Related Experiment Video
Updated: Apr 10, 2026
Technique of Porcine Liver Procurement and Orthotopic Transplantation using an Active Porto-Caval Shunt
Published on: May 7, 2015
Living Donor Liver Transplantation for Pediatric Wilson's Disease-related Acute Liver Failure-Hard Work With High
Somashekara H Ramakrishna1,2, Vellaichamy Katheresan3, Mohan B Kasala4
1Department of Pediatric Hepatology & Transplant Hepatology, Rainbow Children's Hospital, Marathahalli, Bangalore, India.
Insights
Living donor liver transplantation (LDLT) offers excellent outcomes for pediatric Wilson's disease (WD). While patients with acute liver failure (WD-ALF) face more complications, both WD-ALF and chronic liver disease (WD-CLD) groups achieve good long-term survival.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Transplant Surgery
Background:
- Wilson's disease (WD) is a genetic disorder of copper metabolism leading to liver damage.
- Liver transplantation (LT) is a crucial treatment for pediatric WD, especially in cases of acute liver failure (ALF) or decompensated chronic liver disease (CLD).
- Living donor liver transplantation (LDLT) is a viable option for pediatric WD patients.
Purpose of the Study:
- To evaluate the outcomes of LDLT in children with WD.
- To compare the presentation and outcomes of pediatric WD patients with acute liver failure (WD-ALF) versus those with chronic liver disease (WD-CLD).
Main Methods:
- A retrospective analysis of 53 children who underwent LDLT for WD.
- Comparison of clinical presentation, preoperative severity scores (Kings New Wilson Index, pediatric end-stage liver disease/model for end-stage liver disease), and need for critical care interventions between WD-ALF and WD-CLD cohorts.
- Assessment of postoperative complications, intensive care unit (ICU) and hospital stay, and patient survival rates.
Main Results:
- WD-ALF patients presented with higher disease severity scores, more frequent encephalopathy, and ongoing hemolysis compared to WD-CLD patients.
- WD-ALF patients required more intensive preoperative and operative support, including mechanical ventilation, continuous renal replacement therapy (CRRT), and therapeutic plasma exchange (TPE).
- The WD-ALF group experienced longer ICU and hospital stays, a higher incidence of major complications, postoperative neurological complications, and invasive fungal infections, with two perioperative deaths.
Conclusions:
- LDLT is a curative treatment for pediatric WD, yielding excellent short- and long-term outcomes.
- Despite a more complex postoperative course, children with WD-ALF who undergo LDLT demonstrate good long-term survival.
- Early and comprehensive management strategies are essential for optimizing outcomes in pediatric WD patients undergoing LDLT.
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, P = 0.001), higher pediatric end-stage liver disease/model for end-stage liver disease score (35 vs 20, P = 0.001), were more frequently encephalopathic (64% vs 4%, P = 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, P = 0.001), longer hospital stay (20.5 days vs 14 days, P = 0.001), more major complications (57% vs 20%, P = 0.006). WD-ALF cohort also had more postoperative neurological complications (42.9% vs 8%, P = 0.004) and invasive fungal infections (21.4% vs none, P = 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, P = 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.
More Related Videos
Related Concept Videos
01:28Kidney Transplant I: Introduction
01:26Kidney Transplant II: Surgical Procedure