Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia

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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. (Surgery 2001;129:29-38.)

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