1110 Evaluation of neurocognition in children with Biliary Atresia. (17th August 2022)

• Record Type: Journal Article
• Authors: Earl, Megan; Blackmore, Charlotte; Day, Jemma; McAlonan, Grainne; Samyn, Marianne; Dhawan, Anil
• Is Part Of: Archives of disease in childhood. Volume 107(2022)Supplement 2
• Page Start: A256
• Page End: A257
• Publication Date: 2022-08-17
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2022-rcpch.414
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Aims: Biliary Atresia (BA) is a progressive fibro-inflammatory liver disease of unknown aetiology, presenting in infancy. A surgical procedure (Kasai porto-enterostomy) is typically performed, ideally before the age of 8 weeks to try and restore the bile flow. Despite this, nearly half of the children will have undergone a liver transplantation by the age of 5 years. School performance and cognitive development in children and adolescents with BA is poorer compared to peers and similar for those surviving with their native liver and after liver transplantation. The neurodevelopmental difficulties in this population are poorly understood. In particular, studies on neurodevelopmental outcomes in young children with BA are scarce and tend to rely on parental feedback through questionnaires. This study aimed to characterize the cognitive and behavioral phenotype within BA infants by means of a neurodevelopmental assessment and to investigate the extent to which this group deviates from children of typical development (TD). Methods: 42 infants (male n=19) underwent a neurodevelopmental assessment (Mullens Scale of early learning (n=36), Vineland Adaptive Behaviour Scale interview (n=42)) at a mean age of 3.1 years (range 1.2-4.4 years) between October 2020 and January 2022. At time of the assessment 19 had received a liver transplant, 22 were surviving with their native liver, and 1 was on the transplant waiting list. A control group of 42 typically developing infants (TD), matched for age and gender, were recruited. Results: For the Vineland Adaptive Behaviour Scale interview, BA infants scored significantly lower compared to the control group ( t(82) = -5.05, p < .001) including in all separate domains (as can be seen in table 1 ). Similar results were recorded on the Mullens Scale of early learning (as can be seen in figure 1 ) both for total score ( t(66) = - 6.52, p < .001) and across all domains. Whereas BA children surviving with their native liver scored lower on both instruments than children who had received a liver transplant, this difference did not reach significance. Age at Kasai porto-enterostomy did significantly correlate with the Mullens Scale of early learning summary score, with individuals that had an early Kasai surgery having higher summary scores compared to those who presented later (r(33) = - .282, p = .050). Conclusion: Infants and children with BA, regardless of their transplant status, show lower levels of development across all domains, suggesting a global delay. Earlier surgical intervention seems to be a protective factor, but we emphasize that this is preliminary data. More work is ongoing to examine how the age of Kasai may influence neurodevelopmental outcomes and this information may inform surgical referral pathways. In addition, neurodevelopment assessments should become embedded in the routine medical care of children with BA in order to understand the trajectory of brain maturation in these children and to ensure neurodevelopmental needs are addressed alongside physical health.