General and Autism-Related Neurodevelopmental Difficulties in Biliary Atresia

To examine neurodevelopment in biliary atresia (BA) and the relationship of neurodevelopment to key disease-related factors.

In this single-center, observational study, we deployed an anonymized survey of outcomes that was completed by 107 parents of children with BA who were younger than age 12 years. A detailed assessment of general neurodevelopment (Mullens Scale of Early Learning and Vineland Adaptive Behavior Scale) was carried out in 50 infants younger than 5 years old, and emerging autistic traits (Autism Diagnostic Observation Schedule) were assessed in those eligible. Ninety-three age- and sex-matched infants, some with greater likelihood of neurodevelopmental conditions, were used as a reference.

Neurodevelopmental concerns were raised by 37% of parents, and 47% of children required support from at least 1 service. In the sample of children younger than 5 years, those with BA had significantly lower adaptive and cognitive skills (ANCOVA: Vineland Adaptive Behavior Scale, F = 18.26, P < .001; Mullens Scale of Early Learning, F = 9.981, P < .001) when compared with both children with lower and greater likelihood of neurodevelopmental difficulties. A clinical or research diagnosis of autism was made in 30% of 35 children >2 years old. Early surgical intervention and faster clearance of jaundice after surgery was associated with better general neurodevelopmental outcomes (F = 2.428, P = .042) but not with the presence of emerging autistic traits.

High levels of neurodevelopmental difficulties in children with BA reveal a need for greater awareness and enhanced surveillance. That early identification and treatment of BA is linked to better general neurodevelopmental outcome and encourages proactive management. However, the novel observation that BA is associated with autistic traits unrelated to disease management will need further investigation to establish clinical relevance and optimize clinical pathways.