Abstract

Craniosynostosis is a pathologic condition that results from premature fusion of one or more cranial sutures. It occurs in approximately 1:2000 live births. Since the brain expands rapidly in the first few years of life, premature closure of a cranial suture leads to compensatory calvarial expansion in a plane parallel to the fused suture. Untreated, craniosynostosis can cause a characteristic dysmorphic calvarial shape, midface hypoplasia, and can lead to deafness, blindness and mental retardation. In order to understand the dynamic mechanisms that mediate craniosynostosis, we needed to investigate the biologic processes, before, during and after suture fusion. Since clinical specimens limit our investigation to the time at which the samples are excised, we have employed murine models to examine the cascading events that lead to cranial suture fusion. These models have enabled us to dissect, isolate and understand the individual roles of the dura mater, pericranium, suture mesenchyme and osteogenic fronts.

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