Congenital respiratory-biliary fistula

Definition

Congenital respiratory-biliary fistula (RBF) is a rare developmental defect characterized by an anomalous connection of trachea or bronchus with left hepatic duct presenting with respiratory distress, recurrent respiratory infections and biliary expectoration or vomitus.

Disease data

Classification

Morphological anomaly

ORPHA code

2040

OMIM code

ICD10 code

Q32.4

ICD11 code

*Soruce