Summary
Fronto-ethmoidal encephalomeningoceles are rare in europoid populations, but seem to be common in Thailand. In a population survey in a limited area in northern Thailand an incidence of 9 cases in a total population of 31,582, i.e. approximately 1:3,500 was found. Studies in 14 families with an affected member indicate that there is no simple Mendelian inheritance, but a contribution of genetic factors to the etiology of this type of fronto-ethmoidal encephalomeningocele is suggested by the occurrence of the defect in 4 close relatives of affected subjects.
Zusammenfassung
Fronto-ethmoidale Encephalomeningocelen sind in europiden Populationen selten, scheinen jedoch in Thailand häufig zu sein. Bei einer Bevölkerungserhebung in einem begrenzten Bereich in Nord-Thailand fanden sich 9 Fälle in einer Population von 31582, d.h. ca. 1:3500. Untersuchungen in 14 Familien mit einem befallenen Probanden zeigten, daß kein einfacher Mendelscher Erbgang vorliegt; jedoch wird eine Beteiligung einer genetischen Disposition für diesen Typ fronto-ethmoidaler Encephalomeningocelen dadurch nahegelegt, daß die gleiche Anomalie bei 4 nahen Verwandten von Probanden gefunden wurde.
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References
Conway, H.: Effect of supplemental vitamin therapy on the limitation of incidence of cleft lip and cleft palate in humans Plast. reconstr. Surg. 22, 450 (1958).
Davis, W. B.: Hare lip and cleft palate. A study of fourhundred and twenty five consecutive cases. Ann. Surg. 87, 536 (1928).
Douglas, B.: The role of environmental factors in the etiology of ‘so-called’ congenital malformations. Plast. reconstr. Surg. 22, 94 (1958).
Evans, H. M., Nelson, M. M., Asling, C. W.: Multiple congenital abnormalities resulting from acute folic acid deficiency during gestation. Science 114, 479 (1951).
Flatz, G.: Hemoglobin E: Distribution and population dynamics. Humangenetik 3, 189 (1967).
Fogh-Andersen, P.: Inheritance of hare lip and cleft palate. Copenhagen: A. Busick 1942.
Mealy, J., Dzenitis, A. J., Hockey, A. A.: The prognosis of encephaloceles. J. Neurosurg. 32, 209 (1970).
Mohr, U., Schlegel, D., Witt, M., Flatz, G.: Überraschende Befunde bei der Suche nach Burkitt-Tumoren in Thailand. Aktuelle Med. (Beil. z. Münch. med. Wschr.) Nr. 45 (1969).
Nutrition Survey, The Kingdom of Thailand. A report by the interdepartmental committee on Nutrition for national defence. February 1962.
Peer, L. A., Bryan, W. H., Strean, L. P., Walker, J. C., Bernhardt, W. G., Peek, G. C.: Induction of cleft palate in mice by cortisone and its reduction by vitamins. J. int. Coll. Surg. 30, 249 (1958a).
—, Strean, L. P., Walker, J. C., Bernhardt, W. G., Peek, G. C.: Study of 400 pregnancies with birth of cleft lip-palate infants. Plast. reconstr. Surg. 22, 442 (1958b).
Polman, A.: Anencephaly, spina bifida, and hydrocephaly. Genetica 25, 29 (1950).
Schwidde, J. T.: Spina bifida. Amer. J. Dis. Child. 84, 35 (1952).
Suwanwela, Ch., Hongsaprabhas, Ch.: Fronto-ethmoidal encephalomeningocele. J. Neurosurg. 25, 172 (1966).
Vogel, F.: Lehrbuch der allgemeinen Humangenetik. Berlin-Göttingen-Heidelberg: Springer 1961.
Vondra, J.: Die bezeichneten und versteckten Spaltungen der Lippe, des Gaumens und des Oberkiefers. Schweiz. Mschr. Zahnheilk. 67, 874 (1957).
Warkany, J.: Congenital malformations induced by maternal dietary deficiency. New York: Academic Press 1954.
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On leave from Universitäts-Kinderklinik Bonn.
Established and supported by Stiftung Volkswagenwerk, Hannover.
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Flatz, G., Sukthomya, C. Fronto-ethmoidal encephalomeningoceles in the population of northern Thailand. Hum Genet 11, 1–8 (1970). https://doi.org/10.1007/BF00296297
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DOI: https://doi.org/10.1007/BF00296297