Liver transplantation for polycystic liver disease

Polycystic liver disease (PLD) may provoke massive hepatomegaly and severe physical and social handicaps. Data on orthotopic liver transplantation (OL T) for PLD are tare and conflicting. Conservative surgery (resection or fen estration) is indicated for large single cysts, but its value for small dif fuse cysts is questionable. In addition, conservative surgery is not devoid of morbidity and mortality. OLT offers the prospect of a fully curative tr eatment, but controversy remains because those patients usually have preser ved liver function. Thus, we reviewed our experience with OLT for PLD. Sixt een adult women underwent OLT for small diffuse PLD between 1990 and 1999. Mean age was 45 years (range, 34 to 56 years). Fourteen patients had combin ed liver and kidney cystic disease, but only 1 patient required combined li ver and kidney transplantation, whereas 13 patients underwent OLT alone. Tw o patients had isolated PLD. Indications for transplantation were massive h epatomegaly causing physical handicaps (n = 16), social handicaps (n = 16), malnutrition (n = 4), and cholestasis and/or portal hypertension (n = 5). OLT caused no technical difficulty in 15 of 16 patients (surgery duration, 6.8 hours; range, 5 to 8 hours), with blood transfusions of 7.9 units (rang e, 0 to 22 units). One patient who underwent attempted liver-mass reduction pre-OLT died of bleeding and pulmonary emboli. Native liver weight was 10 to 20 kg. Posttransplantation immunosuppression consisted of cyclosporine o r FK506, azathioprine, and steroids (discontinued at 3 months). Morbidity i ncluded biliary stricture (2 patients), revision for bleeding and hepatitis (1 patient), pneumothorax and subphrenic collection (1 patient), and trach eostomy (1 patient). One patient died of lung cancer 6 years posttransplant ation. Both patient and graft survival rates are 87.5% (followup, 3 months to 9 years). Of 15 patients who underwent OLT alone, only 1 patient needed a kidney transplant 4 years after OLT. Kidney function has remained satisfa ctory in the other patients despite the use of cyclosporine or FK506 (last follow-up creatinine level, 1.55 mg/dL; range, 0.80 to 2.85 mg/dL). OLT had a dramatic impact on daily quality of life, enabling these patients to go back to a fully active life style. OLT offers the chance of a definitive tr eatment in patients with extensive, small, diffuse PLD that has evolved int o severely handicapping hepatomegaly. In contrast to previous studies, comb ined liver and kidney transplantation is rarely needed. Patient symptoms an d chances of definitive palliation offered by OLT must be balanced against the risks of transplantation and lifelong commitment to immunosuppression.