Neurobehavioral Correlates of Craniosynostosis

描述(由申请人提供)：该项目将调查神经发育障碍与单缝合颅融合(SSC)之间的关系，SSC定义为单一颅缝过早融合。这些患有SSc的参与者和对照组(n=240)是在婴儿期(R01 DE 013813)招募的，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)18个月；(3)3岁。该项目的长期目标是更好地了解发育中的头骨如何影响人类的生长和功能。现有对患有SSc的学龄儿童的研究(主要是病例记录回顾或对小临床样本的评估)表明，学习、注意力和行为问题的风险显著增加。澄清SSC的影响是一项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原理的信息相互矛盾。一些健康提供者认为，这种手术只是为了减少头面部毁容，而另一些人则认为，这种手术可以防止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)比较患有和不患有自闭症的学龄儿童在七个神经心理功能(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、处理速度和学习成绩)方面的神经发育；2)使用在婴幼儿/幼儿评估期间获得的数据，为7岁的自闭症儿童建立功能结果的预测模型；3)通过CT扫描的量化确定，检查神经行为发育作为手术前后联会严重程度变化的函数；4)验证这样一种假设，即与年龄匹配的对照组相比，患有SSc的儿童在MRI上显示出更严重的结构异常和脑体积减少。

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.