부산대학교 도서관

We report a sister and brother with Caroli's disease. Case 1, a 23-year-old brother, was diagnosed with Caroli's disease after a close examination conducted due to pancytopenia and splenomegaly revealed intrahepatic bile ducts with cystic dilatation, multiple renal cysts, and esophageal varices. In case 2, a 32-year-old sister was found to have intrahepatic bile duct dilatation and several renal cysts, as did her brother, after esophageal varices ruptured, leading to the diagnosis of Caroli's disease. Case 1 repeated complications such as cholangitis and esophageal varices rupture, and developed noncompensated cirrhosis. Chronic renal failure caused by autosomal-recessive polycystic kidney disease deteriorated over time, eventually requiring hemodialysis. Since complications of Caroli's disease can lead to severe outcomes, it is necessary to perform a thorough search of family members at the time of diagnosis in addition to the patient to ensure early discovery of the disease.