正中唇裂，口蓋裂および脳梁欠損症を合併した頭蓋底髄膜瘤（sphenoethmoidal type）の2症例

SPHENOETHMOIDAL MENINGOENCEPHALOCELE ASSOCIATED WITH AGENESIS OF CORPUS CALLOSUM AND MEDIAN CLEFT LIP AND PALATE : REPORT OF TWO CASES

I．はじめに

　頭蓋髄膜瘤meningoencephaloceleは脊椎髄膜瘤me—ningomyeloceleに比べて少なく，中でも頭蓋底髄膜瘤basal meningoencephaloceleは稀で，報告例も少ない。

　我々はすでに正中唇裂，口蓋裂および脳梁欠損症を合併した頭蓋底髄膜瘤（sphenoethmoidal type）の1例を報告し^15），これらの異常が一単位の先天異常である可能性を示唆したが，今回同一異常を呈する症例を追加経験したので，その一単位の先天異常である可能性と診断，治療について検討，報告する。

Two cases of sphenoethmoidal meningoencephalo-cele are reported. Patients are a 1 year 7 month old boy and a 3 year old boy. Although spheno-ethmoidal meningoencephalocele is reasonably classified as a type of basal meningoencephalocele, the authors could not find out any reports on cases designated as such. The reason must be in difficulty to differentiate the sphenoethmoidal type from the transsphenoidal type. Authors could differentiate them using CT scan. Two other cases were found in cases reported as transsphenoidal type. Further, it is assumed that these four cases including our two cases were accompanied with agenesis of corpus callosum and median cleft lip and palate. Despite the fact that the respective anomalies are rare, they are completely the same, which signifies the possibility that 1) sphenoethmoidal meningo-encephalocele, 2) agenesis of corpus callosum and 3) median cleft lip and palate are a single unit of congenital anomalies.It probably should be considered that the cause for these malformations were present even before the formation of the lips which takes place earliest, that is, sixth week of gestation.