Biliary atresia

Biliary atresia is a rare condition in newborn children in which the biliary tract between the liver and the intestine is blocked or absent. If unrecognised, the condition leads to liver failure but not (as one might think) to kernicterus. It has no known cause, and the only effective treatment is by surgery. more...

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, hyperbilirubinaemia is conjugated and therefore does not lead to kernicterus. Ultrasound investigation or other forms of imaging can confirm the diagnosis.

Pathophysiology

There is no known cause of biliary atresia, although it may be associated with a number of rare syndromes, such as malrotation of the intestine.

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.

Links

E-medicine overview

Intro. to pediatric blood tests for liver function

Research Links

Choledochal cyst associated with extrahepatic bile duct atresia

Support groups

Biliary Atresia Network

Children's Liver Association for Support Services

Liver Families

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