奇異な動きを伴う視神経乳頭欠損症の1例

Contractile Coloboma of the Optic Nerve Entrance. Report of a Case

緒言

　視神経乳頭の先天異常のなかで，検眼鏡的にみていわゆる定型的欠損症とは異なつた型の存在は，古くから指摘されているが，近年KindlerがMorning Glory Syndromeと名付ける10例を報告して以来，該部の同様な異常例の報告が相つぎ，それぞれ種々の解釈がなされている。

　最近われわれは，該部附近に奇異なる運動を認め定型的欠損症とは異なつた型の視神経乳頭部異常を有する珍しい症例を経験したので報告する．

This paper describes a case of coloboma of the optic nerve entrance which manifested in-termittent contraction and elevation at its cir-cumference resembling a swimming jellyfish. The patient, a 6-year-old boy, showed smaller cornea, abnormal iris, posterior polar cataract, peripheral retinal degeneration and loss of macular reflex, together with en enlarged optic disc similar to the typical instance of morning glory syndrome. In the present case, there was no white dot characte ristic of morning glory syndrome in the right eye. Findings in the fellow left eye were practically normal. Through close and continuous ophthalmoscopy, the margin of the coloboma manifested contra-ctile movement lasting for about three seconds. The contractile movement set in abont at the rate of 3 times a minute.

Contraction of unusually developed peripapil-lary ciliary muscle was considered to be the somt likely explanation for the movement no-ted in this patient. To our present knowledge, no other instance with an unusual movement of coloboma of the optic nerve entrance has been reported before.