A 25 weeks gestational age, 755 g neonate with esophageal atresia and tracheoesophageal fistula presents with ileal perforation and esophageal pouch perforation

Abstract

Esophageal atresia and tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly with an incidence of 1:2500–5000 live births. In premature and low birth weight infants, EA/TEF is associated with significant mortality. Premature infants are additionally at risk for the development of multiple complications related to prematurity. The standard surgical approach to EA/TEF is fistula ligation followed by primary anastomosis of the esophagus. The optimal timing for repair of EA/TEF in extremely low birth weight (ELBW) infants is unknown, but some surgeons advocate for a staged approach. We present the case of a 25 weeks gestational age, ELBW (755 g) infant with EA/TEF whose course was complicated by iatrogenic esophageal perforation in addition to an isolated ileal perforation. We describe the successful management of this infant with primary repair of both the ileal perforation as well as the esophageal atresia. In addition, a recurrent fistula was managed successfully via an endoscopic approach.