Biliary atresia accounts for approximately 60% of the liver transplantations in infants younger than 1 year of age.
These complicated early transplantations can be prevented only with the use of the Kasai hepatoportoenterostomy. The success of the Kasai procedure is varied, but a good outcome is more likely if the operation is performed before 30 to 45 days of life. Unfortunately, in the United States, infants with biliary atresia are usually identified later and the average age at surgery is 60 to 70 days.
To address this problem, the American Academy of Pediatrics recently requested more studies to evaluate screening strategies for biliary atresia. Potential strategies can capitalize on the well-characterized natural history of the disease. In the preclinical phase, infants appear normal but have an elevated serum concentration of direct or conjugated bilirubin at birth, which rises and plateaus over time. Signs such as jaundice and pale stools develop in the first months of life. Without intervention, end-stage liver disease develops in all infants with biliary atresia before 1 year of age.
Screening of the direct or conjugated bilirubin concentration in newborns can identify infants with biliary atresia early, before symptoms develop. Larger studies are needed to confirm the benefits of screening and to assess the cost-effectiveness.