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.

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This page was last edited on 28 Augustat Regular follow-ups, including ultrasounds and liver biopsies, are performed. Jacques Malzdie, a gastroenterologistfirst described a rare congenital condition in in ParisFrance. Ciliopathy Hepatology Rare diseases Syndromes affecting the hepatobiliary system Syndromes with tumors.

J Chir Paris Nov; Prognosis Quality of life may be significantly affected by recurrent cholangitis.

Pseudocyst of the auricle Mucocele other and ungrouped: Images in this article Fig. Support Center Support Center.

Congenital fibrosis of the liver as a familial defect. Ann Chir Thorac Cardiovasc.

Orphanet: Maladie de Caroli

When the intrahepatic bile duct wall has protrusions, it is clearly seen as central dots or a linear streak. Cystic disease of the liver and kidney. Alagille syndrome Polycystic liver disease. Assessment of portal venous hypertension by catheterisation of hepatic vein. Caroli disease is distinct from other diseases that cause ductal dilatation caused by dde, in that it is not one of the many choledochal cyst derivatives.


PKHD1 is expressed primarily in the kidneys with lower levels in the liverpancreasand lungsa pattern consistent with phenotype of the disease, which primarily affects the liver and kidneys. After cholangitis occurs, patients typically die within 5—10 years.

Prognosis is generally poor.

Fibrose hépatique congénitale.

Other search option s Alphabetical list. Radiological, endoscopic, and surgical intervention may be required for patients with biliary obstruction, abscess formation and liver or faroli duct stones. Caroli disease can present at any age. Caroli disease is also associated with liver failure and polycystic kidney disease. 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.

You can move this window by clicking on the headline. The disease may be diffuse, lobar or segmental. Caroli disease is a congenital disorder comprising of multifocal cystic dilatation of segmental intrahepatic bile ducts. Full text Full text is available as a scanned carloi of the original print version. Etude anatomique d’un nouveau cas. Turbo spin echo T2-weighted axial MRI of Caroli disease, showing cystic dilatations of bile ducts shown as white.

Check this box if you wish to receive a copy of your message. There are two patterns of Caroli disease: Synonyms or Alternate Spellings: Get a printable copy PDF file of the complete article 2. Eur J Gastroenterol Hepatol. It is also classified as a type V choledochal cystaccording to the Todani classification. For all other comments, please send your remarks via contact us.

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Caroli disease | Radiology Reference Article |

Personal information regarding our website’s visitors, including their identity, is confidential. Management and treatment Caaroli depends on the clinical presentation, localization and stage of the disease.

Links to PubMed are also available for Selected References. Choledochal cysts Caroli disease Biliary atresia. Additional information Further information on this disease Classification s 4 Gene s 1 Other website s 2. Views Read Edit View history. Antibiotics are used to treat the inflammation of the bile duct, and ursodeoxycholic acid is used for hepatolithiasis.

Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree. Cases have also been found in infants and adults.

Caroli disease

You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted. Endoscopic retrograde cholangiopancreatography ERCP and percutaneous transhepatic cholangiography PTC should not be used for diagnosis because they are associated with an increased risk of bacterial cholangitis.

Benhamouand R. As medical imaging technology improves, diagnostic age decreases. Most cases of CD are sporadic.

Pyloric stenosis Hiatus hernia.