ABSTRACT
Biliary atresia is an important but rare cause of neonatal jaundice. It is due to an idiopathic obstructive inflammatory process that affects both intra-and extra-hepatic bile ducts. Early diagnosis is essential, since delay in treatment results in irreversible liver damage. If untreated, progressive cholestasis leads to hepatic fibrosis and cirrhosis, and affected infants die from liver failure complicated by portal hypertension and ascites. In the UK, the incidence is approximately one in 16 000 live births. Girls are affected slightly more often than boys.
