Caroli disease

The Caroli 's syndrome, named after its describer Jacques Caroli (1902-1979), is a rare congenital disease of the biliary tract with cystic extensions of the bile ducts in combination with congenital fibrosis of the liver. The isolated expansion of the large bile ducts without connective tissue is known as Caroli 's disease.

Symptoms

In addition to the typical symptoms of Caroli 's disease with stones and recurrent infections in the bile ducts, the patients show the full picture of Caroli's syndrome blood stasis in the portal vein with enlargement of the spleen and varicose veins in the esophagus ( esophageal varices ), resulting in bloody vomiting can express. Here, the progression of the disease in different patients is very different and thus show the different symptoms in one earlier and in others much later.

Diagnosis

Similar to the Caroli 's disease is essential for the diagnosis of the syndrome Caroli the detection of a compound of the cysts in the liver with the bile ducts. This is by means of ultrasonography, computed tomography ( CT), magnetic resonance imaging ( MRI) including magnetic resonance cholangiopancreatography, ERCP or scintigraphy ( hepatobiliary Funktionsszintigrafie using radiolabeled lidocaine derivatives) possible. On MRI also evidence of vascular congestion in the portal circulation may result as an indication of liver fibrosis. However, this is ultimately a histological diagnosis and needs to be put under the microscope after a sampling of the liver ( liver biopsy).

Therapy

If the changes in the liver only one lobe affected, the disease can be cured by surgical removal of this part of the liver ( liver resection ). In a diffuse invasion may endoscopically performed a sphincterotomy or surgery, in which a short circuit connection between the bile ducts and the small intestine is made ( Choledochojejunostomy, Y - Roux hepaticojejunostomy ), improve bile drainage. A healing therapy is here but only by a liver transplant possible. The patients with congenital hepatic fibrosis seem to have a slightly worse survival rate than those with a simple Caroli 's disease, which may be due to the higher rate of infections also in the bile ducts at the time of transplantation.