Transplantation 1993 Dec;56(6):1376-80

Surgical and metabolic aspects of liver transplantation for tyrosinemia

Luks FI, St-Vil D, Hancock BJ, Laberge JM, Bensoussan AL, Russo P, Mitchell G,
Lambert M, Blanchard H

Abstract
Tyrosinemia is the diagnosis of a very small percentage of patients undergoing
liver transplantation worldwide. Tyrosinemia is endemic within our referral area
however, and fully one-third of the liver transplantations at our institution
are done for this disease. Since 1986, 15 patients with tyrosinemia (TYR) and 31
patients with various other indications (non-TYR) have undergone a total of 51
liver transplantations. The 36-month actuarial survival for TYR patients is 87%,
compared with 74% for non-TYR patients. Liver transplantation for hereditary
tyrosinemia and other metabolic disorders without portal hypertension or
previous portohepatic operations is notably easier to perform. Intraoperative
blood loss was less, length of hospital stay was shorter, and incidence of
infections was lower in TYR than in non-TYR patients. Less than 10% of TYR
patients had foci of hepatocellular carcinoma at the time of transplantation.
For this reason, and while most patients with tyrosinemia will eventually
require liver transplantation, our results do not support systematic early
transplantation before the age of two years