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Bronchobiliary fistula: a clinical and surgical challenge. Presentation of a pediatric case

Abstract:

INTRODUCTION: Congenital bronchobiliary fistula is an extremely rare malformation with high morbidity and mortality rates. Up to 2016, 36 cases had been reported worldwide. CLINICAL CASE: 11-year-old male patient with history of chronic lung disease and respiratory insufficiency, bile ptyalism and 66-80% arterial saturation, jaundice, asymmetric thorax, finger clubbing, and disseminated crackling rales. He was diagnosed through fibrobronchoscopy and CT-scan. After fistula closure and right pneumonectomy, recurrence occurred due to bile duct hypoplasia as evidenced by endoscopic retrograde cholangiopancreatography. Left lateral hepatic segmentectomy and fistula closure from the abdomen were carried out. Bronchopleural fistula persisted following intensive nutritional and antibiotic treatment. It was surgically closed using a bovine pericardial patch. Six months later, the patient had no symptoms. DISCUSSION: Given how extremely rare this malformation is, cross-disciplinary treatment and a high grade of suspicion are needed. The presence of bile duct hypoplasia is to be considered, since it requires a thoracoabdominal approach.