Postoperative Outcomes Following Liver Transplantation for Wilson's Disease: A Systematic Review and Meta-Analysis

Abstract

Background: Liver transplant is indicated in patients with Wilson's disease for acute hepatic failure, advanced cirrhosis, and disease refractory to chelation therapy. This study aims to systematically review data about overall morbidity, hepatic, neuropsychiatric, and survival outcomes following liver transplantation for Wilson disease.

Methods: MEDLINE, Embase, and Central were searched from inception until July 2023. Peer-reviewed articles and published abstracts evaluating patients diagnosed with Wilson's disease and undergoing any type of liver transplant as a result of the disease were eligible for inclusion. A restricted maximum likelihood random effects model was used to generate the pooled proportion of each outcome. The risk of bias for each included observational study was assessed using the Methodological Index for Non-Randomized Studies tool.

Results: A total of 39 studies met all inclusion criteria. All studies were observational. Specific indications for liver transplant were most commonly acute liver failure (36.73%), chronic liver failure (45.02%), and acute-on-chronic liver failure (8.35%). The pooled proportions of mortality at 30 days, 1, and 5 years were 0.10 (95% CI 0.08, 0.13; I² = 16%), 0.11 (95% CI 0.09, 0.14; I² = 37%), and 0.15 (95% CI 0.11, 0.20; I² = 81%), respectively. The postoperative complication with the greatest prevalence was biopsy-proven acute rejection with a pooled proportion of 0.20 (95% CI 0.12, 0.31; I² = 84%). The mean MINORS score for risk of bias for all studies was 8.19.

Conclusion: Overall, reporting quality and consistency of outcomes included in the studies was poor as assessed using the MINORS score. Pooled proportions for 30-day, 1- and 5-year mortality are similar, suggesting most postoperative deaths are acute in nature. Future research should incorporate objective measures and the reporting of standardized parameters to allow more robust comparisons between studies.

Keywords: Wilson's disease; hepatobiliary surgery; liver transplantation; meta‐analysis; systematic review.

FIGURE 1

PRISMA diagram—Transparent reporting of systematic reviews and meta‐analysis flow diagram outlining the search strategy results from initial search to included studies.

FIGURE 2

30‐day mortality—Random effects by restricted maximum likelihood meta‐analysis of outcome using data from observational studies.

FIGURE 3

One‐year mortality—Random effects by restricted maximum likelihood meta‐analysis of outcome using data from observational studies.

FIGURE 4

Five‐year mortality—Random effects by restricted maximum likelihood meta‐analysis of outcome using data from observational studies.

FIGURE 5

Biopsy‐proven acute rejection—Random effects by restricted maximum likelihood meta‐analysis of outcome using data from observational studies.

FIGURE 6

Improvement in neurological and psychiatric symptoms—Random effects by restricted maximum likelihood meta‐analysis of outcome using data from observational studies.