May affect entire liver or limited to 1 lobe, more commonly left
Microscopic
• Dilated ducts may show periductal fibrosis, acute and chronic inflammation, ulceration, or abscess formation
• Background liver may show features of congenital hepatic fibrosis or biliary cirrhosis
Top Differential Diagnoses
• Primary sclerosing cholangitis
• Recurrent pyogenic cholangitis
• Polycystic liver disease
ERCP Radiologic image obtained by endoscopic retrograde cholangiopancreatography (ERCP) shows saccular dilatations of the intrahepatic bile ducts near the hilum . Note the connection with normal-appearing ducts .
Gross Appearance This explanted liver shows cystically dilated bile ducts involving both left and right lobes . Bile sludge is present in the dilated ducts. The background liver shows fibrosis and cholestasis consistent with biliary cirrhosis on the background of congenital hepatic fibrosis.
Dilated Duct Transluminal Bridge This low-power view shows a massively dilated bile duct with inspissated bile in the lumen. The lining epithelium is partially denuded. A polypoid fibrovascular cord protrudes into the lumen to form a bridge .
Bile Duct Inflammation This dilated bile duct shows a mixed inflammatory cell infiltrate in the wall, consisting mainly of histiocytes and neutrophils. Fibrosis is also evident. The luminal surface is eroded by inspissated bile .
TERMINOLOGY
Synonyms
• Congenital cystic dilatation of intrahepatic biliary tree
• Communicating cavernous ectasia
• Type V choledochal cyst
Definitions
• Caroli disease: Segmental dilatation of larger intrahepatic bile ducts without other hepatic abnormalities
• Caroli syndrome: Segmental dilatation of larger intrahepatic bile ducts in association with congenital hepatic fibrosis
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
• Total or partial arrest of remodeling of ductal plates during embryogenesis
Caroli disease
– Affects larger ducts, mainly segmental ducts
Caroli syndrome
– Affects larger and smaller ducts, including interlobular ducts
Primarily autosomal recessive inheritance, but can be autosomal dominant
Disease Associations
• Autosomal recessive polycystic kidney disease
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. Note the connection with normal-appearing ducts 
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. Bile sludge is present in the dilated ducts. The background liver shows fibrosis and cholestasis consistent with biliary cirrhosis on the background of congenital hepatic fibrosis.
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