Biliary atresia is a rare disease with high morbidity and mortality for which the natural history is reasonably well understood.
• Early performance of the Kasai portoenterostomy with successful surgical drainage of bile is associated with better outcomes for infants with biliary atresia; therefore, educational programs for pediatricians and other pediatric care providers to increase awareness of biliary atresia may be helpful.
• Published analyses indicate that newborn screening for biliary atresia, either by measuring serum conjugated bilirubin concentrations or using stool color cards, is potentially of sufficient sensitivity and specificity to be cost-effective.
• Further studies are necessary to evaluate the feasibility, effectiveness, and costs of potential screening strategies for early identification of biliary atresia in the United States.