Congenital biliary malformations encompass a spectrum of structural defects in the extra- and intrahepatic bile ducts. The phenotypic expression is determined by the embryologic stage at which development is arrested and by the subsequent impairment of bile flow. Three cardinal symptom complexes dominate the clinical picture: cholestatic jaundice, recurrent cholangitis, and secondary hepatic dysfunction. Cholestatic JaundiceConjugated hyperbilirubinemia is the earliest and most consistent finding. In neonates it is usually recognized after the physiological jaundice window (>2 weeks of life) and is marked by progressive acholic stools and dark urine reflecting absent intra-intestinal bilirubin. In older children jaundice may fluctuate, often... Learn more