Congenital esophageal stenosis due to tracheobronchial remnants: a rare but important association with esophageal atresia

Abstract

Congenital esophageal stenosis caused by tracheobronchial remnants occurred in eight children, six of whom had associated esophageal atresia and/or tracheoesophageal fistula. Symptoms usually began in early infancy but delayed diagnosis was a common feature. The mean lag period between presentation and definitive operation was 4.6 years (range, 1 month to 16 years). Errors in diagnosis were common. Six were initially diagnosed as having inflammatory strictures secondary to reflux esophagitis. Seven children were subjected to repeated esophagoscopy and bouginage of the "stricture" (mean no. = 3.4), with invariable failure to ameliorate dysphagia. Antireflux procedures were performed in three patients. In all children, symptoms were dramatically relieved following resection of the stenotic segment or esophageal replacement. Although a rare entity, congenital esophageal stenosis due to tracheobronchial remnants should be considered a possibility in patients with esophageal stricture, presumed to be inflammatory in nature, which fails to respond to standard therapy.