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