Biliary Atresia With Extrahepatic Cyst: A Diagnostic Dilemma

Biliary atresia (BA) is a rare disorder that usually presents with cholestatic symptoms such as jaundice, pale stool, and dark urine shortly after birth. Intracranial hemorrhage is a rare manifestation. BA may sometimes be associated with a cyst in the biliary tree. Differentiating choledochal cyst with BA or cystic biliary atresia can be quite challenging yet very important as they differ significantly in their prognosis and treatment. Furthermore, clear mucoid fluid in the extrahepatic cyst is an unusual occurrence. This case report aims to raise awareness about this rare variant to facilitate appropriate and timely diagnosis and treatment. We report a case of an infant who presented with intracranial hemorrhage and conjugated hyperbilirubinemia. The ultrasonographic and magnetic resonance cholangiopancreatography (MRCP) findings suggested Type I choledochal cyst, but it was later diagnosed as biliary atresia with an extrahepatic cyst and was not corrected by Kasai’s operation.