Congenital hepatic fibrosis:: Findings at MR cholangiopancreatography

OBJECTIVE. This study describes the MR imaging features of congenital hepatic fibrosis in four children who were examined with MR cholangiopancreatography. CONCLUSION. MR cholangiopancreatography effectively reveals biliary cysts, dilatation of intrahepatic bile ducts, and polycystic kidney disease, findings often associated with congenital hepatic fibrosis. This diagnosis should be suspected when these biliary and renal abnormalities are associated with hepatosplenomegaly in a patient with normal liver function.