Caroli’s Syndrome in a 5½-Year-Old Bangladeshi Girl: A Case Report

Caroli’s syndrome is a rare inherited disorder characterized by multiple segmental cystic or saccular dilatation of the intrahepatic bile duct associated with congenital hepatic fibrosis. Symptoms may appear early late during life and its presentation highly variable. Portal hypertension followed development oesophageal varices main consequence Up to 60% patients are renal involvement. The diagnosis mainly depends on histology imaging method that can show communication between ducts saccule. Important complication cholangitis later progress cholangiocarcinoma. For symptomatic syndrome, liver transplantation only curative treatment. Here, we report case in 5½ year old girl admitted department Pediatric Gastroenterology Nutrition, BSMMU complaints abdominal distension since birth. Her CT scan showed type V choledochal cyst large right lobe polycystic kidney disease. Endoscopy upper GIT revealed grade II MRCP also suggested disease (Type cyst). Finally, she was diagnosed as disease). J Enam Med Col 2021; 11(1): 55-58