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Congenital respiratory-biliary fistula

ORPHA:2040· ICD-10 Q32.4

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.

Prevalence
<1 / 1 000 000
Inheritance
Not applicable
Age of onset
Childhood