Elderly woman presents with a red eye and blurry vision

Issue: April 25, 2012

ByJeffrey L. Marx, MD

ByMichelle C. Liang, MD

A 71-year-old woman was referred to our clinic for a 5-day history of redness and blurry vision in her right eye. She also complained of photophobia, but she denied any significant ocular pain.

The patient had been previously diagnosed by an outside provider with Fuchs’ iridocyclitis for which she was started on Combigan (brimonidine tartrate 0.2%, timolol maleate 0.5%, Allergan) and Pred Forte (prednisolone acetate ophthalmic suspension 1%, Allergan). Because her symptoms did not improve on this treatment, she was referred to our clinic for further management.

The patient had a medical history of hypertension and diabetes, but no significant ocular history.

The patient’s best corrected visual acuity was 20/400 in the right eye (6 months prior it had been 20/40) and 20/20 in the left eye. She was noted to have an afferent pupillary defect in the right eye, as well as a slightly elevated IOP of 25 mm Hg. IOP of the left eye was within normal limits. Extraocular movements and confrontation visual fields were full in both eyes. Slit lamp biomicroscopy of the right eye showed trace conjunctival injection, mild corneal edema with Descemet membrane folds, 1+ cell with flare, 360° of iris neovascularization and a mild nuclear sclerotic cataract. The left eye appeared essentially normal, with the exception of a mild nuclear sclerotic cataract.

Funduscopic examination of the right eye revealed mid-peripheral dot-blot hemorrhages, as well as retinal arteriolar attenuation and dilated but non-tortuous veins (Figure 1). A fluorescein angiogram was performed, which showed delayed choroidal and retinal filling, patchy choroidal filling, and delayed arteriovenous transit time in the right eye (Figure 2). The left eye fluorescein angiogram was within normal limits.

Figure 1. Dilated fundus photography. The right eye was noted to have retinal arteriolar attenuation, mildly dilated but non-tortuous retinal veins and mid-peripheral dot-blot hemorrhages. The left eye appeared within normal limits.

Images: Liang MC, Marx JL

Figure 2. Fluorescein angiography of the right eye. Delayed choroidal and retinal filling are present, as well as patchy choroidal filling with delayed arteriovenous transit time.

What is your diagnosis?

Iris neovascularization

Prior to dilation, the most notable feature of our patient’s examination was the presence of iris neovascularization in her right eye. Given our patient’s clinical history and presentation, our differential diagnosis prior to dilation included the possibility of an ocular disorder resulting in retinal ischemia, such as diabetic retinopathy, central retinal artery or vein occlusion, or ocular ischemic syndrome. Other conditions to consider in a patient with iris neovascularization would include inflammatory, infectious or neoplastic causes such as uveal melanoma. Based on the findings of her dilated fundus examination and fluorescein angiogram, however, our patient was tentatively diagnosed with ocular ischemic syndrome.

Discussion

On clinical examination, patients with ocular ischemic syndrome (OIS) demonstrate signs of chronic ocular hypoperfusion, usually from severe carotid artery stenosis. The typical patient is close to a mean age of 65 years with a slight male predominance (2:1), and a significant history of atherosclerotic disease can be elicited. Because carotid artery stenosis is the most common cause of OIS, patients present with unilateral involvement in 80% of cases. Symptoms include decreased vision (90% of cases), as well as a dull ache (40% of cases) due to either global ocular ischemia or elevated IOP. OIS can affect both the anterior and posterior segments; therefore, findings may include corneal edema, neovascularization of the iris, anterior chamber cell and flare, cataract formation, retinal arteriolar narrowing, venous dilation and beading without tortuosity, mid-peripheral dot-blot hemorrhages, a cherry-red spot, cotton wool spots, and even choroidal neovascularization.

Diagnostic testing by fluorescein angiography can demonstrate delayed choroidal and retinal filling, patchy choroidal filling and an increased arteriovenous transit time. Macular edema and retinal capillary non-perfusion may also be present. If ERG is performed, both the a- and b-waves will be dampened. Further studies, such as a CTA, MRA or carotid duplex ultrasound, are essential for ruling out a carotid artery stenosis that may require surgical intervention. Duplex ultrasound may also show decreased flow velocity in the central retinal artery and reversal of flow in the ophthalmic artery.

Management of OIS includes treatment of ocular symptoms as well as carotid endarterectomy if imaging shows 70% to 99% carotid stenosis. If total carotid obstruction is present, then carotid endarterectomy is not thought to be beneficial. Ocular treatments may include use of intravitreal Avastin (bevacizumab, Genentech) and/or panretinal photocoagulation to treat neovascularization, as well as IOP-lowering medications to treat any secondary glaucoma. Even with prompt diagnosis and treatment, however, OIS carries a poor visual prognosis.

Follow-up

Our patient underwent a complete systemic work-up, including blood work for CBC, BMP, hemoglobin A1c, LFTs, cholesterol, CRP, B12, folate, ACE and anticardiolipin antibody. All lab tests were found to be unremarkable. A carotid duplex ultrasound was performed, which showed total or near total occlusion of both the right common and internal carotid arteries (Figure 3). Interestingly, reversal of flow was also seen in the right ophthalmic artery. The left carotid artery was found to have no significant stenosis. A follow-up CTA was performed, which showed total occlusion of the right carotid artery, and therefore, no surgical intervention was deemed to be beneficial.

Figure 3. Carotid duplex ultrasound. Total or near total occlusion of the right common carotid artery is present. No significant disease is seen in the left common carotid artery.

The patient was treated with a series of intravitreal bevacizumab injections, panretinal photocoagulation and topical IOP-lowering drops. At a 10-month follow-up visit, her IOP was successfully controlled, but her vision had decreased to hand motion in the right eye.

References:

Amselem L, Montero J, Diaz-Llopis M, et al. Intravitreal bevacizumab (Avastin) injection in ocular ischemic syndrome. Am J Ophthalmol. 2007;144(1):122-124.
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Klijn CJ, Kappelle LJ, van Schooneveld MJ, et al. Venous stasis retinopathy in symptomatic carotid artery occlusion: prevalence, cause, and outcome. Stroke. 2002;33(3):695-701.
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North American Symptomatic Carotid Endarterectomy Trial Collaborators. Beneficial effect of carotid endarterectomy in symptomatic patients with high-grade carotid stenosis. N Engl J Med. 1991;325(7):445-453.

For more information:

Michelle C. Liang, MD, and Jeffrey L. Marx, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.
Edited by Catherine A. Cox, MD, and Jordana F. Goren MD, MS. Drs. Cox and Goren can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.