Neurobehavioral Correlates of Craniosynostosis

描述（由申请人提供）：本项目将研究神经发育障碍与单缝颅缝早闭（SSC）之间的相关性，SSC定义为单颅缝过早融合。在婴儿期招募SSC受试者（n=240）和对照组受试者（n=240）（R 01 DE 013813），并在三个时间点进行评估：（1）颅骨成形术前（平均年龄6个月）;（2）年龄18个月;（3）年龄3岁。该项目的长期目标是更好地了解发育中的颅骨如何影响人类的生长和功能。现有的学龄儿童SSC的研究（主要是病例记录审查或小临床样本的评估）表明，学习，注意力和行为问题的风险大大增加。澄清SSC的影响是一个重要的公共卫生目标，因为家庭目前收到的关于颅骨成形术的理由相互矛盾的信息。一些医疗服务提供者认为，这一程序只是为了减少颅面毁容，而其他人认为，该程序可以预防或最大限度地减少神经发育障碍和大脑变形。具体目标是：1）比较有和没有SSC的学龄儿童在七个神经心理功能领域的神经发育（智力、语言能力、注意力和执行功能、学习和记忆、视觉运动技能、处理速度和学业成绩）; 2）使用在婴儿期/幼儿期评估期间获得的数据，为7岁时患有SSC的儿童开发功能结果的预测模型; 3）检查神经行为发育作为手术前至手术后骨结合严重程度变化的函数，如通过CT扫描的量化所确定的; 4）测试以下假设：当与年龄匹配的对照相比时，患有SSC的儿童将在MRI上显示更大程度的结构异常和减少的脑体积。

DESCRIPTION (provided by applicant): This project will investigate the association between neurodevelopmental impairment and single suture craniosynostosis (SSC), defined as the premature fusion of a single cranial suture. This cohort of participants with SSC (n=240) and controls (n=240) were recruited in infancy (R01 DE 013813) and assessed at three time points: (1) prior to cranioplasty (age 6 months on average); (2) age 18 months; and (3) age 3 years. The long term objective of this project is to better understand how the developing cranium affects human growth and function. Existing studies of school-age children with SSC (primarily case record reviews or evaluations of small clinical samples) suggest a substantially elevated risk of learning, attention and behavior problems. Clarification of the impact of SSC is an important public health objective because families currently receive conflicting information regarding the rationale for cranioplasty. Some health providers believe that this procedure is only necessary to reduce craniofacial disfigurement, while others believe that the procedure prevents or minimizes neurodevelopmental disorder and brain deformation. Specific aims are to: 1) Compare the neurodevelopment of school-age children with and without SSC across seven domains of neuropsychological functioning (intelligence, language abilities, attention and executive function, learning and memory, visual-motor skills, processing speed, and academic achievement); 2) Develop predictive models of functional outcomes for children with SSC at age 7 using data obtained during infancy/toddler assessments; 3) Examine neurobehavioral development as a function of pre- to post-surgery change in severity of synostosis, as determined by quantification of CT scans; 4) Test the hypothesis that children with SSC will show greater degree of structural abnormality and decreased brain volumes on MRI when compared to age-matched controls.