Monolobar Caroli's Disease and Cholangiocarcinoma

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 endosco...

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Bibliographic Details
Published in:HPB Surgery 1999, Vol.1999 (4), p.271-277
Main Authors: Abdalla, E K, Forsmark, C E, Lauwers, G Y, Vauthey, J N
Format: Article
Language:English
Subjects:
Aged
Bile Duct Neoplasms - diagnosis
Bile Duct Neoplasms - etiology
Bile Duct Neoplasms - surgery
Bile Ducts, Intrahepatic
Caroli Disease - complications
Caroli Disease - diagnosis
Cholangiocarcinoma - diagnosis
Cholangiocarcinoma - etiology
Cholangiocarcinoma - surgery
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis - diagnosis
Cholangitis - surgery
Cholecystectomy, Laparoscopic
Diagnosis, Differential
Follow-Up Studies
Hepatectomy - methods
Humans
Male
Recurrence
Tomography, X-Ray Computed
Treatment Outcome
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Description
Summary: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.
ISSN:0894-8569
1607-8462
DOI:10.1155/1999/70985