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The Cognitive Profile of Children with Nonsyndromic Craniosynostosis

Kljajić, Marizela M.Sc.; Maltese, Giovanni M.D., Ph.D.; Tarnow, Peter M.D., Ph.D.; Sand, Peter Ph.D.; Kölby, Lars M.D., Ph.D.

Plastic and Reconstructive Surgery: May 2019 - Volume 143 - Issue 5 - p 1037e-1052e
doi: 10.1097/PRS.0000000000005515
Pediatric/Craniofacial: Original Articles

Background: Long-term neuropsychological and cognitive outcomes in patients with nonsyndromic craniosynostosis have proven difficult to evaluate objectively because of methodologic problems with published studies based on their small and biased samples of patients, wide age ranges, and testing with unacceptable psychometric properties. This study evaluated the Full-Scale Intelligence Quotient and its subscales in a cohort with a small selection bias.

Methods: Patients aged 7 to 16 years, born with nonsyndromic craniosynostosis and surgically treated, were tested using the Wechsler Intelligence Scale for Children, Fourth Edition. Ninety-one patients were invited, and 73 patients were tested.

Results: There was no difference in Full-Scale Intelligence Quotient score between patients who had undergone operations for sagittal synostosis or metopic synostosis and norms provided by the test. Patients operated on for sagittal synostosis showed a significantly higher perceptual reasoning intelligence quotient, but also significantly lower working-memory intelligence quotient and processing-speed intelligence quotient compared with the norms. Patients operated on for metopic synostosis showed no differences in any intelligence quotient index compared with the norm. In addition, attrition analysis showed no differences in background factors between responders and nonresponders.

Conclusions: These results derived from a group of patients with uniform age range, and tested using an established tool, revealed that nonsyndromic children having undergone surgery for craniosynostosis exhibited average intellectual ability. However, the analysis indicated possible issues with working memory and processing speed in patients operated on for sagittal synostosis, highlighting impairments potentially associated with neuropsychological problems and that might contribute to learning disabilities.

Göteborg, Sweden

From the Department of Plastic Surgery, Institute of Clinical Sciences and Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska Academy, Göteborg University, Sahlgrenska University Hospital.

Received for publication May 14, 2018; accepted September 26, 2018.

Disclosure:The authors have no financial interest to declare in relation to the content of this article.

Lars Kölby, M.D., Ph.D., Department of Plastic Surgery, Institute of Clinical Sciences, Sahlgrenska Academy, Göteborg University, Sahlgrenska University Hospital, SE-413 45 Göteborg, Sweden,

Copyright © 2019 by the American Society of Plastic Surgeons