ESOPHAGEAL ATRESIA TRACHEOESOPHAGEAL FISTULA AND ASSOCIATED CONGENITAL ESOPHAGEAL STENOSIS

Background. The association of congenital stenosis of the distal esoph agus (CES)in children with esophageal atresia/tracheoesophageal fistul a complex (TEF) has been described but is thought to be rare, Most rep orts have been of individual or small numbers of cases. Objective. The objective of the study was to evaluate the incidence, clinical and ra diographic features of CES associated with TEF, and to compare the cli nical and radiographic features of CES with acquired anastomotic stric tures in TEF patients. Materials and methods. A retrospective review w as undertaken of the records and radiographs of 225 infants with prima ry TEF repair over a 26-year period. Results. A total of 18 of 225 (8 %) cases of CES associated with TEF and 43 of 225 (19 %) cases of anas tomotic strictures were identified. CES was typically a relatively lon g smooth circumferential narrowing at the junction of the mid-esophagu s and distal esophagus, with normal-caliber esophagus above and below; anastomotic strictures, in contrast, were focal. Diagnosis of CES was delayed in 10 cases and missed on one or more fluoroscopic studies in 14 children. Symptoms, including feeding and respiratory problems and foreign body impaction, were common in both CES and anastomotic stric tures; repeated esophageal dilatations were usually necessary. Esophag eal perforation complicated dilatation in 6 (33 %) young children with CES, but none of the children with anastomotic strictures (P < 0.001) . Conclusion. CES in combination with TEF is not rare and usually prod uces clinical symptoms. The diagnosis may be missed or delayed unless specifically evaluated surgically and radiologically. Esophageal dilat ation in CES is potentially hazardous with a high risk of perforation, especially in young children.