Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan

Congenital bronchobiliary fistula (CBBF) is an extremely rare anomaly with myriad presentations that often include common bile duct abnormalities. Traditionally, bronchoscopy and bronchography have been used to establish the diagnosis. A full-term neonate with pneumonia and bilious secretions was diagnosed as having CBBF by means of a HIDA scan. Successful repair consisted of a right thoracotomy, high ligation of the fistula via an extrapleural approach, and cholecystography to confirm biliary drainage into the duodenum. The HIDA scan is a safe and efficient means to diagnose CBBF. When used in combination with surgical contrast studies, it provides clear delineation of all variations of this abnormality, enabling one-stage correction.