The management of thoracobiliary fistulas in children: a systematic review.

Thoracobiliary fistula (TBF) is a rare condition, in which an atypical communication between the bronchial tree and the biliary tree is present. A comprehensive literature search was conducted on Medline, Embase and Web of Science databases for studies reporting TBF in children. Data regarding patient demographics, site of fistula presentation, preoperative diagnostic procedures needed, and treatment modalities employed were extracted for further analysis. The study pool consisted of 43 studies incorporating 48 cases of TBF. The most frequent symptom was bilioptysis (67%), followed by dyspnea (62.5%), cough (37.5%) and respiratory failure (33%). Regarding the origin of fistula, the left hepatic duct was involved in 29 cases (60.4%), the right hepatic duct in 4 cases (8.3%), and the hepatic junction in 1 case (2%). Surgical management was employed in 46 patients (95.8%). Fistulectomy was performed in 40 patients (86.9%), lung lobectomy or pneumonectomy in 6 (13%), Roux en Y hepaticojejunostomy in 3 (6.5%), and decortication or drainage in 3 cases (6.5%). Three patients died (overall mortality 6.3%), while 17 patients suffered from postoperative complications (overall morbidity 35.4%). TBF in children is a rare but morbid entity which evolves as a result of congenital malformation in the majority of cases. Preoperative imaging of the biliothoracic communication and proper surgical treatment are the components of current management.