Caroli Disease

 May fill with bile sludge, stones, &/or pus

image May affect entire liver or limited to 1 lobe, more commonly left


• 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

Radiologic image obtained by endoscopic retrograde cholangiopancreatography (ERCP) shows saccular dilatations of the intrahepatic bile ducts near the hilum image. Note the connection with normal-appearing ducts image .

Gross Appearance
This explanted liver shows cystically dilated bile ducts involving both left and right lobes image. 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 image .

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 image .



• Congenital cystic dilatation of intrahepatic biliary tree

• Communicating cavernous ectasia

• Type V choledochal cyst


• 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


Developmental Anomaly

• Total or partial arrest of remodeling of ductal plates during embryogenesis
image Caroli disease

– Affects larger ducts, mainly segmental ducts

image Caroli syndrome

– Affects larger and smaller ducts, including interlobular ducts

image Primarily autosomal recessive inheritance, but can be autosomal dominant

Disease Associations

Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Caroli Disease

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