LIVER-TRANSPLANTATION FOR ADULT POLYCYSTIC LIVER-DISEASE

Polycystic liver disease, commonly associated with polycystic kidney d isease, can result in massive hepatomegaly and debilitating symptoms. Surgical intervention for symptomatic polycystic liver disease has bee n associated with significant morbidity and inconsistent long-term pal liation; it is more appropriate in patients with a single dominant cys t or cysts which is/are confined to one lobe, At our institution, nine patients have undergone orthotopic liver transplantation for symptoma tic hepatic cysts with excellent long-term results and minimal morbidi ty and mortality. Surgical candidates were selected based on severe li mitations in daily activities and on sequelae of hepatic cystic involv ement. Other factors considered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunction, and prio r surgical intervention, Three patients (33%) required combined liver and kidney transplantation because of renal cystic involvement with re nal insufficiency. The one-year survival rate was 89% with excellent s ymptomatic relief and improved quality of life in all the surviving pa tients. One death occurred in a significantly malnourished 62-year-old female. Complications included one case each of hepatic artery thromb osis requiring retransplantation, biliary leak necessitating biliary r econstruction, and postoperative bleeding requiring re-exploration. Th e mean hospital stay was 23 days and the mean intraoperative blood tra nsfusion requirement was 18 units. Our experience demonstrates that ap propriately selected patients with extensive hepatic involvement with adult polycystic liver disease can have an excellent outcome with tran splantation, with morbidity comparable with other surgical options.