Recovery From Ophthalmoplegia and Proptosis After Repair of Bilateral Carotid-Cavernous Sinus Fistulas

Abstract

A carotid-cavernous sinus fistula is an anomalous communication of dural branches from the intracerebral artery or external carotid artery to the cavernous sinus. Spontaneous carotidcavernous sinus fistulas have been associated with the presence of ruptured carotid aneurysms, atherosclerosis, diabetes mellitus, and genetic disorders such as Ehlers-Danlos syndrome or fibromuscular dysplasia. However, bilateral spontaneous carotidcavernous sinus fistulas are still considered uncommon.1,2 A 36-year-old man presented with 5 months of progressive headaches, diplopia, and progressive blurry vision. He had no history of head trauma, and both the headaches and the blurry vision were exacerbated by lying down in the supine position. Examination revealed significant proptosis, conjunctival injection, and abducens nerve palsies bilaterally (Figure, A and B). His visual field examination was within normal limits; however, his visual acuity was impaired, and tonometry revealed markedly elevated intraocular pressure (37 mm Hg in the left eye and 34 mm Hg in the right; reference range, 10-21 mm Hg). No retro-orbital bruit or upper motor neuron signs were noted. Magnetic resonance imaging of the brain showed an anterior displacement of the pituitary gland by a vascular structure, but no parenchymal changes were seen. An abnormal vascular structure was redemonstrated by magnetic resonance angiography, but the diagnosis of a dural fistula could not be confirmed. Conventional digital subtraction angiography revealed an indirect low-flow dural shunt between meningeal branches of both the internal carotid artery and the external carotid artery with the cavernous sinus bilaterally (Barrow type D; Figure, C). Endovascular embolization of the lesion (Figure, C and D) was performed using a combined anterior and posterior transvenous approach, which led to the immediate lowering of intraocular pressure and an improvement in visual acuity. At 1-month follow-up, the patient had a near complete resolution of his bilateral cranial nerve VI palsies (Figure, E and F; Video).