Long-term neurocognitive and behavioral outcomes in patients with non-syndromic craniosynostosis.

Abstract

Purpose: In this study, we explore the long-term cognitive capabilities and behaviors of patients in late adolescence and early adulthood who underwent corrective surgery for craniosynostosis as infants.

Methods: Patients 16 years of age or older who had undergone surgery as infants for any type of non-syndromic craniosynostosis were identified from the operating records of three craniofacial plastic surgeons from a single institution. Participants underwent standardized neurocognitive testing using the BEERY and WASI. A subset of patients additionally underwent behavioral testing using the CAARS-2, ASR, SRS-2, and BRIEF standardized tests.

Results: Thirty-two participants underwent neurocognitive testing and 22 participants underwent behavioral testing. The mean scores for the WASI FSIQ-4, PRI, and VCI were not significantly different than the general population. The mean score for BEERY VMI was 95, BEERY VP was 97, and BEERY MC was 92, all of which were significantly lower than the population average. Twenty-three percent of participants scored high or very high on the CAARS-2 ADHD index, and of the 44 total patients contacted, 13.6% either confirmed having a diagnosis of autism or tested moderate-severe on the SRS-2 autism screener.

Conclusions: Patients have comparable neurocognition relative to the general population, with deficits in visual and motor integration. However, there is a significantly higher prevalence of ADHD and autism-related behaviors in this cohort. These findings are useful in counseling parents with infants who have been diagnosed with craniosynostosis, as well as in directing early screening and intervention for these patients to provide the resources and therapies families need.