Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia

Background. Congenital esophageal stenosis (CES) has been reported to be a rare association in patients with esophageal atresia or fistula, or both (E A-TEF). This study investigates the clinical characteristics of this associ ation with special reference to its treatment. Methods. A retrospective review of medical and radiographic records of 81 p atients who underwent primary repair of EA-TEF was performed. An associatio n of CES was diagnosed when they showed histologic evidence radiographic im ages 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, adn failure to thrive. Two patien ts had a recurrence of TEF. Three patients were diagnosed with CES in the n eonatal period-2 at the time of primary repair of EA-TEF and 1 on the initi al postoperative esophagram. The remaining 8 patients were diagnosed betwee n the ages of 2 months and 3 years. Although esophageal dilatation was atte mpted in 9 patients, its effectiveness was temporary in all except 2 patien ts and esophageal laceration occurred in 4 patients. Surgical repair includ ing 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 t o fibro-muscular hypertrophy (2) or tracheobronchial remnants (3). The long -term outcome was satisfactorily 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 a considerable i ncidence of esophageal leakage. An antireflux operation concommitant with r epair of CES may be useful to prevent postoperative gastroesophageal reflux in patients with a narrowing close to the esophagogastric junction .