Crouzon craniofacial dysostosis is a well recognised autosomal dominant craniosynostosis syndrome with a birth prevalence of approximately 1 in 60 0001. In 1912, a French neurologist, Octave Crouzon first described this syndrome in a mother and son with the characteristic triad of calvarial deformities, facial anomalies, and exophthalmos. Crouzon’s syndrome is caused by mutation of the FGFR2 gene on chromosome 10q25-10q263. Mutation of the FGFR gene is also responsible for other craniosynostosis such as Apert’s, Pfeiffer’s, Jackson-Weiss’, and Saether-Chotzen’s syndromes. Rarely, acanthosis nigricans may coexist with Crouzon’s syndrome and is caused by mutation in the transmembrane region of the FGFR3 gene (locus 4p16.3). Crouzon’s syndrome has no racial or sex predilections.
Crouzon syndrome was described in 1912 as one of the varieties of craniofacial dysostoses caused by premature obliteration and ossification of two or more sutures; most oftenly, coronal and sagittal sutures are involved. Incidence of Crouzon syndrome is currently estimated to occur in 1 out of every 25,000 people among the general population. For dentists, this disorder is important to understand as many of the physical abnormalities are present in the head, neck region and particularly the oral cavity. Common features are a narrow/high-arched palate, posterior bilateral cross bite, hypodontia, and increased spacing between teeth. Due to maxillary hypoplasia, Crouzon patients generally have a considerable permanent deep bite and subsequently cannot chew using their incisors.
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