- The term “craniosynostosis” refers to the premature fusion of one or more of the cranial vault sutures. The result is fusion of the bones adjacent to the suture and arrested sutural growth of the adjacent bones. The classic theory, known as Virchow’s law, states that premature fusion of a cranial suture results in limited development of the skull perpendicular to the fused suture and a compensatory overgrowth at the remaining open sutures.
- The craniofacial dysostosis syndromes (e.g. Crouzon, Apert, Saethre-Chotzen) are inherited forms of craniosynostosis. They follow an autosomal dominant inheritance pattern.
- The craniosynostosis of Crouzon, Apert, and Pfeiffer syndromes involve sites of pathogenesis either unilaterally or bilaterally along the calvarial articulations of the coronal ring. Most commonly, patients affected with one of these craniofacial dysostosis syndromes will present with bilateral involvement of the coronal sutures.
- Growth is parallel, restricted perpendicularly
- Coronal suture – brachycephaly
- Sagittal suture – scaphocephaly
- Metopic suture – trigonocephaly
