Purpose or Case Report: Case report: Congenital bronchobiliary fistula is a rare congenital malformation, with high morbidity and mortality. It is characterized by a communication between the respiratory and digestive tracts, between a bronchus and the bile duct.
This is a full-term female newborn patient, appropriate for gestational age, who from the third day of life presents with progressive respiratory difficulty associated with abundant waste through a nasogastric tube. She evolved with greater respiratory difficulty, so she was kept on a complete bowel rest and with a nasogastric tube, which increased his output. Airway aspiration was suspected, and an esophagus-stomach-duodenum study was performed to evaluate gastroesophageal reflux, which was normal. The study continued with abdominal ultrasound, showing aerobilia.
The patient evolves with severe respiratory distress and requires mechanical ventilation on day 15, so a chest tomography is performed, which shows aerobilia and a communicating tract between the right main bronchus and the bile duct.
Surgical management was performed with closure of the proximal end of the fistula at the supradiaphragmatic level, without intervening the intrahepatic tract.
The newborn later develops cholestatic syndrome, which is studied with cholangiopancreatography magnetic resonance (MRCP) that reports atresia of the extrahepatic bile ducts, performing an entero-biliary anastomosis in a second surgical procedure.
During her childhood after being discharged, she was diagnosed with several obstructive bronchial syndromes, cholangitis and malnutrition, remaining stable at the outpatient level. She is currently 15 years of age, her only chronic illness being with chronic liver disease, without evidence of clinical or elastographic fibrosis.
Bronchobiliary fistula presents as bronchitis and repeated bronchopneumonia, bilioptysis, cholangitis and sepsis in some cases. Proposed etiologies are either union of an accessory bronchus with an aberrant bile duct or duplication of the foregut. This diagnosis can be associated with other malformations such as bile duct atresia and right diaphragmatic hernia. Computed tomography with 2D and 3D reconstructions and MRCP are recommended for diagnostic workup.
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