Biliary atresia

Biliary atresia is a serious disease requiring prompt early diagnosis to optimise treatment outcomes. Treatment should ideally occur before 30-45 days of life.

A fibro-obliterative obstruction of the extrahepatic biliary tree progressing to intrahepatic ducts, which can develop in utero or during the neonatal period.

Presentation may include jaundice, pale stools, or hepatomegaly.

Biliary atresia should be suspected in any baby with jaundice and pale stools, jaundice or pale stools persisting beyond 14 days of age, or if the direct or conjugated bilirubin is ≥17.1 micromoles/L (≥1 mg/dL).

Hepatoportoenterostomy is warranted in infants without evidence of end-stage liver disease, but many go on to require liver transplantation within a few years.

Most common complications or sequelae are growth failure, portal hypertension, cholangitis, and ascites. Long-term management focuses on optimisation of growth and nutrition.

This condition is fatal without surgery and is the leading indication for paediatric liver transplantation. At least 70% of children with biliary atresia will undergo liver transplantation, 50% of them by age 2 years.

Biliary atresia is a progressive idiopathic, necroinflammatory process that may involve a segment or the entire extrahepatic biliary tree. Even with appropriate, timely surgical intervention, it is often an unrelenting inflammatory process.