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.