Thoracoscopic repair of esophageal atresia with distal tracheoesophageal fistula and a proximal type-H tracheoesophageal fistula

The purpose of this study was to report a combined approach using thoracoscopy and flexible bronchoscopy to identify and repair a rare tracheoesophageal fistula (TEF) anatomic variant. This variant had both a distal tracheoesophageal fistula and a rare proximal fistula in an H-type configuration located high in the thoracic inlet. A 2045 g, ex-35 week female underwent thoracoscopy for what was preoperatively believed to be Gross type C anatomy. After ligation of the distal fistula, ventilation remained challenging and intraoperative flexible bronchoscopy revealed a proximal fistula. The proximal fistula was in a H-type configuration high in the thoracic inlet. Both fistulae, as well as the esophageal atresia, were repaired thoracoscopically. A postoperative contrast study at 10 days and 3 months revealed an intact esophageal repair without a leak or stricture. A postoperative rigid bronchoscopy at 10 days showed no airway abnormalities. A narrated video of the operation is available under the supplemental data of this publication. The thoracoscopic repair of this rare tracheoesophageal fistulae variant with H-type anatomy between the proximal pouch and trachea located high in the thoracic inlet is feasible in low birth weight infants. Intraoperative flexible bronchoscopy is a valuable tool for the identification of a second fistula.