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Intellectual Functioning of Children With Isolated PRS, PRS-Plus, and Syndromic PRS

Stephanie Malarbi1,2,3,4, Anita K Chisholm3,4, Julia K Gunn-Charlton1,2,3

  • 1The Royal Children's Hospital, Melbourne, Australia.

The Cleft Palate-Craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association
|July 28, 2022
PubMed

Insights

Children with Pierre Robin sequence (PRS) often experience cognitive delays, particularly those with PRS-plus. Subgrouping PRS patients aids in identifying those at risk for intellectual functioning deficits.

Area of Science:

  • Pediatric neurology
  • Developmental pediatrics
  • Genetics

Background:

  • Pierre Robin sequence (PRS) is a congenital condition characterized by a hypoplastic mandible, glossoptosis, and airway obstruction.
  • Cognitive development in children with PRS can be variable, necessitating further investigation into factors influencing intellectual outcomes.
  • Understanding the neurodevelopmental profile of children with PRS is crucial for early intervention and support.

Purpose of the Study:

  • To describe the intelligence quotient (IQ) of children diagnosed with Pierre Robin sequence (PRS).
  • To investigate the association between PRS subtypes and cognitive functioning.
  • To identify potential risk factors for cognitive delay in children with PRS.

Main Methods:

  • A prospective cohort study was conducted involving 45 children with PRS.
  • Children were classified into three subgroups: isolated PRS, PRS-plus (additional medical features), and syndromic PRS, based on genetic evaluation and medical records.
  • Intelligence quotient (IQ) was assessed at 5 or 8 years of age using standardized Wechsler scales (WPPSI-III/IV, WISC-IV/V).

Main Results:

  • Overall, 36% of children with PRS scored 1-2 standard deviations below the mean IQ, a significantly higher proportion than expected (P=.001).
  • A significant association was found between PRS subtype and IQ scores (P=.026).
  • Specifically, 75% of children in the PRS-plus subgroup exhibited IQ scores 1-2 standard deviations below the mean, compared to 35% in the syndromic PRS group and 20% in the isolated PRS group.

Conclusions:

  • Subgroup classification of PRS is valuable for identifying children at risk of cognitive delay.
  • The PRS-plus subgroup demonstrated a higher prevalence of low intellectual functioning.
  • Children with isolated PRS generally presented with average or higher IQ scores, while syndromic PRS showed a mixed cognitive profile.
Abstract

Keywords:
Pierre Robin sequencegeneticsmental developmentpsychological assessment

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