Living Donor Liver Transplantation for Pediatric Wilson's Disease-related Acute Liver Failure-Hard Work With High Rewards.

BACKGROUND

Liver transplantation (LT) is indicated for children with Wilson's disease (WD) presenting with acute liver failure (ALF) or with chronic liver disease (CLD) that has progressed to decompensation. We present our experience of living donor liver transplantation (LDLT) for pediatric WD, discuss the challenges of managing WD-ALF and compare outcomes of children presenting with WD-ALF with WD-CLD.

METHODS

We compared presentation and outcomes of the WD-ALF and WD-CLD cohorts. Fifty-three children (WD-ALF: 28 (53%), WD-CLD: 25 (47%)) underwent LDLT for WD.

RESULTS

WD-ALF group had higher Kings New Wilson Index (KNWI) (15 vs 9,  = 0.001), higher pediatric end-stage liver disease/model for end-stage liver disease score (35 vs 20,  = 0.001), were more frequently encephalopathic (64% vs 4%,  = 0.001), and had ongoing hemolysis (86% vs 28%, <0.001). Preoperative mechanical ventilation, operative continuous renal replacement therapy (CRRT), therapeutic plasma exchange (TPE) was needed in 32%, 46.5%, and 89% of WD-ALF children, respectively. WD-ALF patients had longer postoperative ICU stay (4.5 days vs 3 days,  = 0.001), longer hospital stay (20.5 days vs 14 days,  = 0.001), more major complications (57% vs 20%,  = 0.006). WD-ALF cohort also had more postoperative neurological complications (42.9% vs 8%,  = 0.004) and invasive fungal infections (21.4% vs none,  = 0.024). There were two perioperative (90 day) mortalities in WD-ALF group and none in WD-CLD group. Patient survival of the entire cohort at median follow-up of 26 months was 94.3% and all survivors had good allograft function neurological sequelae. Patient survival was inferior for WD-ALF cohort though the difference was not statistically significant (88.5% vs 100%, log rank test,  = 0.089).

CONCLUSION

LDLT is a curative treatment for children with WD with excellent short-term and long-term outcomes. WD-ALF patients can have a complicated postoperative course but have good long-term survival.