Biliary atresia overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Saud Khan M.D.

Biliary atresia, is a disease of the liver in which the bile ducts, inside and outside, are blocked due to damage and scarring. In the congenital form, the common bile duct between the liver and the small intestine is blocked or absent. The acquired type most often occurs in the setting of autoimmune disease, and is one of the principal forms of chronic rejection of a transplanted liver allograft.

Babies born with biliary atresia can undergo surgery called the Kasai procedure before 8 weeks of age, however, a liver transplant may still be necessary.

Biliary atresia can be classified into several subtypes, since it does not arise from a specific cause. As such, it is broadly classified according to syndromic and non-syndromic expression. Davenport et al. grouped biliary atresia based on the similarities between the different presentations.

Biliary atresia is a progressive fibrosing obstructive cholangiopathy of the intrahepatic and extrahepatic biliary system, resulting in obstruction of bile flow and neonatal jaundice.

There have been many theories about etiopathogenesis such as Reovirus 3 infection, congenital malformation, congenital CMV infection, autoimmune theory. This means that the etiology and pathogenesis of biliary atresia are largely unknown.

Biliary atresia must be differentiated from other diseases that present with symptoms typical of cholestasis in neonates, jaundice and acholic pale stools, which are main features of obstructive jaundice, associated with conjugated (direct) hyperbilirubinemia.

Biliary atresia is a very rare disorder. About one in 10,000 to 20,000 babies in the U.S are affected every year. Biliary atresia seems to affect girls slightly more often than boys. Within the same family, it is common for only one child in a pair of twins or only one child within the same family to have it. Asians and African-Americans are affected more frequently than Caucasians. There does not appear to be any link to medications or immunizations given immediately before or during pregnancy.

If left untreated, patients with biliary atresia may progress to develop cirrhosis and liver failure. No cure exists for biliary atresia, but the timely diagnosis and surgical intervention improves short- and long-term outcomes in most patients. Special attention to the nutritional needs and diet are essential for children with this disorder. Special supplements, formulas, and dietary restrictions may be necessary for affected infants.