Caroli Disease
Joseph Misdraji, MD
Key Facts
Terminology
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Congenital segmental dilatation of intrahepatic bile ducts
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Caroli syndrome when associated with congenital hepatic fibrosis
Clinical Issues
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Characterized by cholangitis, hepatolithiasis, abscess, and sepsis
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Associated with ARPKD
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Cholangiocarcinoma develops in 7-24% of cases
Image Findings
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Cholangiogram shows intrahepatic saccular duct dilatation
Microscopic Pathology
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Dilated intrahepatic bile ducts may show periductal fibrosis, inflammation, or frank abscesses
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Background liver can show congenital hepatic fibrosis
Radiologic image obtained by endoscopic retrograde cholangiopancreatography (ERCP) shows the saccular dilatations of the intrahepatic bile ducts near the hilum  . |
Gross photograph of liver shows clusters of dilated and cystic intrahepatic bile ducts  . |
TERMINOLOGY
Synonyms
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Congenital cystic dilatation of intrahepatic biliary tree
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Communicating cavernous ectasia
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Type V choledochal cyst
Definitions
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Caroli disease: Congenital segmental dilatation of larger intrahepatic bile ducts
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Caroli syndrome: Congenital segmental dilatation of larger intrahepatic bile ducts in conjunction with congenital hepatic fibrosis (CHF)
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
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Total or partial arrest of remodeling of ductal plate of larger intrahepatic bile ducts
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Caroli disease
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Only larger ducts affected
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