[Orthotopic liver transplantation in therapy of advanced polycystic liver disease]

Polycystic liver disease (PLD) is an autosomal-dominant hereditary disease which usually presents together with polycystic kidney degeneration (ADPKD). The renal problems determine the course of this disease. Due to the development of dialysis an increasing number of patients present with symptoms from their liver cysts: These range from compression caused by hepatomegaly, which can severely limit the quality of life, to chronic liver failure. Ten patients with advanced symptoms of PLD underwent orthotopic liver transplantation, five of them with combined renal transplantation. Postoperative complications occurred in three patients. One patient died postoperatively from multiorgan failure after experiencing coagulopathy of unknown origin. After follow-up of 6-60 months, all patients had better quality of life after transplantation. There was a complete relief of symptoms; liver or renal failure did not occur. Liver transplantation should be considered in patients with highly symptomatic PLD. In the case of severe hepatomegaly or liver and renal failure the combined liver and renal transplantation are able to cure the PLD and ADPKD without rising the disadvantage of immunosuppression incurred with single organ transplantation.