Abstract
BACKGROUND: Bronchobiliary fistula (BBF) is a rare but fatal disease. Due to its rarity, only a limited number of cases have been reported, leading to a lack of consensus on appropriate treatment strategies. METHODS: We conducted a retrospective analysis of clinical data of 17 patients diagnosed with BBF between January 1, 2012, and May 30, 2025, focusing on the the presenting symptoms, diagnostic approaches, treatment modalities, and outcomes. RESULTS: All 17 patients had cough and pathognomonic biliptysis. Sputum analysis confirmed bile components in all samples from 3 patients, and fiber bronchoscopy revealed yellow-green bilious sputum in 6 patients. Computed tomography (CT) or magnetic resonance imaging (MRI) demonstrated communication between the bile duct and the bronchial tree in 9 patients. Cholangiography showed contrast medium passing through a fistulous tract into the bronchi in 10 patients. Surgical intervention was performed in 6 patients, 3 of whom achieved long-term survival (> 24 months). Minimally invasive interventions provided effective symptomatic palliation in 10 of 11 patients. Among these 10 patients, 3 achieved long-term survival (> 24 months), with an additional 3 remaining alive and under ongoing follow-up (though not yet reaching the 24-month threshold). Of the 8 fatalities, 2 were directly attributed to uncontrolled BBF and its complications (sepsis or hepatic failure). The remaining deaths resulted from progressive malignancy (n = 3), postoperative complications (pneumothorax/respiratory failure, n = 1; hemorrhagic shock/disseminated intravascular coagulation, n = 1), and post-transplant septic shock (n = 1). CONCLUSIONS: BBF is associated with poor prognosis. Minimally invasive therapies offer effective palliation in malignant cases, whereas surgical intervention may provide curative potential in selected benign cases. Individualized, multidisciplinary management is essential for optimizing outcomes.