Craniofacial dysostosis syndromes: stages of reconstruction

The term "craniofacial dysostosis" is used in a general way to describe syndromal forms of craniosynostosis. These disorders are characterized by sutural involvement that not only includes the cranial vault but also extends into the skull base and midfacial skeletal structures. Craniofacial dysostosis syndromes have been described by Carpenter, Apert, Crouzon, Sathre-Chotzen, and Pfeiffer. Although the cranial vault and cranial base are believed to be the regions of primary involvement, there is also significant impact on midfacial growth and development. In addition to cranial vault dysmorphology, patients with these inherited conditions exhibit a characteristic "total midface" deficiency that is syndrome specific and must be addressed as part of the staged reconstructive approach.