Introduction: Liver transplantation (LT) is primarily indicated for children with Wilson’s disease (WD) presenting with Acute Liver Failure (ALF) or with chronic liver disease with decompensation (CLD). We herein present our experience with Living donor liver transplantation (LDLT) for paediatric WD and compare outcomes of children presenting with ALF or CLD.  
Methods: A retrospective analysis including all children with WD who underwent LDLT over a 5 year period was performed. Patient demographics,Lab parameters including Serum Ceruloplasmin,24 Hr Urinary Copper, Coomb’s negative haemolytic anemia, preoperative clinical variables,KF Ring,Leipzig score, surgical details, post-operative course and survival were collected. Presentation and outcomes of the ALF and CLD cohorts were compared.
Results: A total of 30 children (female=17, 56.7%) underwent LDLT for WD those with median Kings wilson index score of 15.  Median age was 9 years (IQR 7 -12). 16 had acute presentation (ALF/ACLF) while 14 patients had features CLD at presentation. ALF group had lower Hb (p=0.004),low ALP/Bilirubin ratio (p=0.001) higher white cell count (p=0.002),Total bilirubin (p=0.001),INR (p=0.001),  more haemolysis (p=0.029) and were more likely to be encephalopathic (p=0.003) and needing ventilatory and renal support. Median MELD/PELD at LT was 28 (CLD=18, ALF=36, p=0.001). Except Donor work up time  There was no difference in donor and grafts details, intraoperative blood loss or intraop transfusion requirement between two groups.
Median ICU and hospital stay for whole cohort was 4 and 15 days respectively. Major complications occurred in 8 patients (26.7%), including re-exploration in 4 (13.3%). There was no peri-operative mortality (90 days) in the entire cohort. Patients with ALF had longer post-operative inotrope requirement(37.5% vs 14.3% p=0.215), more post-op transfusion (81.3% vs 28.6% p=0.009),more neurological complications (50% vs 7.1% p=0.017),longer post-operative ICU stay (p=0.012) , longer hospital stay (p=0.006) and higer readmission within 3 months (p=0.143).  Three children (all ALF group) had invasive fungal infections. Survival at median follow up of 38 months was 96.7%, with no long term neurological sequelae. One mortality (ALF group)at 6 months due to persistent disseminated fungal infection
Conclusion: LDLT is a curative treatment for children with WD with excellent long-term outcomes.Post transplant survival is similar in both groups,All survivors having excellent graft function with no evidence of disease recurrence. Higher morbidity-particularly short-term neurological complications and longer post-operative stay is seen in those with acute presentation.