All issues > Volume 24(4); 1981

Case Report

J Korean Pediatr Soc. 1981;24(4):401-406. Published online April 15, 1981.

Caroli's Disease.

Jong Hoon Park¹, In Hyun Cho¹, Sun Ja Lee¹, Dong Hyuk Kum¹, Soo Dong Pai²

¹Dept, of Pediatrics，Korea General Hospital, Seoul, Korea
²Dept, of Surgery, Korea General Hospital, Seoul,Korea

Abstract

Caroli*s disease, a rare syndrome characterized by congenital, segmental saccular dilatation of intrahepatic bile ducts, is first described by Caroli & associaties in 1958. It is though by many to be part of spectrum of congenital hepatic fibrosis. The disease usually presents with bile stasis and stone formation with further complication arising from recurrent cholangitis and liver abscess. The preoperative diagnosis is not easy but should be considered in patients who have recur-rent episode of cholangitis, cholestasis, biliary tract stones, recurrent urinary tract infections, ureteric colic or hematuria particulary if urography demonstrates renal cysts or medullary-sponge kidney. Definite diagnosis was made by operative cholangiogram aad/or percutaneous transhepatic cholangiogram in most cases. We experienced one case of Caroli’s disease in 11-year-old girl who was admitted due to epigastric pain and intermittent fever. Liver scanning revealed diffuse liver abscess pattern. We present one example of Caroli’s disease with review of literature.

Keywords :Segmental saccular dilatation of intrahepatic bile ducts;Congenital hepatic fibrosis;Operative cholangiogram