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Caroli's Disease.

Journal of the Korean Pediatric Society 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
선천성 간내 담도 확장증 (Caroli’s Disease) 1예
박종훈1, 조인현1, 이선자1, 금동희1, 배수동2
1고려병원 소아과
2고려병원 일반외과
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.
Key Words: Segmental saccular dilatation of intrahepatic bile ducts, Congenital hepatic fibrosis, Operative cholangiogram

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