Biliary atresia

 

Biliary atresia is a rare disease that is characterised by a blockage in the biliary ducts (group of tubes that collect the bile produced in the liver and transport it to the intestine) occurring in the perinatal period. It is the most common cause of neonatal cholestasis. The incidence (the number of new cases of a pathology observed during a period and for a given population) reported worldwide varies between 1 in 20,000 and 1 in 3,100 live births; Asia and the Pacific countries are most affected. The incidence in Europe amounts to 1 in 18,000 live births. Girls are slightly more commonly affected than boys. Biliary atresia is a non-hereditary disease of unknown origin and is characterised by a blockage in the biliary ducts occurring in the perinatal period. It is the most common cause of neonatal cholestasis (biliary stasis).

The diagnosis is considered in cases of neonatal jaundice (icterus) with pale stools and hepatomegaly (increase in the volume of the liver detectable by palpation). If not treated, biliary atresia develops into biliary cirrhosis within several months and results in the death of the child during the first years of their life. As there is no drug treatment for biliary atresia, it is necessary to perform a Kasaï procedure (hepatoportoenterostomy). This procedure must be performed quickly in order to restore the bile flow to the intestine. If this procedure fails and the biliary cirrhosis progresses, a liver transplant may be necessary. The survival rate of children affected by biliary atresia is very good, and most will enjoy a near-normal quality of life.

Prof. Barbara Wildhaber
2011