Biliary Atresia Screening: Why, When, and How?

Abstract

Biliary atresia is one of the most important liver diseases in childhood. At an incidence of 1 in 13 000 live births in the United States, it is considered a rare disease; however, it is the indication for 40% to 50% of all liver transplants performed in children.1 It uniquely presents only during the first few months of life and seems to be a phenotype caused by at least several etiologies, including a proposed perinatal insult that initiates an immune-mediated obliteration of the extrahepatic bile duct lumen and a proposed embryonic or fetal defect in the normal morphogenesis of the biliary tree.2 Outcome is uniformly poor unless a hepatic portoenterostomy (HPE) (the Kasai operation) reestablishes bile drainage from the liver into the jejunum and leads to resolution of jaundice. If HPE is not successful or not performed, liver transplantation is the only life-saving alternative. Even with successful HPE, the majority of these children's conditions will progress to cirrhosis, leaving survival rates without liver transplantation at only 20% by 20 years … Address correspondence to Ronald J. Sokol, MD, Children's Hospital, Box B290, 13123 E 16th Ave, Aurora, CO 80045. E-mail: sokol.ronald{at}tchden.org