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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HONselect – Caroli Disease

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. As medical imaging technology improves, diagnostic age decreases. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy.

Associated Data Supplementary Materials. Rev Med Chir Mal Foie. 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.

Check this box if you wish to receive a copy of your message. Intestinal atresia Duodenal atresia Meckel’s diverticulum Hirschsprung’s disease Intestinal malrotation Dolichocolon Enteric duplication cyst. It is named after Jacques Carolia French gastroenterologist, who described it in 7. Retrieved from ” https: Langerhans cell histiocytosis Lymphangioleiomyomatosis Cystic bronchiectasis.


Caroli disease | Radiology Reference Article |

Pyloric stenosis Hiatus hernia. Diagnostic methods The diagnosis is suspected on clinical grounds and confirmed through detection of cystic dilatation in the biliary tree through imaging studies. In other projects Wikimedia Commons.

Summary and related texts. 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. Before dying inhe was honored with the rank of commander in the Legion of Honour in Some patients remain asymptomatic throughout the disease course. Some develop intra- or extra-hepatic calculi, leading to recurrent cholangitis with bacteremia and sepsisand acute pancreatitis.

Caroli disease

Acta Paediatr Acad Sci Hung. The treatment depends on clinical features and the location of the biliary abnormality. Can Med Assoc J. Ciliopathy Hepatology Rare diseases Syndromes affecting the hepatobiliary system Syndromes with tumors.

Choledochal cysts Caroli disease Biliary atresia. This article has been cited by other articles in PMC. The diagnosis is suspected on clinical grounds and confirmed through detection of cystic dilatation in the biliary tree maldie imaging studies. Unable to process the form. There is a slight female gender bias. Antenatal diagnosis Cases of prenatal diagnosis based on ultrasonographic findings have been reported.

The second form is more diffuse, and when associated with portal hypertension and congenital kaladie fibrosisis often referred to as “Caroli syndrome.


Proc Staff Meet Mayo Clin. Case 3 Case 3. Biliary hamartomas Caroli disease Choledochal cysts Bile duct hamartoma. Antibiotics are used to treat the inflammation of the bile duct, and ursodeoxycholic acid is used for hepatolithiasis.

Exact prevalence and annual incidence data are not available for CD, but the disease is known to be very rare. The simple type presents with RUQ pain and recurrent attacks of cholangitis with fever and jaundice.

Fibrose hépatique congénitale.

When the intrahepatic bile duct wall has protrusions, it is clearly seen as central dots or a linear streak. Pseudocyst of carolk auricle Mucocele other and ungrouped: Besides bacterial cholangitis, complications include liver abscess, biliary infection, and in late stages, cholangiocarcinoma. Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia. Cutaneous ciliated cyst Hidrocystoma no epithelium: Assessment of portal venous hypertension by catheterisation of hepatic vein.

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Cases have also been found in infants and adults.