Abstract
Purpose: The therapy for rare congenital esophageal stenosis (CES) varies according to its etiology. In this study, we aimed to determine preferable strategies in infants diagnosed as having CES.
Methods: We retrospectively reviewed the cases of 10 pediatric patients (mean age, 16 months; male, n = 9; female, n = 1) diagnosed as having CES between 2000 and 2015.
Results: Symptoms at onset comprised vomiting (n = 7), presence of foreign bodies (n = 1), and dysphagia (n = 2). All infants were assessed by esophagography, which revealed abrupt narrowing in nine and tapered narrowing in one. It was difficult to classify the preoperative diagnosis of CES by gastrofiberscopy and endoscopic ultrasonography, as well as intraesophageal manometry and 24-h pH monitoring. The etiologies were identified as membranous web formation (n = 1), tracheobronchial remnants (n = 4), fibromuscular thickening (n = 4), and bronchial gland dysfunction (n = 1). Nine patients were surgically treated by myotomy (n = 2) and segmental resection with end-to-end anastomosis (n = 7). Two of the three patients who did not improve after dilation underwent surgery. We added fundoplication in seven of nine patients with surgery. None of the patients had gastroesophageal reflex disease.
Conclusion: Determining the appropriate strategies is difficult for patients with CES. Balloon dilation often fails to provide symptomatic improvement except for MB. We advocate surgical management as a first-line strategy.
