Crouzon syndrome (dysostosis craniofacialis; oxycephaly; craniofacial dysostosis; parrot-head syndrome; möbius-crouzon syndrome; hereditary craniofacial dysostosis)   327

General: Autosomal dominant; manifestations present at birth.

Ocular: Bilateral exophthalmos; hypertelorism (wide interpupillary distance); obliquity of palpebral fissures with outer canthus slanting downward; nystagmus; exotropia; upper field defects due to pressure upon the optic nerve on its lower part; bluish sclera; exposure keratitis in extreme exophthalmos; cataract; papilledema; secondary optic atrophy; corneal dystrophy; ptosis; strabismus; keratoconus.

Clinical: Prognathism; maxillary hypoplasia with short upper lip; synostosis of coronal and lambda sutures; parrot-beaked nose (psittachosrhina); widening temporal fossae; headaches.

David DJ, Sheen R. Surgical correction of Crouzon syndrome. Plast Reconstr Surg 1990; 85:344-354.

Gray TL, Casey T, Selva D, et al. Ophthalmic sequelae of Crouzon syndrome. Ophthalmology 2005; 112: 1129-l134.

Krause AC, Buchanan DN. Dysostosis craniofacialis (Crouzon). Am J Ophthalmol 1959; 22:140.

Kreiborg S, Aduss H. Pre- and postsurgical facial growth in patients with Crouzon's and Apert's syndromes. Cleft Palate J 1986; 23[Suppl l]:78-90.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: https://www.ncbi.nlm.nih.gov/omim/.

Muhlbauer W, et al. Early operation in craniofacial dysostosis. World J Surg 1989; 13:366-372.

Posnick JC. Craniofacial dysostosis. Staging of reconstruction and management of the midface deformity. Neurosurg Clin N Am 1991; 2:683-702.

Renier D, Marchac D. Craniofacial surgery for craniosynostosis: functional and morphological results. Ann Acad Med Singapore 1988; 17:415-426.

Strohecker B. Surgical reconstruction of patients with Crouzon's syndrome: a lifetime of needs. Plast Surg Nurs 1995; 15:23-29.

Turvey TA, Long RE Jr, Hall DJ. Multidisciplinary management of Crouzon syndrome. J Am Dent Assoc 1979; 99: 205-209.