Biliary Atresia: Advances in Screening, Diagnosis, and Therapeutic Approaches

Biliary atresia is characterized by obstruction of the extrahepatic bile ducts and is the leading indication for pediatric liver transplantation. This review organizes recent advances across 3 intervals of the disease's natural history: before birth, from birth to the Kasai hepatic portoenterostomy, and after surgery. The initial biliary insult appears to begin in utero, with proposed etiologies spanning genetic, viral, immune, toxic, and vascular mechanisms. Because outcomes are best when surgery occurs within the first 30 to 45 days of life, early identification is essential.