Biliary Atresia

Biliary atresia is a progressive obliterative cholangiopathy affecting 1 in 15,000–20,000 North American live births, lethal without intervention.[e910-c3,e5518-c7,e7749-c1] The Kasai portoenterostomy remains first-line treatment, with timing critical: procedures before 45 days achieve 50% 12-year native-liver survival versus <20% after 75 days.[e662-c1,e7749-c6,e7754-c2] Success is defined by total bilirubin <2 mg/dL at 3 months, predicting 86% 2-year transplant-free survival.[e7753-c1,e7753-c2] The START trial definitively showed no benefit from routine high-dose corticosteroids post-Kasai, though selective protocols in younger cohorts (<45 days) may improve drainage rates from 40% to 84%.[e308-c2,e7753-c9,e7753-c13] Technical execution matters: optimal hilar transection at the fibrous plate–parenchyma junction yields 80%+ drainage success; extended dissection into liver substance reduces efficacy.[e7750-c9,e7750-c10] Kasai revision is indicated only for patients with initially successful drainage who develop recurrent cholangitis, not for primary failures. Biliary atresia remains the leading indication for pediatric liver transplantation, with 5-year survival now exceeding 90%, though only 30% achieve ideal long-term profiles free of immunosuppression sequelae.[e7750-c30,e7750-c33] Emerging biomarkers (MMP-7) and newborn screening protocols (direct bilirubin >1 mg/dL) promise earlier diagnosis, addressing persistent racial and socioeconomic disparities in referral timing.[e7749-c12,e7752-c13,e7752-c14,e9973-c14]