ABSTRACT
The pathology of biliary atresia is characterised by an obstructive biliary pattern that includes fibrosis, inflammation and proliferation. The Kasai procedure or portoenterostomy was first described by Morio Kasai in the 1950s as a treatment for biliary atresia. In this procedure the complete extrahepatic biliary tree is removed and the portal plate is mobilised and exposed. Steroid use in biliary atresia is controversial. Steroid use is postulated to stimulate bile flow and decrease biliary fibrosis. Long-chain fatty acids require chylomicrons for entrance into enterocytes and thus may not be absorbed in the setting of biliary atresia, leading to steatorrhoea. The investigation for biliary atresia includes clinical exam, laboratory data, imaging studies and liver biopsy. Typical findings on liver biopsy in biliary atresia are expansion of portal tracts, infiltration of inflammatory cells, portal tracts with bile plugs and parenchymal changes. Ventricular septal defect, atrial septal defect and hypoplastic heart are cardiac anomalies that have been associated with biliary atresia.
