BILIARY TRACT DISEASE Biliary Atresia Goals of Treatment BA is uncommon but is associated with very high morbidity and mortality, and is a leading cause of liver transplant in young children Early detection, before 30 to 45 days of life, can significantly improve patient outcomes and potentially avoid transplantation In the United States, the average age of diagnosis unfortunately remains at approximately 60 days of life The goal of an ED provider is to suspect BA and facilitate its diagnosis and subsequent definitive surgical care to improve patient outcomes In patients who have undergone surgical correction, an ED provider must recognize high-risk complications, including ascending cholangitis, as well as progression of underlying disease leading to cirrhosis, portal hypertension, and liver failure CLINICAL PEARLS AND PITFALLS Patients with BA have an elevated conjugated (direct) bilirubin shortly after birth, even within the first 24 to 48 hours of life According to the American Academy of Pediatrics, all infants with persistent jaundice beyond to weeks of age should have their conjugated bilirubin measured to evaluate for cholestasis BA should be considered in any young infant with even minimal conjugated hyperbilirubinemia (greater than normal) regardless of the ratio to unconjugated bilirubin Identification within the first 30 to 45 days of life leads to improved outcomes Patients with acholic stools should be investigated for biliary disease, however acholic stools and hyperpigmented urine are relatively late findings Cholangitis is the most common complication after a hepatoportoenterostomy (Kasai) procedure Current Evidence