A rare congenital liver disorder with varied clinical presentations –Report of two cases
Caroli’s disease and Caroli’s syndrome are rare congenital cystic disorders of intrahepatic biliary radicals. It is still unclear whether they represent distinct entities or, are different stages of the same disease distinguished by hepatic fibrosis. Here we are reporting two cases-a three-year-old boy presenting with portal hypertension, cholestatic jaundice and rickets, and a ten-year-old girl with recurrent episodes of cholangitis. They were diagnosed as Caroli’s syndrome and Caroli’s disease respectively.