Biliary atresia is a rare condition of no known cause in which the biliary tract between the liver and the intestine is blocked or absent. It occurs in newborn children. Treatment is by surgery. If unrecognised, the disease leads to liver failure and kernicterus.

Symptoms and diagnosis

Initially, the symptoms are indistinguishable from neonatal jaundice, a common phenomenon. Prolonged jaundice that is resistant to phototherapy and/or exchange transfusions should prompt a search for secondary causes. By this time, liver enzymes are generally measured, and these tend to be grossly deranged. Ultrasound investigation or other forms of imaging can confirm the diagnosis.

Pathophysiology

There is no known cause of biliary atresia, although it is associated with a number of rare syndromes.

As the biliary tract cannot transport bile to the intestine, bile is retained in the liver and results in damage and the ultimate destruction of that organ.

Treatment

If the intrahepatic biliary tree is unaffected, surgical reconstruction of the extrahepatic biliary tract is possible.

If the atresia is complete, only liver transplantation is a therapeutic option.