Levi Benjamin, Wan Derrick C, Wong Victor W, Nelson Emily, Hyun Jeong, Longaker Michael T
Hagey Laboratory of Pediatric Regenerative Medicine Research, Department of Surgery, Plastic and Reconstructive Surgery Division, Stanford University School of Medicine, Stanford, California 94305, USA.
J Craniofac Surg. 2012 Jan;23(1):13-9. doi: 10.1097/SCS.0b013e318240c6c0.
Craniosynostosis describes the premature pathologic partial or complete fusion of 1 or more of the cranial sutures. Over the past few decades, research on craniosynostosis has progressed from gross description of deformities to an understanding of some of the molecular etiologies behind premature suture fusion. Studies on patients with syndromic craniosynostosis have resulted in the identification of several genes, molecular events, and deformational forces involved in abnormal growth and development of the cranial vault. Conservation of craniofacial development and sequence homology between humans and other species have also led to insightful discoveries in cranial suture development. In this review, we discuss the development of the cranial vault and explain the basic science behind craniosynostosis in humans as well as in animal models and how these studies may lead to future advances in craniosynostosis treatments.
颅缝早闭是指一条或多条颅骨缝过早发生病理性部分或完全融合。在过去几十年里，对颅缝早闭的研究已从对畸形的大体描述发展到对过早缝融合背后一些分子病因的理解。对综合征性颅缝早闭患者的研究已确定了一些与颅穹异常生长发育相关的基因、分子事件和变形力。颅面发育的保守性以及人类与其他物种之间的序列同源性也在颅骨缝发育方面带来了有深刻见解的发现。在本综述中，我们讨论颅穹的发育，并解释人类以及动物模型中颅缝早闭背后的基础科学，以及这些研究如何可能带来颅缝早闭治疗方面的未来进展。
