Purpose: Survival of infants with intestinal failure (IF) has improved, yet multiple medical factors may impact development. This study examines neurodevelopmental outcomes at 12-15 and 26-32 months corrected age in a cohort of children with IF.

Methods: Prospective single centre neurodevelopmental assessments of children admitted to an intestinal rehabilitation program between 2011-2014. Outcome measures include: Mullen Scales of Early Learning (12 and 26 months) and Vineland-II Adaptive Behaviour Scales (26 months). Standard scores were correlated with medical and demographic factors.

Results: Thirty-one children (17 males), median gestational age 34 weeks (interquartile range (IQR) 30-36) with birth weight (BW) 2.12 kilograms (IQR 1.24-2.52). Ten (32.2%) were dependent on parental nutrition (PN) at the second assessment. At 12 months, 11/30 (36.7%) scored below average (>1 standard deviation below mean) on the Early Learning (EL) composite and 24/30 (80%) were below average on the Gross Motor (GM) subscale. Comparatively, at 26 months, mean EL scores were significantly lower, most notably in Visual Reception (VR) and Fine Motor (FM) subscales (p=.001). Mean GM scores remained low, and parents reported the lowest scores on the motor domain of the Vineland-II. 

At 12-15 months, factors significantly (p<.05) associated with lower scores are primarily related to prematurity: lower VR scores correlate with lower BW (r= -.469) and longer neonatal intensive care admission (r= -.384), while lower FM scores correlate with longer NICU (r= -.442) and hospital (r= -.431) admissions.

At 26-32 months, the impact of IF-related variables, over and above early neonatal influences are more apparent. There is a significant correlation between lower FM scores and longer hospital admissions (r= -.392), while lower GM scores correlate with shorter small bowel length (r= .393) and greater PN days (r= -.506). Total PN days remain significant even when adjusting for prematurity.  

Additional significant risk factors include: central nervous system co-morbidities, necrotizing enterocolitis etiology and history of sustained conjugated bilirubin.

Conclusion: Children with IF are at risk of developmental delay in multiple domains. While gross motor skills appear to be most significantly affected, early learning deficits become more apparent at >2 years of age, which could impact future school performance. Early developmental follow up and intervention are important for this population.