Esophageal atresia/tracheoesophageal fistula and associated congenital esophageal stenosis

Abstract

Background. The association of congenital stenosis of the distal esophagus (CES) in children with esophageal atresia/tracheoesophageal fistula complex (TEF) has been described but is thought to be rare. Most reports have been of individual or small numbers of cases. Objective. The objective of the study was to evaluate the incidence, clinical and radiographic features of CES associated with TEF, and to compare the clinical and radiographic features of CES with acquired anastomotic strictures in TEF patients. Materials and methods. A retrospective review was undertaken of the records and radiographs of 225 infants with primary 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 anastomotic strictures were identified. CES was typically a relatively long, smooth circumferential narrowing at the junction of the mid-esophagus 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 strictures; repeated esophageal dilatations were usually necessary. Esophageal 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 produces clinical symptoms. The diagnosis may be missed or delayed unless specifically evaluated surgically and radiologically. Esophageal dilatation in CES is potentially hazardous with a high risk of perforation, especially in young children.