Caroli disease is limited to the dilatation of larger intrahepatic bile ducts, whereas Caroli syndrome describes the combination of small bile ducts dilatation and congenital hepatic fibrosis. However, some series show that extrahepatic duct involvement may exist 2. They are also classified as a type V choledochal cyst , according to the Todani classification. Caroli disease and Caroli syndrome are rare autosomal recessive disorders with a slight female predilection.
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Caroli disease is limited to the dilatation of larger intrahepatic bile ducts, whereas Caroli syndrome describes the combination of small bile ducts dilatation and congenital hepatic fibrosis. However, some series show that extrahepatic duct involvement may exist 2. They are also classified as a type V choledochal cyst , according to the Todani classification.
Caroli disease and Caroli syndrome are rare autosomal recessive disorders with a slight female predilection. Presentation is in childhood or young adulthood. Caroli disease presents with RUQ pain, recurrent cholelithiasis and cholangitis with fever and jaundice. Caroli syndrome presents with the previous symptoms along with signs of portal hypertension, including hematemesis and melena secondary to bleeding varices.
The ductal plate is a layer of hepatic precursor cells that surround the portal venous branches, and is the anlage of the intrahepatic bile ducts. The manifestation of ductal plate malformation depends on the level of the biliary tree that is affected At the other end of the fibropolycystic disease spectrum are Von Meyenburg complexes, also known as biliary hamartomas which result from discrete foci of ductal plate malformation affecting the smallest bile ducts 5.
The disease may be diffuse, lobar or segmental. Dilatation is most frequently saccular rather than fusiform, a feature that might help in the differential diagnosis. Prognosis is generally poor. If disease is localized, segmentectomy or lobectomy may be offered.
In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. It is named after Jacques Caroli , a French gastroenterologist, who described it in 7. For a further CT differential, consider also focal hypodense hepatic lesions on a non contrast CT scan. Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Updating… Please wait. Unable to process the form.
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Read it at Google Books - Find it at Amazon. Promoted articles advertising. Edit article Share article View revision history Report problem with Article. URL of Article. Article information. System: Hepatobiliary. Tags: biliary tree , caroli disease.
Support Radiopaedia and see fewer ads. Cases and figures. Case 2: with medullary sponge kidney Case 2: with medullary sponge kidney. Case 3 Case 3. Imaging differential diagnosis. Recurrent pyogenic cholangitis Recurrent pyogenic cholangitis. Multiple biliary hamartomas Multiple biliary hamartomas. Loading more images Close Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys.
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Les infections et le cholangiocarcinome en sont les principales complications secondaires. Caroli Disease CD is a congenital dilatation of the intrahepatic bile ducts. In combination with liver fibrosis or cirrhosis, it is called Caroli Syndrome CS. Infectious complications and intrahepatic cholangiocarcinoma are secondary problems.
Maladie de Caroli
Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Caroli disease CD is a rare congenital liver disease characterized by non-obstructive cystic dilatations of the intra-hepatic and rarely extra-hepatic bile ducts. Exact prevalence and annual incidence data are not available for CD, but the disease is known to be very rare. There is a slight female gender bias.
Cholangite tuberculeuse simulant une maladie de Caroli : difficultés diagnostiques
We'd like to understand how you use our websites in order to improve them. Register your interest. Le foie et les voies biliaires sont une localisation exceptionnelle de la tuberculose TBC. The liver and the bile ducts represent an exceptional location of tuberculosis. The clinical symptomatology is not specific.