Disorders in foregut diverticulization: Congenital hepaticoesophageal fistula in a baby with lryngotracheoesophageal cleft

Congenital fistulae between the biliary tract and the proximal foregut are rare. Nineteen cases have been reported involving a fistula tract between the left hepatic duct and the distal trachea or proximal bronchus. Herein the authors describe a fistula between the hepatic duct and the esophagus. A baby girl had a type III laryngotracheoesophageal cleft repaired in the neonatal period. In the course of investigating her bilious vomiting a DISIDA scan showed radionuclide excretion into the distal esophagus. A percutaneous cholangiogram through the gallbladder showed no drainage from the common bile duct into the duodenum, while the proximal left hepatic duct drained into the distal esophagus. The fistula was divided adjacent to the esophagus and the biliary system was drained through a Roux-en-Y cholecystojejunostomy. Fourteen months later she is doing well with no evidence of hepatic compromise.