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

Describe the intelligence quotient (IQ) of children with Pierre Robin sequence (PRS). Prospective cohort study. Neurodevelopmental follow-up clinic within a hospital. Children with PRS (n = 45) who had been in the Neonatal Intensive Care Unit (NICU) were classified by a geneticist into 3 subgroups of isolated PRS (n = 20), PRS-plus additional medical features (n = 8), and syndromic PRS (n = 17) based on medical record review and genetic testing. Children with PRS completed IQ testing at 5 or 8 years of age with the IQ scores were more than 1 to 2 standard deviations below the mean for 36% of the overall sample, which was significantly greater compared to test norms (binomial test PRS subgroups can help identify children at risk for cognitive delay. The majority of children with PRS-plus had low intellectual functioning, in contrast to the third of children with syndromic PRS who had low IQ and the majority of children with isolated PRS who had average or higher IQ.

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Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.