Wilson's disease is an inherited disorder of
copper metabolism, presenting with prominent hepatic and
neurologic manifestations. There is an established place for
liver transplantation in the presence of
liver disease, while the indication for
neurologic manifestations is debated. Between 1993 and 2005, 11 patients were liver transplanted for
Wilson's disease at our institution. We retrospectively reviewed the medical records of the patients. The pathology of the explanted livers was analyzed. The patients were divided into three groups based on the evolution of the disease. Postoperative data gathered included patient and graft outcome, complications, neurologic status, and
copper metabolism. Six males and five females were transplanted at a mean age of 29.7 yr (range 15-48 yr). Three patients had a fulminant presentation, two patients had decompensation of established disease, and six patients had
chronic disease. Neurologic features were prominent in five patients. The pathologic analysis of the explanted graft showed
cirrhosis in all patients. The five patients with fulminant and acute on chronic presentations also showed
necrosis in the explant. The mean postoperative follow-up was 56.8 months (range 10-129 months). Two patients were re-transplanted. One patient died because of
severe sepsis. Two patients with severe
neurologic dysfunction showed significant remission of symptoms.
Liver transplantation is a safe and effective treatment for both acute and chronic presentations of
Wilson's disease. Acute presentation correlates with the presence of
necrosis in the explanted liver. In our series, there was a relevant improvement of the neurologic features after
transplantation.