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HPB Surgery
Volume 11 (1999), Issue 4, Pages 271-277
Case Report

Monolobar Caroli's Disease and Cholangiocarcinoma

1Department of Surgery, University of Florida, Gainesville, Florida, USA
2Department of Medicine, Division of Gastroenterology, University of Florida, Gainesville, Florida, USA
3Department of Pathology, University of Florida, Gainesville, Florida, USA

Received 7 February 1997

Copyright © 1999 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Caroli's Disease (CD) is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. This report describes a patient with cholangiocarcinoma arising in the setting of monolobar CD. In spite of detailed investigations including biliary enteric bypass and endoscopic retrograde cholangiography, the diagnosis of mucinous cholangiocarcinoma (CCA) was not made for almost one year. The presentation, diagnosis and treatment of monolobar CD and the association between monolobar CD and biliary tract cancer are discussed. Hepatic resection is the treatment of choice for monolobar CD.