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KMID : 0377619720220010041
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Korean Jungang Medical Journal
1972 Volume.22 No. 1 p.41 ~ p.47
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TYPICAL BUT SYMPTOMLESS CROUZON’S DISEASE AND SPINA BIFIDA FOUNDED IN THE ADULT
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Lee, Seung-Jae
Lee, Bae-Choon/Lee, Jung-Kak/Kim, Won-Bae
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Abstract
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Crouzon´s disease (craniofacial dysostosis) and spina bifida (Rachischsis), which are varieties of developmental defects, has been interested to neurosurgeon, pediatrician and also the former to ophthalmologists because of it was that usually appearing various neurologic manifestations with their peculiar characteristic features.
The vast majority of cases have been known as embryogenic origin formed in early uteriene life or just post-natal period, and why the reason of the unbalance of the growth and development between the defected and anomalous coverings and its underneath brain and spinal cord, moreover, these developmental anomalies are founded in infancy and early child-hood most of all.
The degree, severity and intensities of these disease are variable as minor to serious, and the serious case has been considered usually resulted in death in utero, the minors may be accompanied by no symptoms and the moderate cases are usually accompanies by a degree of disability".
The cause of these disease is not clear here yet, but only there are many postulated theories in reviewing the literatures, existing several underlying factors as maternal anoxia, ionizing radiations, certain drugs, certain infections and metabolic disease during pregnancy1,4)
In the case of Crouzon´s disease; 1) Familiar and hereditary theory 1,2,3,5,,6,9,10,12,13) 2) The theory of defect in the portion of the blastmal mesenchymes destined to form the sutures (defect in the germ plasm)1,4,5), and 3) The theories of the intrauterine meningitis (especially inflammation of the membranes of the brain from that since the bones of the calvarium are membranous in origin) and rickets, syphilis, pituitary dysfunction and birth traumas,5,8) are the postulated etiologic theories, and the middle one is the most generally accepted" 4,5,8).
The case of spina bifida is also a same etiologic base as above.
The incidence of these disease is not obviouse even lots of reported case in the literatures.
And then, by Anderson et a18), the oxycephalic variety was 8% among the ratio of l/64a craniosynostosis in all the hospitalized patient and 72 to 28% between male and female cases
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