両側性朝顔症候群にもやもや病を合併した1例

Moyamoya disease in a case of bilateral morning glory syndrome

要約　目的：両眼に朝顔症候群がある症例にもやもや病が併発した報告。症例：2歳10か月の男児が眼位異常で近医を訪れ，両眼の乳頭異常を発見されて紹介され受診した。所見と経過：両眼に朝顔症候群の乳頭異常があった。磁気共鳴画像検査（MRI）で右視神経低形成，完全脳梁欠損があり，加えてもやもや病が疑われた。磁気共鳴血管造影（MRA）で両側の内頸動脈狭窄，中大脳動脈の狭窄，基底核領域のもやもや血管があり，両側もやもや病と診断された。左右それぞれに血行再建術が行われた。6歳8か月の現在，新しい脳梗塞はなく，矯正視力は右0.9，左0.3である。結論：朝顔症候群では，脳虚血発作がなくても，もやもや病が併発している可能性がある。

Abstract.　Purpose：To report a child of morning glory syndrome in both eyes associated with moyamoya disease. Case：A male child showed abnormal eye position at the age of 2 years 10 months. He was detected to have abnormal optic disc in both eyes and was referred to us. Findings：Both eyes showed morning glory syndrome. Magnetic resonance imaging（MRI）showed hypoplasia of right optic nerve,total agenesis of corpus callosum,and findings suggestive of moyamoya disease. Magnetic resonance angiography（MRA）showed narrowing of bilateral internal carotid artery,attenuation of middle cerebral artery,and blurring of vessels characteristic for moyamoya disease. He later received vascular reconstruction surgery. He has been doing well until present at the age of 6 years 8 months with corrected visual acuity of 0.9 right and 0.3 left. Conclusion：This case illustrates that morning glory syndrome may be associated with moyamoya disease.