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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.
Summary
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.
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