Distal congenital esophageal stenosis associated with esophageal atresia

A series of six patients with congenital esophageal stenosis associated with esophageal atresia (EA) and distal tracheoesophageal fistula is presented. Three patients required only repeated dilatations, and have had good results. Two patients required limited resections of the distal esophagus, with excellent results. One patient died following a Heller myotomy. Tracheobronchial rests were present in the distal esophagus in the latter three patients. Diagnosis of congenital distal esophageal stenosis following repair of EA requires a high index of suspicion and a careful review of previous esophagrams. It is important to exclude anastomotic stricture and stenosis associated with gastroesophageal reflux. This requires barium esophagram, esophagoscopy with biopsy, and esophageal pH monitoring. Once a congenital basis for distal esophageal stenosis is suspected, management consists of dilatation by bouginage followed by balloon dilatation. Resection is reserved for persistent stenoses from tracheobronchial rests, which usually do not respond to dilatations.