Proptosis, Diplopia, and an Ocular Bruit in a Patient With Ehlers-Danlos Syndrome

Abstract

We describe a case of a 30-year-old female with a history of the Vascular form of Ehlers-Danlos Syndrome who presented to a community Emergency Department with complaints of eye pain, blurred vision, and diplopia. On examination, she was noted to have proptosis, scleral injection and an ocular bruit. Imaging studies demonstrated dissection of the carotid artery and a carotid cavernous sinus fistula. Patient underwent endovascular coil embolization of the lesion with resolution of symptoms.

Ehlers-Danlos syndromes and their manifestations in the visual system

“…Characteristic facial features in vEDS include micrognathia, prominent eyes, narrow nose, thin lip vermilion. This apparent proptosis may be accompanied by various ocular signs including lagophthalmos with consequent exposure keratopathy, conjunctival hyperemia, telangiectasias along the eyelid margin, and chemosis ( 29 , 30 ).…”