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강원석(W S Kang),최창수(C S Choi),주상언(S A Joo),박충기(C K Park),김진민(J M Kim),임규성(K S Lim) 대한소화기학회 1986 대한소화기학회지 Vol.18 No.2
Caroli's disease is characterized by congenital, segmental, saccular dilatations of the intrahepatic bile ducts. There are two types; the one type is only intrahepatic saccular dilation, the another is combined with Choledochal cyst, Congenital hepatic fibrosis, Medullary sponge kidney, or pancreatic cyst. This syndrome is rare and presents at any age, but usually in childhood or early adult life. Clinical manifestations are usually RUQ pain, and fever, chilling when combined with cholangitis. Here we are reporting a case of Caroli's disease diagnosed by ultrasonogram, abdominal computed tomography and percutaneous transhepatic cholangiogram. A brief review of the literature was attempted.