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Caroli disease is a rare congenital disorder characterized by segmental, non-obstructive, saccular or fusiform dilation of intrahepatic bile ducts. It falls under Type V of the Todani classification of Choledochal cysts .

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• Caroli disease: Involves only the dilatation of intrahepatic bile ducts.
• Caroli syndrome: Caroli disease with congenital hepatic fibrosis; often associated with portal hypertension and renal cystic disease such as autosomal recessive polycystic kidney disease (ARPKD).

Etiopathogenesis

• Autosomal recessive inheritance pattern.
• Associated with ductal plate malformation.
• May coexist with congenital hepatic fibrosis and renal cystic diseases (e.g., ARPKD)Grainger & Allison's - ….

Associated Conditions:

• Autosomal recessive polycystic kidney disease (ARPKD)
• Congenital hepatic fibrosis
• Medullary sponge kidney (MSK)

Clinical presentation

• Recurrent episodes of cholangitis.
• Right upper quadrant abdominal pain, fever, jaundice.
• Portal hypertension, splenomegaly, and esophageal varices (in Caroli syndrome).
• Increased risk of cholangiocarcinoma.

Complications