Neurocognitive and Motor Functions in Biliary Atresia Patients: A Cross-sectional, Prospective National Cohort Study

Objectives: The aim of the study was to evaluate the neurocognitive and motor development of biliary atresia (BA) patients in childhood and adolescence and to identify risk factors for impaired outcome. Methods: We invited all BA patients between ages 1 and 20 years followed up at Helsinki University Children's Hospital in Finland between 1 January 2019 to 31 January 2020 to participate. All participants underwent age-appropriate validated neurocognitive tests. Participants between 3.0 and 16.9 years of age were assessed with the Movement Assessment Battery for children, version 2. Guardians of participants between ages 5 and 17 years filled the Five-to-Fifteen-Revised (5-15R) parental questionnaire. Results: The mean (+/- standard deviation [SD]) total intelligence quotient (IQ) of the 39 participants was 91 +/- 15, lower compared with test norms (mean IQ 100 +/- 15, P < 0.01). Earlier clearance of jaundice (COJ) had a positive effect on mean (+/- SD) total IQ (COJ = 3 months post-portoenterostomy 84 +/- 13, P < 0.05). Out of 30 participants assessed, 13 (43%) were either at risk or fulfilled the criteria for impaired motor development. Guardians reported elevated rates of functional difficulties affecting everyday life. There were no significant differences between native liver and liver transplanted (16/41%) groups. Conclusions: IQ is moderately, and motor scores markedly impaired in BA patients compared with normative data. Standardised cognitive and motor assessment before school-age for all BA patients is advisable to identify individuals in need of additional support.