Pathologies

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Fibrosing

Tissue may show unwanted scarring. Fibrosing means the formation of an excessive amount of scar tissue in organs or tissues during a reparative process. Tissues that are normally separated by membranes and sliding layers can stick together by collagen formation. This leads to annoying movement restrictions if no functional treatment is given.

In liver cirrhosis due to alcoholism, chronic inflammatory processes are the cause of fibrosis. The formation of 'advanced glycation endproducts' (AGEs) in diabetes patients also leads to tissue stiffening and to movement restrictions. In inflammatory processes, fibroblasts tend to randomly produce connective tissue if the repair process goes awry.

The pathogenesis is an embryonic ductal plate malformation, which leads to dilatations of the bile duct tree. Different types are distinguished. 

Types where there is no communication with the bile duct:

- biliary hamartomas: diffuse small (less than one centimeter) biliary cysts. The condition is benign and does not cause any symptoms. The radiological image can be confused with liver metastasis.

- polycystic liver diseases

Types in which there is permanent communication with the bile duct (large bile ducts):

- choledochal cysts;

- Caroli's disease or syndrome: This concerns isolated dilatations of the large intrahepatic bile ducts, which can lead to cholangitis. In the case of Caroli's syndrome, this is accompanied by congenital liver fibrosis and autosomal recessive polycystic kidney disease. In that case, there is a risk of portal hypertension, which can be complicated by esophageal varices.

Presents with marked hepatomegaly with multiple cysts.

Polycystic liver diseases are part of the autosomal dominant polycystic kidney diseases. These are genetic disorders of the cholangiocytes, which increase cell proliferation and secretion of these cholangiocytes and thus give rise to the development of biliary cysts. Rarely, the cysts are isolated in the liver, in which case it is autosomal dominant polycystic liver disease. Symptomatic PCLD occurs mainly in middle-aged and younger women. At least 10 large liver cysts are involved and the disorder is associated with cerebral aneurysms and mitral valve prolapse. The liver cysts may be complicated by bleeding or infection (mainly after previous kidney transplantation) or by marked hepatomegaly (> 5 liters) with secondary compression on the other organs. Treatment consists of stopping estrogens and administering somatostatin analogues. These inhibit bile secretion in the cyst and reduce the volume. In extreme hepatomegaly with the need for continuous analgesia and in the development of malnutrition, a liver transplant is indicated. In case of associated renal insufficiency, this should be done simultaneously with a kidney transplant.

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