Caroli's Disease: Radiologic Spectrum with Pathologic Correlation
Angela D. Levy1,2,
Charles A. Rohrmann, Jr.1,3,
Linda A. Murakata4 and
Gael J. Lonergan1,2
1 Department of Radiologic Pathology, Armed Forces Institute of Pathology,
M-121, 6825 16th St, N.W., Washington, DC 20306-6000.
2 Department of Radiology and Nuclear Medicine, Uniformed Services University of
the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814.
3 Department of Radiology, University of Washington, 1959 N.E. Pacific, Seattle,
WA 98195-7115.
4 Department of Hepatic Pathology, Armed Forces Institute of Pathology,
Washington, DC 20306-6000.
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Fig. 1. —7-month-old male infant with Caroli's syndrome (Caroli's
disease accompanied by congenital hepatic fibrosis). Photomicrograph of
histopathologic specimen shows portal bile duct surrounded by chronic
inflammation. Lumen is dilated and contains soft, friable bilirubin calculi
(asterisk). Fibrous portal expansion with bile ductules along septa
(arrows) is consistent with congenital hepatic fibrosis. (H and E,
x20)
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Fig. 2. —48-year-old man with chronic renal failure, right upper
quadrant pain, fever, and chills. Percutaneous transhepatic cholangiogram
obtained through transhepatic drain shows mild fusiform dilatation of
extrahepatic bile ducts. Multiple strictures (open arrow) and
saccular dilatations (solid arrow) of right anterior segmental
intrahepatic bile ducts are seen. Remainder of visualized intrahepatic bile
ducts appears normal. Narrowing from normal sphincter mechanism is visible in
distal common bile duct.
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Fig. 3. —22-year-old woman with fever. Contrast-enhanced CT scan shows
marked intrahepatic ductal dilatation involving entire liver. Enhancing
central fibrovascular bundles (central dot sign) (arrow) are
identified in many of dilated ducts. Enlarged spleen is partially visible.
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Fig. 4. —26-year-old woman with recurrent cholangitis and septicemia.
Percutaneous transhepatic cholangiogram shows diffuse saccular dilatation of
intrahepatic bile duct and fusiform dilatation of extrahepatic duct.
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Fig. 5A. —51-year-old woman with cholangitis, bleeding esophageal
varices, and liver failure. Percutaneous transhepatic cholangiogram shows
diffuse fusiform intrahepatic bile duct dilatation. Multiple intrahepatic
biliary strictures, filling defects from intraductal lithiasis
(arrow), and focal narrowing in common bile duct are present.
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Fig. 5B. —51-year-old woman with cholangitis, bleeding esophageal
varices, and liver failure. Photograph of cut surface of autopsy liver
specimen shows extensive hepatic fibrosis, dilated ducts, and intrahepatic
biliary lithiasis (arrow).
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Fig. 6A. —41-year-old man with abdominal pain and fever.
Contrast-enhanced CT scan shows intra- and extrahepatic bile duct dilatation.
Many intrahepatic ducts contain peripherally enhancing fibrovascular bundles
(arrow).
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Fig. 6B. —41-year-old man with abdominal pain and fever. Photograph of
sectioned liver explantation specimen shows fibrovascular bundles
(arrow) located along periphery of dilated ducts.
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Fig. 7A. —77-year-old man with Caroli's disease and complicating
cholangiocarcinoma. ERCP shows fusiform dilatation of left hepatic duct, which
contains focal stricture and localized mass effect (solid arrows).
Note marked ectasia of more proximal duct that contains filling defect
(open arrow) from intrahepatic lithiasis.
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Fig. 7B. —77-year-old man with Caroli's disease and complicating
cholangiocarcinoma. Photograph of sectioned autopsy specimen shows tumor
infiltrating along course of left hepatic duct (black arrows) and
intrahepatic lithiasis (white arrow).
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Fig. 8A. —26-year-old woman with Caroli's disease and complicating
papillary adenocarcinoma. Contrast-enhanced CT scan shows saccular dilatation
of right intrahepatic bile ducts. Ducts contain enhancing soft-tissue masses
(asterisk).
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Fig. 8B. —26-year-old woman with Caroli's disease and complicating
papillary adenocarcinoma. Photograph of sectioned liver explantation specimen
shows cauliflowerlike masses of papillary adenocarcinoma protruding from
dilated intrahepatic bile ducts.
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Copyright © 2002 by the American Roentgen Ray Society.
