Severe papilledema and multiple hypercoagulability abnormalities in patient with dural arterio-venous fistulas.

Abstract

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.