Denouement and Discussion
1. Craniofacial dysostosis; 2, exophthalmos with divergent strabismus; and 3, prognathism and beaked nose.
In the hereditary form, which constitutes 25% to 50% of the cases, there is an apparent irregular dominance.
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.
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
SYDNEY S. GELLIS, MURRAY FEINGOLD, JAMES R. TOPPIN, EDWARD R. McGOVERN. Picture of the Month. Am J Dis Child. 1966;111(3):283–284. doi:10.1001/archpedi.1966.02090060093009