Summary

• A devastating disease requiring prompt early diagnosis preferably before 6 to 8 weeks of age.
• 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 excluded in any baby with jaundice associated with pale stools, with jaundice persisting beyond 14 days of age, or if the conjugated bilirubin is higher than 34.2 micromol/L (>2 mg/dL) or 20% of the total.
• Hepatoportoenterostomy is warranted in infants less than 100 days of age and 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 2 years of age.