Caroli syndrome
| Classification according to ICD-10 | |
|---|---|
| Q44.5 | Other congenital malformations of the bile ducts |
| ICD-10 online (WHO version 2019) | |
The Caroli disease , named after its Erstbeschreiber Jacques Caroli (1902-1979) is a rare inherited disease of the bile ducts with cystic extensions of the bile ducts in combination with a congenital fibrosis of the liver . The isolated expansion of the major biliary tract without increased 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 biliary tract, patients with the full picture of Caroli's syndrome show blood congestion in the portal vein with an enlargement of the spleen and varicose veins in the esophagus ( esophageal varices ), which result in bloody vomiting can express. The progression of the disease is very different in different patients and thus the various symptoms appear earlier in one and much later in others.
diagnosis
Similar to Caroli's disease, the detection of a connection between the cysts in the liver and the bile ducts is crucial for the diagnosis of Caroli syndrome. This can be done using sonography , computed tomography (CT), magnetic resonance tomography (MRT) including magnetic resonance cholangiopancreatography , ERCP or scintigraphy (hepatobiliary functional scintigraphy using radioactively labeled lidocaine derivatives). The MRI can also show evidence of blood congestion in the portal vein circulation as an indication of liver fibrosis. However, this is ultimately a histological diagnosis and must be made under the microscope after a sample has been taken from the liver ( liver biopsy ).
therapy
If only one lobe is affected by the changes in the liver, the disease can be cured by surgical removal of this part of the liver ( liver resection ). In the case of diffuse infestation, an endoscopic papillotomy or an operation that creates a short-circuit connection between the biliary tract and the small intestine ( choledochojejunostomy , Y-Roux hepaticojejunostomy) can improve biliary drainage. A healing therapy is only possible here with a liver transplant . The patients with congenital liver fibrosis seem to have a slightly poorer survival rate than those with simple Caroli disease, which is possibly due to the higher rate of infections in the biliary tract at the time of the transplant.
Individual evidence
- ↑ a b c O. Yonem, Y. Bayraktar: Clinical characteristics of Caroli's syndrome. In: World Journal of Gastroenterology. Volume 13, 2007, PMID 17461492 , pp. 1934-1937.
- ^ S. Habib et al .: Caroli's Diesease and Orthotopic Liver Transplantation. In: Liver Transplantation. Volume 12, 2006, PMID 16498655 , pp. 416-421.
