients with congenital esophageal stenosis (CES) from diagnostic and therapeutic points of view.

Methods: From 1976 to 1999, 14 patients with CES were treated at the authors' hospital. In each diagnostic examination, an esophagogram, 24-hour pH monitoring, a manometric study, and an endoscopy were performed. The medical, radiologic, and operative reports of these patients were reviewed.

Results: Esophagogram results showed an abrupt stenosis in 5 patients and a tapered stenosis in 9. After 24-hour pH monitoring and an endoscopy, we were able to rule out the possibility of gastroesophageal reflux with esophagitis in most of the patients. Of the 11 patients who initially underwent bougienage or balloon dilatation, 8 of them had recurrent stenosis. Eleven patients required surgical treatment, including the 8 who had recurrent stenosis after undergoing dilatation. Nine of the patients underwent a resection of the stenotic segment and esophago-esophagostomy. An antireflux procedure was performed on 2 patients. Another patient had an esophageal rupture, which required a jejunal interposition. A histopathologic study showed that tracheobronchial remnants were present in 6 patients, and fibromuscular thickening was found in 4 patients.

Conclusions: The efficacy of dilatation seems to be limited and may even result in severe complications such as an esophageal rupture. In this study, most patients ultimately required surgical treatment. Surgical repair for CES is the authors' preferred treatment, although initial dilatation may be effective for some patients.