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Neurodevelopment of children with single suture craniosynostosis: a review

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Abstract

Introduction

Rates of neurocognitive risk range from 35–50% of school-aged children with isolated single suture craniosynostosis (SSC). It has been hypothesized that early surgical intervention to release suture fusion reduces risk for increased intracranial pressure (ICP) and the corresponding risk to neurodevelopment. However, studies assessing children with SSC have been inconsistent in finding an association between neurocognitive development, age of surgery, and ICP.

Review

SSC produces notable distortion of the cranial vault and underlying brain mass. Although a linear relationship between skull distortion, ICP, and neurocognitive deficits has generally been assumed, recent studies have postulated an interactive process between the skull and developing brain that results in neuroanatomical changes that are not limited to areas directly beneath the fused suture. The specific neuropsychological deficits identified in children with SSC including problems with attention and planning, processing speed, visual spatial skills, language, reading, and spelling may be related to the anatomic differences that persist after correction of suture fusion.

Conclusions

Available literature on neurocognitive development of children with SSC is suggestive of mild but persistent neuropsychological deficits, which become more significant as cognitive demands increase at school age. Anatomical studies of children without SSC are beginning to identify particular groups of brain structures that if disrupted or malformed, may be associated with specific cognitive deficits. Controlled research investigating the relationship between persistent anatomical changes and neurocognitive functioning of school-aged children with SSC is needed.

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Acknowledgment

This work was supported by a grant from the National Institute of Dental and Craniofacial Research (NIDCR grant # R01 DE 13813 awarded to Dr. Speltz).

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Authors and Affiliations

  1. Department of Surgery, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Kathleen A. Kapp-Simon & Pravin K. Patel

  2. Department of Psychiatry and Behavioral Sciences, University of Washington, Seattle, WA, USA

    Matthew L. Speltz

  3. Children’s Craniofacial Center, Children’s Hospital and Regional Medical Center, Seattle, WA, USA

    Matthew L. Speltz & Michael L. Cunningham

  4. Division of Craniofacial Medicine, Department of Pediatrics, University of Washington, Seattle, WA, USA

    Michael L. Cunningham

  5. Department of Neurological Surgery, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Pravin K. Patel & Tadanori Tomita

  6. Division of Plastic Surgery, Children’s Memorial Medical Center, Chicago, IL, USA

    Pravin K. Patel & Tadanori Tomita

  7. Cleft Lip and Palate Institute, 2225 Enterprise Dr. Suite 2506A, Westchester, IL, 60525, USA

    Kathleen A. Kapp-Simon

Authors
  1. Kathleen A. Kapp-Simon
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  2. Matthew L. Speltz
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  3. Michael L. Cunningham
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  4. Pravin K. Patel
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  5. Tadanori Tomita
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Correspondence to Kathleen A. Kapp-Simon.

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Kapp-Simon, K.A., Speltz, M.L., Cunningham, M.L. et al. Neurodevelopment of children with single suture craniosynostosis: a review. Childs Nerv Syst 23, 269–281 (2007). https://doi.org/10.1007/s00381-006-0251-z

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  • DOI: https://doi.org/10.1007/s00381-006-0251-z

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