Orthotopic liver transplantation for Wilson's disease - A single-center experience

Background. Wilson's disease is an inherited disorder of copper metabolism characterized by reduced biliary copper excretion, which results in copper accumulation in tissues with liver injury and failure. Orthotopic liver tra nsplantation (OLT) can be lifesaving for patients with Wilson's disease who present with fulminant liver failure and for patients unresponsive to medi cal therapy. The aim of this study is to review our experience with OLT for patients with Wilson's disease. Methods. Between 1988 and 2000, 21 OLTs were performed in 17 patients with Wilson's disease. Patient demographics, pre-OLT laboratory data, operative data, and early and late postoperative complications were reviewed retrospe ctively. One-year patient and graft survival was calculated. Results. Eleven patients had fulminant Wilson's disease; in six patients th e presentation was chronic. Mean patient age at presentation was 28 years ( range 4-51 years); mean follow-up was 5.27 years (range 0.4-11.4 years). Ne urologic features of Wilson's disease were not prominent preoperatively and did not develop post-OLT except in one patient who developed acute neurops ychiatric illness and seizure. Renal failure, present in 45% of patients wi th fulminant Wilson's disease, resolved post-OLT with supportive care. One- year patient and graft survivals were 87.5% and 62.5%, respectively. Fiftee n survivors have remained well with normal liver function and no disease re currence. Conclusion. Liver transplantation for hepatic complications of Wilson's dis ease cures and corrects the underlying metabolic defect and leads to long-t erm survival in patients who present with either acute or chronic liver dis ease. Acute renal failure develops frequently in patients with fulminant Wi lsonian hepatitis and typically resolves postoperatively.