CAROLI’S SYNDROME- A RARE CASE REPORT

Caroli’s disease and caroli’s syndrome are two rare congenital diseases of intrahepatic bile ducts. Caroli’s syndrome characterized by saccular dilatation of intrahepatic bile ducts associated with congenital hepatic fibrosis. We present a case of 4 year male child who came with gradual distension of abdomen since birth with mild jaundice. Ultrasonography of whole abdomen revealed dilatation of intrahepatic bile ducts. Liver biopsy was done to confirm the diagnosis. On histopathological examination there was cholestatic, fibrotic and dilated intrahepatic bile ducts on H&E and Masson trichome stain. Caroli’s syndrome develop due to defect in bile duct differentiation which affect the smaller interlobular ducts and congenital hepatic fibrosis. Caroli’s disease confers an approximately 7% risk of malignancy. When disease is localized to one hepatic lobe, hepatectomy relieves the symptoms and appears to remove the risk of malignancy. In diffuse caroli’s disease, liver transplantation is treatment of choice.