Introduction Wilson's disease is an autosomal recessive disorder of copper metabolism which may manifest as acute or chronic liver disease and/or neuropsychiatric disorders. Orthotopic liver transplantation (OLT) is life saving when performed for acute liver failure or end-stage liver disease but there are conflicting reports on the effects of OLT on neuropsychiatric symptoms. Furthermore, a recent report from an Italian group has highlighted poor outcomes following OLT in those with neuropsychiatric symptoms.
Aim To evaluate the indications and outcomes of OLT for patients with Wilson's disease and in particular to assess the effects of OLT on neuropsychiatric symptoms, and whether these disorders are associated with poor outcomes.
Methods Data were acquired by retrospective analysis of case notes and databases.
Results 21 adults with Wilson's disease have been transplanted in Birmingham between 1987 and 2011. 53.3 % (11/21) were performed for acute liver failure and 42.8% (9/21) for decompensated cirrhosis. One patient was transplanted due to hepatocellular carcinoma. Median age was 19.5 at diagnosis and 25 at time of OLT. 10 patients had neuropsychiatric symptoms prior to OLT (Parkinsonism in three patients, anxiety in two patients, epilepsy, OCD, depression, ataxia and migraine). These symptoms resolved in 4/10 post-OLT. However, four patients developed new onset neuropsychiatric symptoms within 2 weeks of OLT; two patients had seizures, one developed psychosis and another became euphoric. Kayser–Fleisher (KF) rings are purportedly associated with neuropsychiatric disorders; in our cohort 61.9% (13/21) of patients had KF rings and 11/13 of these had neuropsychiatric symptoms. Four patients had significant post-operative complications comprising four haemorrhagic episodes and two biliary leaks. Actuarial survival rates were 95% at both 1 and 5 years. One patient died within 3 months of OLT and three patients died >10 years post OLT. 3/4 deaths were due to multi-organ failure secondary to sepsis. Graft survival was 90% at 1 year and 81% at 5 years. Five patients have required re-grafting; three due to chronic rejection and two due to hepatic artery thrombosis. There were no appreciable differences in long term outcomes between those with and without pre-existing neuropsychiatric symptoms; 2/4 deaths and 2/5 re-grafts were in those with neuropsychiatric manifestations.
Conclusion Outcomes of OLT for Wilson's disease are comparable to transplantation for other diagnoses. Neuropsychiatric symptoms improved in <50% of patients post-OLT and may also occur for the first time following OLT. At our centre pre-existing neuropsychiatric symptoms were not associated with poor outcomes.
Competing interests None declared.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.