4.1 Polycystic liver disease (CLD) 

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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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