Liver transplantation for Wilson disease

Abstract

Liver transplantation (LT) is a life-saving and curative treatment for Wilson disease (WD), providing restoration of function of the liver and mitigation of portal hypertension. Indications for LT in patients with WD include acute liver failure or end-stage liver disease not treatable by medical therapy. LT is also used to treat hepatocellular carcinoma when it develops in patients with WD when tumor resection is not feasible. LT solely for neurologic or psychiatric WD remains controversial. Living liver donation as well as cadaveric orthotopic and auxiliary LT are options for transplantation for WD. Outcomes for LT for WD are excellent, and supportive measures while awaiting transplantation help bridge the patient to a more successful outcome. Future hepatocyte or stem cell transplantation may augment or supplant current LT for WD.

Keywords: Wilson disease; acute liver failure; end-stage liver disease; liver transplant.