111 Cortical Thickness | Crouzon INTRODUCTION The Crouzon-Pfeiffer syndrome is a common form of syndromic craniosynostosis,1 and mutations in the fibroblast growth factor receptor (FGFR2) gene are responsible for phenotypic severity in accelerated cranial suture fusion, facial anomalies, and exorbitism.2 Clinically, a severe sequela of (CP) syndrome is intracranial hypertension (ICH), which may be due to factors such as cranial growth restriction, venous outflow obstruction, hydrocephalus, and obstructive sleep apnea.3-5 Hence, cranial vault expansion is commonly performed, and at our center, it involves procedures such as fronto-orbital advancement, biparietal out-fracturing, and occipital expansion.6 Compared with fronto-orbital advancement, occipital expansion has produced a greater gain in intracranial volume at our center while reducing the incidence of papilledema and tonsillar herniation.7 Cerebral cortical thickness is an important in vivo biomarker for brain development and cognitive ability.8 9As a subcomponent of cortical volume, cortical thickness is a general measure of neuronal density, dendritic arborization, and glial support.10 Due to advancement in image-processing techniques, its use in recent years across a variety of disciplines has risen and demonstrated it to be of increasing importance in establishing a morphologic link to various pathologic and non-pathologic neuropsychological outcomes.9, 11-15 More recently it has demonstrated sensitivity to evidence of ICH in the syn-dromic craniosynostosis population.16 Almost two-thirds of patients with CP syndrome develop ICH and undergo cranial vault expansion,17 yet they exhibit—on average— global cortical thinning.16 Since most cases of CP syndrome develop with normal intelligence,18, 19 we wondered whether the apparent discrepancy between evidence of global cortical thinning and development of normal intelligence could be resolved by a better understanding of lobar cortical findings proximate to skull regions involved in cranial vault expansion procedures. Hence, the primary aim of this study was to compare differences in cortical thickness following frontal versus occipital primary vault expansion in CP syndrome patients. Our secondary aim was to determine whether any relationship between synostosis pattern and cortical thickness exists. 7
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