Crouzon syndrome: cephalometric analysis and evaluation of pathogenesis

Abstract

Crouzon syndrome is a craniofaciostenosis characterized by brachycephaly, ocular proptosis, and maxillary retrusion. The hypothesis has been forwarded that an alteration in anterior cranial base synchondrosis activity is responsible for the skeleton abnormalities which are associated with this disorder. The present work was aimed at assessing this pathogenetic hypothesis. Cephalometry was used as the analysis method and care was taken in determining the three-dimensional measurements of some functional spaces (e.g., orbit, rhinopharynx, and nasal cavity). The results indicate that in Crouzon syndrome the craniofacial alterations depend not only on reduced synchondrosis activity of the anterior cranial base, but also of the posterior cranial base.