Biliary atresia is a rare disease characterized by biliary obstruction which occurs during the perinatal period. It is the most common cause of neonatal cholestasis (biliary retention). The reported world incidence varies from 1/20 000 and 1/3 100 live births, Asia and Pacific countries being the most affected. Girls are slightly more affected than boys. Biliary atresia is a non hereditary condition of unknown origin.
Different types of biliary atresia (where bile ducts are partially or totally blocked or absent).
The diagnosis is suspected when a baby presents with Jaundice, discoloured stools and hepatomegaly (enlargement of the liver). Other aetiologies of a neonatal cholestatic icterus can be excluded upon further investigations of condition of the bile ducts. Untreated the disease leads to liver cirrhosis and to death within the first years of life.
Typical discoloured stool in biliary atresia. A primary treatment with medication has no sense in the treatment of biliary atresia. When a biliary atresia is suspected, a multi-step treatment begins: a surgical intervention in the form of a porto-enterostomie (Kasai procedure) must be performed as soon as possible during the newborn period, in order to restore bile flow to the intestine. During this surgery, the surgeon removes any problem bile ducts outside the liver. The small intestine is then attached to the liver. This provides a path that can allow bile to drain from the liver. If this operation does not succeed, or if liver cirrhosis develops, a liver transplantation might be needed.
Porto-enterostomie ( Kasai procedure)