Purpose: Evaluation of neurocognitive function in children with intestinal failure (IF) is critical to optimize long-term outcomes. Recent research suggests that early cognitive abilities are delayed in this population, although later developmental outcomes are unknown. We examined the neuropsychological outcomes of children with IF at early school-age.

Methods: Prospective single centre neuropsychological assessments of children in an intestinal rehabilitation program between 2012-2016. Transplant recipients were excluded. Assessments included measures of general intellect (WPPSI-IV, WISC-IV/V), academics (WIAT-II/III), learning and memory (CMS, CVLT-C), language (PPVT-4, EVT-2), visual-motor integration (Beery VMI) and fine-motor dexterity (Purdue Pegboard). DSM-IV or V criteria was used to diagnose learning disability (LD), intellectual disability (ID), and/or attention deficit hyperactivity disorder (ADHD). Age-normed scores were correlated with social and medical variables.  

Results: Sample included 28 children (15 males), age 5-8 years, with the following etiologies: necrotizing enterocolitis (NEC) (8), gastroschisis (6), atresia (5), volvulus (4), Hirschsprung’s disease (3), and other (2). 17/28 (61%) were premature (<37 weeks gestational age (GA)) Overall, intellectual functioning was within the low end of normal range (mean Full Scale IQ=89, range: 53-123). 13/28 children (46%) received diagnoses: 8 LD, 3 ID, 2 combined LD and ADHD. Total number of septic episodes in the first year of life (median 2, range 0-7) was significantly correlated with lower scores on most measures, while having a sibling at home was a positive predictor (Table 1). Using linear regression to adjust for GA, total first-year septic episodes remains a significant predictor (p<.05) of working memory, visual-motor and visual memory scores, with a trend for predicting intellectual functioning (p=.067). Additional risk factors significantly correlated with lower scores on ≥2 cognitive functions include: length of hospitalization in first year of life, NEC diagnosis, gestational age, birth weight, and sustained conjugated bilirubin.

Conclusion: Early school-age assessment is important for children with IF, as they are at high risk of learning / attention issues. Medically effective treatment of septic episodes within the first year of life is critical for improving long-term cognitive outcomes. Certain social factors (e.g. sibling at home) are linked with positive outcomes.