Neurobehavioral Correlates of Craniosynostosis

在这项为期5年的多中心纵向研究中，将招募患有四种类型的单缝颅缝早闭的婴儿：矢状、额位、右侧单侧冠状和左侧单侧冠状（n=258）。 还将随访病例匹配的健康正常婴儿“对照”组（n=285）。 长期目标是绘制单缝融合的神经行为过程，并更好地了解发育中的颅骨如何影响人类大脑的生长和功能。 具体目标是：（1）明确婴儿期和幼儿期三个时间点单缝融合和非单缝融合婴儿的神经行为发育和父母适应情况（颅骨整形手术前和术后两次，分别在18和36个月时）;（2）颅缝早闭患儿中，阐明神经行为发育与骨和脑组织异常之间的关系，如术前CT扫描所示;（3）在患有单冠骨缝早闭的儿童中，阐明神经行为发育与存在基因突变之间的关系;（4）为患有颅缝早闭的婴儿建立36个月预后的预测模型;（5）确定颅骨整形手术的年龄与手术前后神经行为发育之间的关系。这项研究将在位于西雅图、芝加哥、圣路易斯和亚特兰大的医疗中心进行。 西雅图儿童医院和地区医疗中心是牵头机构。

In this multi-site, 5-year longitudinal study, infants with one of four types of single-suture craniosynostosis will be recruited: sagittal, metopic, right unilateral coronal, and left unilateral coronal (n=258). A case-matched "control" group of healthy, normal infants (n=285) will also be followed. The long-term objectives are to chart the neurobehavioral course of single-suture fusions and to better understand how the developing cranium affects human brain growth and function. Specific aims are to: (1) Clarify the neurobehavioral development and parental adjustment of infants with and without single-suture fusions at three time points in infancy and early childhood (just prior to cranioplastic surgery and twice post-surgery, at 18 and 36 months of age); (2) Among children with craniosynostosis, clarify relations between neurobehavioral development and abnormality in bone and brain tissue as indicated by measures taken pre-surgery CT scans; (3) Among children with unicoronal synostosis, clarify relations between neurobehavioral development and presence of genetic mutations; (4) Develop predictive models of 36-month outcomes for infants with craniosynostosis; and (5) Determine the relation between age of cranioplastic surgery and pre- and post- surgery neurobehavioral development. This research will take place in medical centers located in Seattle, Chicago, St. Louis and Atlanta. Seattle's Children's Hospital and Regional Medical Center is the lead agency.