Clinical problems with developmental anomalies of the biliary tract.

Cholestatic jaundice defined as conjugated hyperbilirubinemia is a typical feature of neonatal liver disease. Biliary atresia is the most common disorder producing cholestasis during the first 2 months of life. Syndromic and non-syndromic paucity of the intralobular bile ducts and choledochal cysts can also present with cholestasis during early life. Liver dysfunction from obstruction of the biliary tree must be differentiated from numerous disorders affecting hepatocytes such as congenital infection and inborn errors of metabolism. Early recognition and a stepwise diagnostic evaluation of the cholestatic infant are essential in successfully treating many metabolic and infectious liver diseases of the infant as well as surgically relieving obstruction in patients with biliary atresia.