Focal dilatation of intrahepatic bile ducts (Caroli's disease), cholangiocarcinoma, and sclerosis of extrahepatic bile ducts: A case report

J. Ludwig, R. H. Wiesner, Nicholas F La Russo

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Abstract

A 33-year-old woman with clinical and radiologic abnormalities interpreted as primary sclerosing cholangitis died of hepatic failure. Unexpectedly, the autopsy revealed focal dilatation of intrahepatic bile ducts (Caroli's disease) with cholangiocarcinoma. We know of only four previous cases of cholangiocarcinoma complicating focal dilatation of intrahepatic bile ducts. The lumen of the common hepatic duct and that of the common bile duct was narrowed by striking periductal fibrosis. Although tumor was found in the vicinity of these ducts, the periductal fibrotic sleeves were free of neoplastic tissue. These extrahepatic changes still may have been tumor-related, but they also may have been another, hitherto unobserved, manifestation of the fibropolycystic hepatobiliary diseases.

Original languageEnglish (US)
Pages (from-to)53-57
Number of pages5
JournalJournal of Clinical Gastroenterology
Volume4
Issue number1
StatePublished - 1982

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Bile Duct Diseases
Caroli Disease
Intrahepatic Bile Ducts
Extrahepatic Bile Ducts
Cholangiocarcinoma
Sclerosis
Dilatation
Common Hepatic Duct
Sclerosing Cholangitis
Liver Failure
Common Bile Duct
Autopsy
Neoplasms
Fibrosis

ASJC Scopus subject areas

  • Gastroenterology

Cite this

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abstract = "A 33-year-old woman with clinical and radiologic abnormalities interpreted as primary sclerosing cholangitis died of hepatic failure. Unexpectedly, the autopsy revealed focal dilatation of intrahepatic bile ducts (Caroli's disease) with cholangiocarcinoma. We know of only four previous cases of cholangiocarcinoma complicating focal dilatation of intrahepatic bile ducts. The lumen of the common hepatic duct and that of the common bile duct was narrowed by striking periductal fibrosis. Although tumor was found in the vicinity of these ducts, the periductal fibrotic sleeves were free of neoplastic tissue. These extrahepatic changes still may have been tumor-related, but they also may have been another, hitherto unobserved, manifestation of the fibropolycystic hepatobiliary diseases.",
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T1 - Focal dilatation of intrahepatic bile ducts (Caroli's disease), cholangiocarcinoma, and sclerosis of extrahepatic bile ducts

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AU - Ludwig, J.

AU - Wiesner, R. H.

AU - La Russo, Nicholas F

PY - 1982

Y1 - 1982

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AB - A 33-year-old woman with clinical and radiologic abnormalities interpreted as primary sclerosing cholangitis died of hepatic failure. Unexpectedly, the autopsy revealed focal dilatation of intrahepatic bile ducts (Caroli's disease) with cholangiocarcinoma. We know of only four previous cases of cholangiocarcinoma complicating focal dilatation of intrahepatic bile ducts. The lumen of the common hepatic duct and that of the common bile duct was narrowed by striking periductal fibrosis. Although tumor was found in the vicinity of these ducts, the periductal fibrotic sleeves were free of neoplastic tissue. These extrahepatic changes still may have been tumor-related, but they also may have been another, hitherto unobserved, manifestation of the fibropolycystic hepatobiliary diseases.

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