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March 1966

Picture of the Month

Author Affiliations

From the departments of pediatrics and neurosurgery, Brooklyn-Cumberland Medical Center and the State University of New York, Downstate Medical Center, Brooklyn.

Am J Dis Child. 1966;111(3):283-284. doi:10.1001/archpedi.1966.02090060093009

Denouement and Discussion 

Craniofacial Dysostosis  (Crouzon's Disease)

MAJOR MANIFESTATIONS  1. Craniofacial dysostosis; 2, exophthalmos with divergent strabismus; and 3, prognathism and beaked nose.

GENETICS  In the hereditary form, which constitutes 25% to 50% of the cases, there is an apparent irregular dominance.

ETIOLOGY  Syphilis, intra-uterine infections, rickets, birth trauma, and malfunction of endocrine glands are no longer considered etiological factors. Premature closure of the cranial sutures has been attributed to a genetic factor affecting the anlage of the sutures and the mesoderm from which the base and the sides of the skull develop.

PHYSICAL FINDINGS  There is always an abnormality in the shape of the skull, the abnormality depending upon the suture which is prematurely closed. Exophthalmos is quite striking and is almost always bilateral. Occasionally the exophthalmos may be so marked that it is impossible for the patient to close his eyelids. A divergent strabismus is usually present with

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