Caroli disease

Caroli disease is a rare congenital disorder in which large intrahepatic bile ducts are segmentally and nonobstructively dilated. It can cause recurrent cholangitis, intrahepatic stones, and, in some cases, portal hypertension.

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Intrahepatic bile ducts: The bile-carrying ducts located within the liver that drain into the hepatic ducts. These are the ducts that become segmentally dilated in Caroli disease.
Biliary tree: The network of ducts that carries bile from the liver to the intestine. Caroli disease affects a specific portion of this branching duct network.
Magnetic resonance cholangiopancreatography: An MRI technique that produces detailed images of the bile and pancreatic ducts. It can show the characteristic saccular duct dilations and their communication with bile ducts.
Caroli syndrome: The combination of Caroli disease and congenital hepatic fibrosis. Unlike isolated Caroli disease, this form includes fibrosis and may cause portal hypertension.
Cholangiectasis: Abnormal dilation of bile ducts, occurring within the liver, outside it, or both. Caroli disease is a congenital, segmental form of intrahepatic cholangiectasis.
Ductal plate malformation: A developmental abnormality of embryonic biliary structures that can produce congenital liver and bile-duct disorders. Caroli disease is linked to abnormal remodeling of the embryonic ductal plate.
Computed tomography: An imaging method that creates cross-sectional views using X-rays and computer processing. CT can reveal intrahepatic duct dilation, stones, and associated liver changes.
Choledochal cyst: A congenital cystic dilation of the extrahepatic bile duct or, less often, intrahepatic ducts. Its typical extrahepatic location contrasts with the intrahepatic duct dilation of Caroli disease.
Bile stasis: Slow or impaired movement of bile through the biliary system. Dilated ducts can retain bile, encouraging stone formation and bacterial growth.
Autosomal recessive polycystic kidney disease: An inherited disorder causing cystic kidney disease and, often, congenital liver fibrosis. Its association with Caroli syndrome reflects shared developmental biliary abnormalities.