Intrahepatic Stones and Caroli's Disease Y. Mishima 1 1The Second Department of Surgery, Tokyo Medical and Dental University, School of Medicine pp.381-386
Published Date 1984/4/25
DOI https://doi.org/10.11477/mf.1403106996
  • Abstract
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 Caroli's disease is defined as a congenital segmental dilatations of the intrahepatic bile ducts. Caroli himself classified this disease into two forms: the pure type without hepatic fibrosis and the more common type with periportal fibrosis. Clinically, cholangitis from early childhood is the characteristics of Caroli's disease. In the former type, the intrahepatic duct dilatation is observed mainly in the peripheral part of the liver and resulting cysts may contain stones. Microscopically, the liver parenchym is quite normal. In the latter, the liver is cirrhotic and symptoms due to portal hypertension are always dominant. To make the diagnosis, the intravenous cholangiography is essential, but hepatic scans, transhepatic cholangiography, endoscopic cholangiography, and ultrasonography are also helpful. Hepatic lobectomy or choledochojejunal anastomosis is the usual procedure, along with removal of the stones.

 Because there are some confusions as to its clinical definition, the Research Committee on Intrahepatic Stones, Ministry of Health and Welfare, Japan has concluded that Caroli's disease is not a real clinical entity but should be considered as a subtype of the congenital intrahepatic duct dilatations.

Copyright © 1984, Igaku-Shoin Ltd. All rights reserved.


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