La revue de médecine interne – Vol. 33 – N° S2 – p. AA – Fièvre récurrente: penser à la maladie de Caroli – EM|consulte. C’est une cause rare de cholestase chronique et de lithiases intrahépatiques. Nous rapportons deux cas de maladie de Caroli monolobaire. Request PDF on ResearchGate | Maladie de Caroli monolobaire. À propos de 12 cas | BackgroundCaroli’s disease is the dilatation of the segmental intrahepatic.

Author: Tonris Sajinn
Country: El Salvador
Language: English (Spanish)
Genre: Automotive
Published (Last): 2 November 2011
Pages: 339
PDF File Size: 2.31 Mb
ePub File Size: 19.91 Mb
ISBN: 864-4-23786-435-8
Downloads: 61914
Price: Free* [*Free Regsitration Required]
Uploader: Maujas

HONselect – Caroli Disease

Some patients remain asymptomatic throughout the disease course. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy.

J Chir Paris Nov; The simple type presents with RUQ pain and recurrent attacks of cholangitis with fever and jaundice. Benhamouand R. Disease definition 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.

After reviewing 46 cases of Caroli disease before The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. Congenital fibrosis of the liver as a familial defect. Ciliopathy Hepatology Rare diseases Syndromes affecting the hepatobiliary system Syndromes with tumors.

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.

Caroli disease

Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree. Anatomical, clinical and nosological studies ]. Acta Paediatr Acad Sci Hung. Jacques Caroli, a gastroenterologistfirst described a rare congenital condition in in ParisFrance. Check for errors and try again. Cutaneous columnar cyst Keratin implantation cyst Verrucous cyst Adenoid cystic carcinoma Breast cyst.


Caroli disease is typically found in Asia, and diagnosed in persons under the age of Archived from the original on Webarchive template wayback links Infobox medical condition new.

On a CT scan, Caroli disease can be observed by noting the many fluid-filled, tubular structures extending to the liver. Asphyxiating thoracic dysplasia basal body: Caroli disease is also associated with liver failure and polycystic kidney disease. Antibiotics are used to treat the inflammation of the bile duct, and ursodeoxycholic acid is used for hepatolithiasis.

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Other search option s Alphabetical list. However, some series show that extrahepatic duct involvement may exist 2. Some develop intra- or extra-hepatic calculi, leading ee recurrent cholangitis with bacteremia and sepsisand acute pancreatitis.

Quality of life may be significantly affected by recurrent carkli. The manifestation of ductal plate malformation depends on the level of the biliary tree that is affected Eur J Gastroenterol Hepatol. You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Caroli disease is a rare autosomal recessive disorder which has no recognised gender predilection.

Only comments written in English can be processed.

Cutaneous ciliated cyst Hidrocystoma no epithelium: You can move this window by clicking on the headline. Thus, the simple type of Caroli disease results from the abnormal development of the large bile ducts. Alagille syndrome Polycystic liver disease.


The documents contained in this web site are presented for information purposes only. Morbidity is common and is caused by complications of cholangitis, sepsischoledocholithiasisand cholangiocarcinoma.

Abnormal development of the embryonic bile ducts at the stage of ductal plate has been suggested.

Caroli disease – Wikipedia

Ce Center Support Center. Support Radiopaedia and see fewer ads. Presentation is in childhood or young adulthood. With a liver transplant, cholangiocarcinoma is usually avoided in the long run.

Read it at Google Books – Find it at Amazon. This page was last edited on 28 Augustat Bowel gas and digestive habits make it difficult to obtain a clear sonogramso a CT scan is a good substitution. Maladie de Caroli monolobaire Monolobar Caroli disease.

Prognosis depends on the clinical course and the risk of cholangiocarcinoma. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

Copyright and License information Disclaimer. For all maladoe comments, please send your remarks via contact us. Access to the PDF text. Creative Commons Attribution 4.

Author: admin