Parietal encephaloceleを伴ったHoloprosencephalyの1例

A Case of Holoprosencephaly with Parietal Encephalocele

I．はじめに

　胎生期3-5週という初期における中枢神経系の障害により，prosencephalonが分化，発達せず，左右大脳半球分離不全，olfactory bulb, olfactory tractの欠如などを生じ，しばしば顔面正中の奇形を合併するholo—prosencephalyは中枢神経系先天性異常のうちでも比較的稀で，重篤な疾患の1つである^2,8）．本疾患は，1882年にKundrat^6）がarhinencephalyと命名後，1963年にDeMyer and Zeman^3）がdiencephalonをも含めたpro—sencephalonの分化，発達の障害に注目しholopro—sencephalyとして発表し．以後この名称が一般的に使用されている．今回，われわれは，のholopro—sencephalyにencephaloceleを合併し，さらに興味深い脳室形態を示した1例を経験したので，その幾つかの問題点をふまえて報告する．

Holoprosencephaly, as described by DeMyer, is a cerebral malformation in which the embryonic pro-sencephalon fails to divide into cerebral hemispheres. According to the degree of division in the prosencepha-Ion, he classified holoprosencephaly into three types ; alobar, semilobar and lobar type. We have experi-enced a case of holoprosencephaly with parietal encephalocele. The patient was admitted to our hos-pital 4 hours after birth because of parietal encepha-locele. A radical operation was carried out and en-cephalocele was diagnosed histologically.