Neurodevelopment in patients with biliary atresia up to toddler age: Outcomes and predictability.

Aim: To assess neurodevelopment in young patients with biliary atresia (BA) and to determine the predictive value of General Movement Assessment (GMA) at infant age for neurodevelopmental impairments at toddler age.

Method: Infants diagnosed with BA were prospectively included in a longitudinal study. Neurodevelopmental status was previously assessed before Kasai porto-enterostomy (KPE) and one month after KPE using Prechtl's GMA, including motor optimality scores. At 2-3 years, neurodevelopment was assessed using the Bayley Scales of Infant Development, and compared to the Dutch norm population. The predictive value of GMA at infant age for motor skills and cognition at toddler age was determined.

Results: Neurodevelopment was assessed in 41 BA patients. At toddler age (n = 38, age 29 ± 5 months, 70 % liver transplantation), 13 (39 %) patients scored below-average on motor skills, and 6 (17 %) patients on cognition. Abnormal GMA after KPE predicted both below-average motor skills and cognitive score at toddler age (sensitivity, 91 % and 80 %; specificity 83 % and 67 %; negative predictive value, 94 % and 94 %; and, positive predictive value, 77 % and 33 %, resp.).

Interpretation: One-third of toddlers with BA show impaired motor skills. GMA post-KPE has a high predictive value to identify infants with BA at risk of neurodevelopmental impairments.