Background, With the poor results of resective and fenestration proced
ures for polycystic liver disease (PCLD), we present the first series
of patients receiving orthotopic liver transplantation for this condit
ion. Methods. Five of our six patients with PCLD had polycystic kidney
disease also. Three of these five received combined organ transplants
, while the other two required subsequent kidney transplants. Results,
Forty-eight and 52 months after orthotopic liver transplantation, all
surviving patients had relief of their pain, distention, and anorexia
, Two patients had succumbed to infectious complications and died at 1
5 and 24 months after transplant. Conclusions. We conclude that patien
ts with PCLD can be transplanted safely for the relief of their disten
tion and anorexia, with good results. Those patients with both PCLD an
d polycystic kidney disease who are not dialysis dependent can be mana
ged for several years with isolated liver transplantation and then rec
eive kidney transplantation if needed. Those who are dialysis dependen
t should receive combined liver-kidney transplantation. Unfortunately,
patients with polycystic disease seem to be very susceptible to infec
tious complications after organ transplantation.