The boy who lived: staged repair of congenital diaphragmatic hernia with esophageal atresia and tracheoesophageal fistula in a 32-week, 1.5 kg infant, and review of the literature.

Abstract

Congenital diaphragmatic hernia (CDH) associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) is a rare and often fatal combination with reported survival rates of 6%-26%. We aim to analyze the literature on left sided CDH with EA and TEF and report our experience, hypothesizing that delaying right chest approach for EA/TEF repair improves outcomes. We report a case of a 1.5 kg 32-week patient who survived a staged approach of initial CDH repair and abdominal control of TEF with gastrostomy to water seal and vessel loop encircling the gastroesophageal junction followed by EA/TEF repair at 18 days of life. This case report and review of the literature highlights the benefit of a staged surgical approach for left CDH, EA, and TEF; initially proceeding with CDH repair and abdominal control of the TEF first, followed by EA/TEF repair once the patient stabilizes.