Neurocognitive outcomes in congenital diaphragmatic hernia survivors: a cross-sectional prospective study

J Pediatr Surg. 2016 Oct;51(10):1627-34. doi: 10.1016/j.jpedsurg.2016.05.011. Epub 2016 Jun 1.

Abstract

Background/purpose: Congenital diaphragmatic hernia (CDH) survivors may have persistent neurocognitive delays. We assessed neurodevelopmental outcomes in CDH survivors from infancy to late teenage years.

Methods: A cross-sectional study was conducted on 37 CDH survivors to examine neurocognitive functioning. Overall cognitive score was tested with the early learning composite (ELC) of Mullen Scales of Early Learning (n=19), and Full Scale IQ (FSIQ) of Wechsler Intelligence Scale for Children-Fourth Edition (n=18).

Results: ELC was 85.7±16.4, lower than the expected norm of 100, P=0.004, and 6 survivors had moderate, and 3 severe delay, which is not greater than expected in the general population (P=0.148). FSIQ was 99.6±19.1, consistent with the expected norm of 100, P=0.922, and 3 survivors had moderate and 2 severe delay, which is greater than expected (P=0.048). Although ELC was lower than FSIQ (P=0.024), within each testing group overall cognitive ability was not associated with participant age (ELC, P=0.732; FSIQ, P=0.909). Longer hospital stay was the only factor found to be consistently associated with a worse cognitive score across all participants in our cohort.

Conclusions: A high percentage of survivors with CDH have moderate to severe cognitive impairment suggesting that these subjects warrant early testing with implementation of therapeutic and educational interventions.

Keywords: Congenital diaphragmatic hernia; Cross-sectional study; Early learning composite score, ELC; Full scale IQ, FSIQ; Mullen Scales of Early Learning, MSEL; Wechsler Intelligence Scale–Fourth Edition, WISC-IV.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis
  • Cognition Disorders / etiology*
  • Cross-Sectional Studies
  • Developmental Disabilities / diagnosis
  • Developmental Disabilities / etiology*
  • Female
  • Hernias, Diaphragmatic, Congenital / complications*
  • Humans
  • Infant
  • Intellectual Disability / diagnosis
  • Intellectual Disability / etiology*
  • Intelligence Tests
  • Male
  • Neuropsychological Tests
  • Prospective Studies
  • Survivors