Congenital Esophageal Stenosis: To Dilate or To Resect

Background/Purpose: Congenital esophageal stenosis (CES) is a rare condition in children. The definitive preoperative diagnosis often is difficult to make, and a standard therapeutic protocol remains controversial. This study was carried out to determine the proper management strategy in children suffering from CES. Materials and Methods: The medical records of 17 patients with CES treated during a period of 10 years (from 1995 to 2005) were retrospectively reviewed. Each patient was evaluated as regard to the clinical presentation, pathology, management, and outcome. Results: The ages of patients at time of diagnosis ranged from 3 months to 9 years. The sites of stenosis were located more frequently at the lower third of the esophagus (n=10) than the upper third (n=3) and middle third (n=4). The diagnosis was made by esophagogram, esophagoscopy and was confirmed by histopathologic examination. Fifteen patients were diagnosed primarily, while 2 patients were diagnosed after unsuccessful surgical treatment for an initial misdiagnosed achalasia of the cardia. Six patients had confirmed tracheobronchial remnants (TBR), five had fibromuscular stenosis (FMS), and 2 had membranous web stenosis (MS). The histopathogy was unknown in 4 patients due to inadequate biopsies taken during esophagoscopy. All patients were treated initially by repeated esophageal dilatations (2-8 times) over two to thirty month period. The dilatation alone was successful in 11 patients, but was complicated by esophageal perforation in one case. Six patients required surgery; five of them were treated by resection and anastomosis, and one required esophageal replacement. The pathology of this later group was TBR in 5 patients and FMS in one. Conclusion: 1. this study emphasizes the diagnostic difficulties in some children with CES; 2. Esophageal dilatation may be tried initially 3. Resection should be reserved for cases not responding to repeated dilatation particularly those with proven TBR.