Background: Orthotopic liver transplantation (OLT) is indicated for patients with acute liver failure (ALF) or decompensated cirrhosis due to Wilson Disease (WD). Goal: To review the long-term outcomes of patients who underwent OLT for WD. Methods: A retrospective review of data on 22 patients with OLT for WD from 1988-2007 at a single tertiary center. Results: Demographics, mode of presentation, renal function and outcomes are presented in figure 1. Mean length of follow-up was 11.4 ± 6.2 years (range 1.0-23.4 years). All children presented with ALF. Three adults underwent OLT after stopping medical therapy. Two patients died from intra- or immediate post-operative complications. 40.9% of patients required hemodialysis prior to transplant. After transplant, no patients required chronic hemodialysis or received kidney transplantation. Neurologic complications of WD other than encephalopathy were present in one patient with chronic WD pre-transplant and completely resolved after transplant.

Conclusions: OLT provides excellent long-term survival for patients with ALF or decompensated cirrhosis due to WD. Renal dysfunction, common in pre-transplant WD patients, completely resolved after transplant.

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