The objective of this study was to determine the indications for and results of
liver transplantation in patients with
Wilson's disease on the basis of results of a survey with retrospective review of data obtained on 55 transplants performed at centers in the United States and Europe. The study group comprised 32 females and 23 males, aged 8.5 to 51 yr, with features diagnostic of
Wilson's disease. Indication for orthotopic
liver transplantation included
hepatic insufficiency (n = 32), wilsonian
fulminant hepatitis (n = 21), intractable neurological
Wilson's disease (n = 1) and
gastrointestinal hemorrhage (n = 1). Forty-three patients have survived, at this writing, from 3 mo to 20 yr. Mean and median survival after orthotopic
liver transplantation were 2.7 and 2.5 yr, respectively. Survival at 1 yr was 79%. Nonfatal complications occurred in five patients. Of the seven patients given transplants for
hepatic insufficiency who manifested neurological and/or psychiatric manifestations at the time of orthotopic
liver transplantation, four showed improvement of these symptoms. One patient given a transplant for intractable neurological disease improved but died of a vascular complication. Our data demonstrate that
liver transplantation is life-saving but not without risk for patients with wilsonian
fulminant hepatitis or chronic severe
hepatic insufficiency unresponsive to medical
therapy. Furthermore, neurological or psychiatric symptoms due to
Wilson's disease may improve after
liver transplantation; however, the role of this procedure in the management of patients with neurological
Wilson's disease in the absence of
hepatic insufficiency is still uncertain.