Ethmoidal-ophthalmic Arteriovenous Fistula Presenting with Peri-orbital Edema
Objective:
Promote early identification and possible intervention in rare causes of peri-orbital edema such as ethmoidal-orbital arteriovenous fistulas and thus, prevent grievous complications.
Background:
Dural arteriovenous fistulas (DAVFs) account for 10%–15% of intracranial arteriovenous malformations. Ethmoidal-ophthalmic fistulas are rare entities with an incidence of 5% presenting with ocular symptoms, headache, cranial nerve deficits etc. The most common site of drainage is into
superior sagittal sinus through intracranial cortical veins. CT brain with
angiography is an initial diagnostic method however
DSA remains the gold standard of visualization of DAVFs. Symptomatic DAVFs are managed endovascularly by permanent
embolization, coiling or surgical clipping.
Design/Methods:
51-year-old male with past medical history of diabetes, Hepatits B (on Tenofovir), was referred to the ED for right eye periorbital edema for 1 week with no improvement with Augmentin for presumed orbital cellulitis. Periorbital swelling was painful, progressive with impaired visual acuity (Right 20/40 and Left 20/25), pain with extra ocular movement and global diffuse headache. Intraocular pressure in right eye was 10, 12 in left eye. Not associated with trauma, fever, focal neurological deficits.
CT head, MRI brain and orbits, along with MRA head wit gadolinium revealed changes concerning for dural AV fistula.
A cerebral angiogram confirmed bilateral ethmoidal-ophthalmic artery arteriovenous malformation draining into retro-orbital cerebral vein with reflux into the sylvian vein and the cavernous sinus.
Results:
This was managed by balloon test occlusion of right ophthalmic artery and partial embolization of right ophthalmic AVM. The patient then underwent a 2nd stage embolization in 2 weeks followed by a 3rd stage 1 month later.
Successful microballoon occlusion of the right ophthalmic artery vascular supply was confirmed by cerebral angiography and intraoperative visual evoked potentials.
Conclusions:
Ethmoidal-ophthalmic fistulas may present with ophthalmic symptoms like peri-orbital edema that are prone to rupture resulting in intracranial
hemorrhage and should be approached with caution.