Crouzon Syndrome

Crouzon syndrome was first described by a French neurosurgeon in 1912. It is the most frequent form of craniofacial dysostosis, occurring in approximately 1 per 25,000 births. About half of the cases occur as sporadic mutations and the remaining are familial and inherited as an autosomal-dominant trait [6,7]. There is occasional difficulty in differentiating between severe and mild forms of Crouzon syndrome, due to its variable expressivity (Fig. 26.1).

Seizures occur in approximately 12% of those with Crouzon syndrome but actual mental deficiency is found in only 3%. The association of increasing hydrocephalus is rare [1]. Conductive hearing deficits occur in 55% and cervical spine anomalies occur in approximately 30%, along with sacral and rib anomalies [7].

Crouzon syndrome is characterized by cran-iofacial skeletal anomalies; however, there is no clear characteristic pattern of limb involvement. A small proportion of Crouzon syndrome patients will have elbow abnormalities (ankylo-

Severe Crouzon
Fig. 26.1. a Mild form of Crouzon syndrome. b

sis or radial head sub-luxation) and minor hand abnormalities (short or malaligned fingers). Brachycephaly is the typical cranial form secondary to bilateral coronal synostosis. Other sutures may be involved, such as the lambdoid, sagittal and metopic sutures. There are also abnormal growth centers in the cranial base and facial sutures, leading to orbital, zygomatic and maxillary hypoplasia with normal soft tissue development. Often, there is associated upper-lid ptosis present, with significant exophthalmos, strabismus, exorbitism and hypertelorism. Associated with the mid-face hypoplasia, constricted maxilla, dental crowding and class III malocclusion is a paradoxical retrogenia. Intelligence is usually normal and the incidence of clefting is low. There are some general similarities in the craniofacial features displayed with Apert syndrome but, as a rule, Crouzon syndrome is a much less severe deformity, with a few distinct differences.

The initial surgical procedure for Crouzon syndrome is usually cranio-orbital decompression and reshaping. This is usually performed at 6-12 months, unless signs of elevated ICP

F Sil b

Moderately severe form of Crouzon syndrome.

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