Abstract
BackgroundEncephalomeningocele, especially in the frontoethmoidal region, is a form of neural tube defect which affects patients in Southeast Asia more commonly than in Western countries. Its underlying cause is not known but teratogenic environmental agents are suspected. However, nutritional deficiency, as in spina bifida, cannot be excluded.MethodsThis study reports 21 cases of meningocele (without brain tissue in the lesion) and encephalomeningocele (with brain tissue) that were admitted to our hospital for surgical corrections in the period of ten years, from 1990 to 1999. Clinicopathological findings, as well as occupations of family members and prenatal exposures to infectious agents or chemicals were reviewed and analyzed.ResultsThe most commonly involved area was the frontoethmoidal region, found in 20 cases. The combined pattern between nasoethmoidal and nasoorbital defects was found most frequently (11 from 21 cases) and had more associated abnormalities. Encephalomeningocele had more related abnormalities than meningocele with proportions of 0.6 and 0.3, respectively.ConclusionsHere, we confirmed that genetic defects are not likely to be the single primary cause of this malformation. However, we could not draw any conclusions on etiologic agents. We suggest that case control studies and further investigation on the role of nutritional deficiencies, especially folic acid, in the pathogenesis of encephalomeningocele are necessary to clarify the underlying mechanisms.
Highlights
Encephalomeningocele, especially in the frontoethmoidal region, is a form of neural tube defect which affects patients in Southeast Asia more commonly than in Western countries
Encephalomeningocele is a congenital malformation characterized by protrusion of meninges and/or brain tissue due to a skull defect
The frontoethmoidal type which its defect locates in the area of frontal and ethmoidal bones, is exclusively common in Southeast Asia, including Thailand
Summary
Encephalomeningocele, especially in the frontoethmoidal region, is a form of neural tube defect which affects patients in Southeast Asia more commonly than in Western countries. Its underlying cause is not known but teratogenic environmental agents are suspected. Encephalomeningocele is a congenital malformation characterized by protrusion of meninges and/or brain tissue due to a skull defect. It is one form of neural tube defects as the other two, anencephaly and spina bifida. The frontoethmoidal type which its defect locates in the area of frontal and ethmoidal bones, is exclusively common in Southeast Asia, including Thailand. Despite of the higher incidence of this congenital defect in this area, little is known about its etiology and pathogenesis. Only aflatoxin has been proposed to be a teratogenic agent for (page number not for citation purposes)
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