Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia

Abstract

Background: Congenital esophageal stenosis (CES) has been reported to be a rare association in patients with esophageal atresia or tracheoesophageal fistula, or both (EA-TEF). This study investigates the clinical characteristics of this association with special reference to its treatment.

Methods: A retrospective review of medical and radiographic records of 81 patients who underwent primary repair of EA-TEF was performed. An association of CES was diagnosed when they showed histologic evidence or persistent radiographic images of esophageal narrowing since the neonatal period.

Results: Eleven of 81 EA-TEF patients (14%) were identified with CES. Their symptoms were difficulty in swallowing solid food, food impaction, emesis, stridor, repeated respiratory infection, and failure to thrive. Two patients had a recurrence of TEF. Three patients were diagnosed with CES in the neonatal period-2 at the time of primary repair of EA-TEF and 1 on the initial postoperative esophagram. The remaining 8 patients were diagnosed between the ages of 2 months and 3 years. Although esophageal dilatation was attempted in 9 patients, its effectiveness was temporary in all except 2 patients and esophageal laceration occurred in 4 patients. Surgical repair including myotomy and resection of the narrow segment was performed in 7 patients, in 6 of whom Nissen/Collis-Nissen fundoplication was added. A histologic examination was performed in 5 patients, revealing that their CES was due to fibro-muscular hypertrophy (2) or tracheobronchial remnants (3). The long-term outcome was satisfactory in all patients except one who showed wrap herniation.

Conclusions: The association between CES and EA-TEF is not rare. Esophageal dilatation was not universally effective and carried with it a considerable incidence of esophageal leakage. An antireflux operation concomitant with repair of CES may be useful to prevent postoperative gastroesophageal reflux in patients with a narrowing close to the esophagogastric junction.