Moyamoya病相关眼部临床特点分析

Clinical feature of ocular presentations in Moyamoya disease

目的:探讨与Moyamoya病相关眼部临床特点和发生机制。方法:回顾性分析74例Moyamoya病患者的临床资料和眼部症状。结果:74例Moyamoya病患者中眼部症状与其它症状同时发生者23例(31%),其中仅以眼部症状伴头痛表现为首发症状就诊者3例(4%);DSA检查显示单侧或双侧颈内动脉狭窄者24例(32%),其中3例患者双侧颈内动脉完全闭塞,而无任何眼部症状;23例患者所伴眼症表现(含合并有多种表现患者)主要为一过性黑朦13%,偏盲17%,视力下降甚至丧失44%,瞳孔大小异常13%,瞳孔直接/间接对光反射消失13%,上睑下垂9%,眼球震颤4%,眼球运动障碍13%,眼眶压痛9%。结论:眼科医生应掌握Moyamoya病的发病特点及临床表现,特别是对于有眼部症状的Moyamoya病患者应该做到早期诊断,早期治疗,预防严重的视力下降。

AIM： To probe the clinic characteristics and mechanisms of ocular onset features in patients with Moyamoya disease. METHODS： A retrospective analysis was performed on 74 patients who had Moyamoya disease, and the general clinic data and ocular findings were reviewed. RESULTS： Of the 74 patients, 23 （31%） had ocular manifestations accompanied by other symptoms, including 3 （4.1%） with ocular syndrome accompanied with headache as the first symptoms of Moyamoya disease. Digital subtract angiography （DSA） examination revealed 24 patients （32.4%） had unilateral or double stenosis of internal carotid artery, among which 3 patients had ocular syndrome. All positive eye findings induded amaurosis fugax （13%）, hemianopia （17%）, decline or loss of the visual ability and lesions of visual fields （44%）, papillary size dysfunction （13%）, pupil direct/indirect light reflex （13%）, ptosis （9%）, nystagmus （4%）, ocular dyscinesia （13%）, and fossa orbitalis tenderness （9%）. CONCLUSION： Ophthalmologists should master the onset characteristics and clinical manifestation of Moyamoya disease. Early diagnosis and treatment is very important for prevention the vision deterioration, especially to patients with the ocular syndrome as the initial onset syndrome.