Pathological Study on Anencephaly(Autopsy Cases)

Abstract

Anencephaly is the most common and the most serious congenital malformation which occurs spontaneously in man and other mammals and which is compatible with completion with the pregnancy but incompatible with life (Anderson et al., 1967). In this common maldevelopment, the brain is either absent or rudimentary and there is an associated defect of the cranial vault and scalp (Urich. 1976). Frequently anencephaly is combined with other malformations such as anterior lobe of pituitary body unaccompanied by a posterior lobe and marked adrenal hypoplasia, while such features as harelip, cleft palate. spina bifida, hyperplasia of the thymus, hydronephrosis, hydroureter and abnormalities of major visceral arteries are present also in some cases (Vogel and McClenahan, 1952). The frequency of the disease varies between approximately 0.5 and 2.0 per thousand births in the United States and most of Europe; it is locally higher in Britain and lower in Africa, Asia, and South America (Friede, 1976). In our country, many cases of anencephalic fetuses have been reported, too. The frequency of the anencephaly in our country is approximately 1.5 per thousand births (이명복 등, 1969). And another report show that the CNS malformations comprise 17.3 percent of all abnormal births and anencephaly is 7 out of 53 cases of the CNS malformation(이제구, 1970). Although anencephaly have been reported for centuries, the underlying cause is still uncertain. In past years, several aspects of this anomaly have been studied, e.g., etiologic factors, and familial and genetic prevalence, complications, gross embryologic development, endocrine development and their interreIationships. Recently we have experienced 10 cases of anencephaly during a period of 4 years, from 1977 to 1980. The current study is made to report 10 cases of anencephaly generally and to discuss the pathogenesis of anencephaly.