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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Fibrosis of the liver as a congenital anomaly. Caroli disease is a rare autosomal recessive disorder which has no recognised gender predilection.

Full text Full text is available as a scanned copy of the original print version. Using an ultrasoundtubular dilation of the bile ducts can be seen. Acta Paediatr Acad Sci Hung. Radiological, endoscopic, and surgical intervention may be required for patients with biliary obstruction, abscess formation and liver or bile duct stones.

Antibiotics are used for cholangitis. Polycystic kidney disease cargo: Caroli disease is also associated with liver failure and polycystic kidney disease. Ursodeoxycholic acid may be used to prevent stone formation. The key diagnostic procedure is magnetic resonance cholangiography showing a characteristic aspect of abnormal bile ducts.

Etude anatomique d’un nouveau cas. Cutaneous ciliated cyst Hidrocystoma no epithelium: The manifestation of ductal plate malformation depends on the level of the biliary tree that is affected Outline Masquer le plan.


After reviewing 46 cases of Caroli disease before Thank you for updating your details. 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.

Morbidity is common and is caused by complications of cholangitis, sepsischoledocholithiasisand cholangiocarcinoma. Articles Cases Courses Quiz. Some patients remain asymptomatic throughout the disease course. Multiple biliary hamartomas Multiple biliary hamartomas. Webarchive template wayback links Infobox medical condition new. Synonyms or Alternate Spellings: The treatment depends on clinical features and the location of the biliary abnormality. Recurrent pyogenic cholangitis Recurrent pyogenic cholangitis.

Fibrose hépatique congénitale.

Quality of life may be significantly affected by recurrent cholangitis. Intestinal atresia Duodenal atresia Meckel’s diverticulum Hirschsprung’s disease Intestinal malrotation Dolichocolon Enteric duplication cyst. Besides bacterial cholangitis, complications include liver abscess, biliary infection, and in late stages, cholangiocarcinoma. In contrast, in the periportal type of Caroli disease or Caroli syndromeboth the central intrahepatic bile ducts and the ductal plates of the smaller peripheral bile ducts are affected, with the latter leading to the development of fibrosis.

Annular pancreas Accessory pancreas Johanson—Blizzard syndrome Pancreas divisum.

Caroli disease | Radiology Reference Article |

Support Center Support Center. As medical imaging technology improves, diagnostic age decreases. Differential diagnosis The differential diagnosis should include primary sclerosing cholangitis, isolated polycystic liver disease, and hepatic cystic hamartoma see these termsas well as hepatic and choledochal cysts.


Congenital fibrosis of the liver as a familial defect. Caroli disease communicating cavernous ectasia, or congenital cystic dilatation of the intrahepatic biliary tree is a rare inherited disorder characterized by cystic dilatation or ectasia of the bile ducts within the liver.

Bardet—Biedl syndrome mitotic spindle: Top of the page – Article Outline. Cystic disease of the liver and kidney.

Check for errors and try again. This article has been cited by other articles in PMC. Support Radiopaedia and see fewer ads. Epidemiology Clinical presentation Pathology Radiographic features Treatment and prognosis History and etymology Differential diagnosis References Images: Log in Sign up.

In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. Case 3 Case 3. Journal page Archives Contents list.

Antenatal diagnosis Cases df prenatal diagnosis based on ultrasonographic findings have been reported. By using this site, you agree to the Terms of Use and Privacy Policy.