{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T03:38:23Z","timestamp":1788406703155,"version":"build-2803163510"},"reference-count":35,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,7,15]],"date-time":"2021-07-15T00:00:00Z","timestamp":1626307200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To assess the relationship of surface area of the cerebral cortex to intracranial volume (ICV) in syndromic craniosynostosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>Records of 140 patients (64 males, 76 females; mean age 8y 6mo [SD 5y 6mo], range 1y 2mo\u201324y 2mo) with syndromic craniosynostosis were reviewed to include clinical and imaging data. Two hundred and three total magnetic resonance imaging (MRI) scans were evaluated in this study (148 patients with fibroblast growth factor receptor [<jats:italic>FGFR<\/jats:italic>], 19 patients with <jats:italic>TWIST1<\/jats:italic>, and 36 controls). MRIs were processed via FreeSurfer pipeline to determine total ICV and cortical surface area (CSA). Scaling coefficients were calculated from log\u2010transformed data via mixed regression to account for multiple measurements, sex, syndrome, and age. Educational outcomes were reported by syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Mean ICV was greater in patients with <jats:italic>FGFR<\/jats:italic> (1519cm<jats:sup>3<\/jats:sup>, SD 269cm<jats:sup>3<\/jats:sup>, <jats:italic>p=<\/jats:italic>0.016) than in patients with <jats:italic>TWIST1<\/jats:italic> (1304cm<jats:sup>3<\/jats:sup>, SD 145cm<jats:sup>3<\/jats:sup>) or controls (1405cm<jats:sup>3<\/jats:sup>, SD 158cm<jats:sup>3<\/jats:sup>). CSA was related to ICV by a scaling law with an exponent of 0.68 (95% confidence interval [CI] 0.61\u20130.76) in patients with <jats:italic>FGFR<\/jats:italic> compared to 0.81 (95% CI 0.50\u20131.12) in patients with <jats:italic>TWIST1<\/jats:italic> and 0.77 (95% CI 0.61\u20130.93) in controls. Lobar analysis revealed reduced scaling in the parietal (0.50, 95% CI 0.42\u20130.59) and occipital (0.67, 95% CI 0.54\u20130.80) lobes of patients with <jats:italic>FGFR<\/jats:italic> compared with controls. Modified learning environments were needed more often in patients with <jats:italic>FGFR<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Despite adequate ICV in <jats:italic>FGFR<\/jats:italic>\u2010mediated craniosynostosis, CSA development is reduced, indicating maldevelopment, particularly in parietal and occipital lobes. Modified education is also more common in patients with <jats:italic>FGFR<\/jats:italic>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.14984","type":"journal-article","created":{"date-parts":[[2021,7,15]],"date-time":"2021-07-15T19:53:37Z","timestamp":1626378817000},"page":"118-124","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Cerebral cortex maldevelopment in syndromic craniosynostosis"],"prefix":"10.1111","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4460-512X","authenticated-orcid":false,"given":"Alexander T","family":"Wilson","sequence":"first","affiliation":[{"name":"Department of Plastic and Reconstructive and Hand Surgery Erasmus University Medical Center Rotterdam the Netherlands"},{"name":"Section\u00a0of Plastic Surgery Yale School of Medicine New Haven CT 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