硬脑膜动静脉瘘患者严重乳头水肿及多重高凝异常。

Chi-Wei Tien, Patrick K. Nicholson, E. Margolin
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摘要

一位54岁的男性在过去的六个月里发现了右侧脉动性耳鸣,最近开始经历站立时的短暂性视觉障碍。MRI显示两个独立的硬脑膜动静脉瘘(bdAVF)在矢状窦和右乙状窦硬脑膜。神经眼科检查显示严重的双侧视盘水肿,视力保留,但视野检查显示早期神经纤维束缺损。高凝血谱检测显示d -二聚体升高,蛋白S水平显著降低,同型半胱氨酸水平升高。本病例强调了所有bdavf患者进行神经眼科会诊的重要性,因为静脉高压可引起颅内压升高和由此引起的乳头水肿。直到视力丧失不可逆转的晚期,乳头水肿才会影响中枢视力,因此筛查其存在和周围视力丧失的预处理程度至关重要。这也表明,所有bdAVF患者都应该开始进行高凝检查,以防止未来的血栓栓塞事件。
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Severe papilledema and multiple hypercoagulability abnormalities in patient with dural arterio-venous fistulas.
A 54-year-old man noticed right-sided pulsatile tinnitus for the past six months and recently started experiencing transient visual obscurations when standing up. MRI demonstrated two separate brain dural arteriovenous fistulas (bdAVF) in sagittal and right sigmoid dural sinuses. Neuro-ophthalmological exam demonstrated severe bilateral optic disc edema with preserved visual acuity but early nerve fiber bundle defects on visual field testing. Hypercoagulable profile testing revealed very elevated D-dimer, significantly decreased protein S level and elevated homocysteine levels. This case highlights importance of referring all patients with bdAVFs for neuro-ophthalmological consultation as venous hypertension can cause increased intracranial pressure and resultant papilledema. Papilledema does not affect central vision until late stages when visual loss is irreversible thus screening for its presence and pre-treatment extent of peripheral visual loss is paramount. It also demonstrates that hypercoagulable workup should be initiated in all patients with bdAVF in order to prevent future thromboembolic events.
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