Liver transplantation for Wilson disease: Current knowledge and future perspectives

Abstract

Liver transplantation currently represents a therapeutic option for patients Wilson’s disease presenting with end-stage liver disease or acute liver failure. Indeed, it has been associated with excellent post-operative survival curves, in view of young age at transplant and absence of recurrence. Attention has shifted over the past decades to a wise expansion of indications for liver transplantation. Evidence has emerged supporting transplantation of carefully selected patients with primarily neuropsychiatric symptoms and compensated cirrhosis. The rationale behind this approach is the potential for surgery to improve copper homeostasis and consequently ameliorate neuropsychiatric symptoms. However, several questions remain unanswered, such as how to establish thresholds for assessing pre-transplant neuropsychiatric impairment, how to standardize preoperative neurological assessments, and how to define post-operative outcomes for patients meeting these specific criteria. Furthermore, a disease-specific approach will be proposed both for the liver transplant evaluation of Wilson’s disease candidates and for patient care during the transplant waiting period, highlighting peculiarities of this systemic disease.