脊髄分裂症Diastematomyeliaについて

ON DIASTEMATOMYELIA

I．緒言

　Diastematomyeliaは脊髄またはその馬尾が2分しているかあるいは大きな裂溝を有する脊髄奇形の1種であり，多くの場合脊椎の種々な奇形が共存する。この脊髄または馬尾の左右2分裂は椎体後面より脊椎管前壁に発生する骨性，軟骨性また時に靱帯性の突起が脊髄または馬尾の中央を穿くために生ずると考えられている。一般に脊髄膜も左冶に分裂されて，半分の脊髄が、各々の脊髄膜管中に存在する。本疾患では脊髄は左右2分され，各脊髄膜管中には脊髄の左右半分が存在するが，diplomy—eliaでは脊髄が重複し，各管中に完全な脊髄が存在する。

　Diastematomyeliaは，ギリシヤ語系ラテン語よりの合成語でdia＝apart,histemi＝standよりのdiastem＝in—tervalとmyelo＝marrowより成つている。

Diatematomyelia is a type of split notochord syn-drome and in the past this was thought to be and also described as a disease existing only in pathol-ogy. However more recently diastematomyelia has been attracting more clinical interest and it has been shown to be both improvable and even curable when any clinical symptoms which refer to this anomaly in the course of physical growth of a patient are treated surgically following proper preoperative early diagnosis. Diatematomyelia causes slowly deteriorat-ing disturbances of gait and urination in childhood and its diagnosis may sometimes be difficult because of its slow clinical course which resembles cauda equina tumor, poliomyelitis, neuromuscular diseases, etc.

In this paper a historical review of important past publications on this subject is geven and three cases are also described which were preoperatively diag-nosed and then operated on by author with satis-factory results. Interesting family histories of the patients were observed showing that the mothers of all three patients experienced spontaneous abortions and it was concluded that the higher the number of abortions the earlier the patient showed symp-toms.

Embryological and pathological observations on its aetiology, diagnostic problems including radiology, operation and results are discussed. It is emphasized that early diagnosis based on association with other concomitant mesenchymal disraphic disorders or very early symptoms and early operative treatment before serious symptoms occur are important in order to achieve better therapeutic results.