Biliary atresia

Abstract

Biliary atresia (BA) can be a devastating disease of infancy invariably leading, if untreated, to cirrhosis, liver failure and death. It is the commonest indication for pediatric liver transplantation throughout the developed world. In common with North America and Western Europe, the incidence in the UK is about 1 in 16,000 live births. It is clearly higher in countries such as Japan, and probably China, at about 1 in 10,000. All large series have a slight female preponderance. Although usually an isolated abnormality found in otherwise normal term infants, there are a group of infants who should be distinguished by the presence of other abnormalities and a poorer prognosis. About 10 % of infants in European and North American series (but only ~3 % in Japanese series) will have a specific constellation of anomalies which we have termed the Biliary Atresia Splenic Malformation (BASM) syndrome. The possible associations include polysplenia, asplenia, situs inversus, preduodenal portal vein, absence of the inferior vena cava, malrotation and congenital heart abnormalities. Most infants with this syndromic form of BA are female. © 2009 Springer Berlin Heidelberg.