Hepatology 1994 Mar;19(3):583-7

Liver transplantation for Wilson's disease: indications and outcome

Schilsky ML, Scheinberg IH, Sternlieb I
Department of Medicine, Albert Einstein College of Medicine, Bronx, New York
10461

Abstract
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