Neurodevelopmental outcome of young children with extrahepatic biliary atresia 1 year after liver transplantation

STUDY DESIGN: Forty children < 2 years of age receiving extrahepatic liver transplantation were tested with the Bayley Scales of Infant Development before transplantation and again at 3 and 12 months after transplantation. Neurodevelopmental status 1 year after transplantation was organized by a descriptive statistic of normal, suspect, or delayed. Disease and transplantation variables were investigated for association with delayed neurodevelopmental outcome. RESULTS: Before transplantation mental development was in the low-average range (92 +/- 13.2) with psychomotor development 1 SD below the norm (82.5 +/- 13). Three months after transplantation both mental (80.1 +/- 12.6) and psychomotor (69 +/- 16.1) scores dropped 1 SD, but 1 year after transplantation mental and psychomotor scores recovered to the pretransplantation level of functioning. One year after transplantation 35% of the study group was diagnosed as developmentally delayed. Delayed development was associated with decreased weight (p < 0.04), low albumin (p < 0.02), length of hospital stay (p < 0.04), and age at transplantation (p < 0.05). CONCLUSION: Young children undergoing liver transplantation are at risk for developmental delay. Aggressive nutritional support before transplantation and timing of transplantation before malnutrition develops may reduce developmental delays.