ORTHOTOPIC LIVER-TRANSPLANTATION FOR SYMP TOMATIC POLYCYSTIC LIVER-DISEASE

Polycystic liver disease (PLD) is an autosomal-dominant hereditary dis ease which usually presents together with polycystic kidney degenerati on (ADPKD). The renal problems determine the course of this disease. D ue to the development of dialysis an increasing number of patients pre sent with symptoms from their liver cysts: These range from compressio n 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 combine d renal transplantation. Postoperative complications occurred in three patients. One patient died postoperatively from multiorgan failure af ter experiencing coagulopathy of unknown origin. After follow-up of 6- 60 months, all patients had better quality of life after transplantati on. There was a complete relief of symptoms; liver or renal failure di d not occur. Liver transplantation should be considered in patients wi th highly symptomatic PLD. In the case of severe hepatomegaly or liver and renal failure the combined liver and renal transplantation are ab le to cure the PLD and ADPKD without rising the disadvantage of immuno suppression incurred with single organ transplantation.