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March 1966, Vol 111, No. 3 >
Article | March 1966

Picture of the Month FREE

SYDNEY S. GELLIS, MD; MURRAY FEINGOLD, MD; JAMES R. TOPPIN, MD; EDWARD R. McGOVERN, MD
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Received for publication Aug 18, 1964.

Reprint requests to 141 Midwood St, Brooklyn, NY 11225 (Dr. Toppin).


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

REFERENCES

1 +
Crouzon, M.O.:  Dysostose Cranio-faciale Hereditaire , Bull Soc Med Hop Paris 33:545, 1912;.
2 +
Lake, M.S., and Kuppinger, J.C.:  Craniofacial Dysostosis (Crouzon's Disease) , Arch Ophthal 44:37, 1950;.
3 +
Parks, M.M., and Castenbader, F.D.:  Craniofacial Dysostosis (Crouzon's Disease) , Amer J Ophthal 33:77, 1950;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Crouzon, M.O.:  Dysostose Cranio-faciale Hereditaire , Bull Soc Med Hop Paris 33:545, 1912;.
2 +
Lake, M.S., and Kuppinger, J.C.:  Craniofacial Dysostosis (Crouzon's Disease) , Arch Ophthal 44:37, 1950;.
3 +
Parks, M.M., and Castenbader, F.D.:  Craniofacial Dysostosis (Crouzon's Disease) , Amer J Ophthal 33:77, 1950;.

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