This article is more than 5 years old. Information may no longer be current.

Man presents with unilateral decreased vision and eye pain

The right eye featured corneal edema, mild anterior chamber cellular reaction, neovascularization of the iris and angle, and multiple cotton wool spots.

Issue: December 25, 2014

ByErica Liu, MD

ByCaroline R. Baumal, MD

ByDavid Salz, MD

Add topic to email alerts

Receive an email when new articles are posted on

Please provide your email address to receive an email when new articles are posted on .

Subscribe

Added to email alerts

You've successfully added to your alerts. You will receive an email when new content is published.

Click Here to Manage Email Alerts

You've successfully added to your alerts. You will receive an email when new content is published.

Click Here to Manage Email Alerts

Back to Healio

We were unable to process your request. Please try again later. If you continue to have this issue please contact customerservice@slackinc.com.

Back to Healio

A 67-year-old man presented with complaints of persistent, dull ocular pain, redness, photophobia, tearing and decreased vision in his right eye for 1 week. He reported the symptoms began after mild blunt trauma from his granddaughter’s toy.

Ocular history included controlled mixed-mechanism glaucoma previously treated with laser iridotomy. He was a 25-year insulin-dependent diabetic without any retinopathy. Medical history included severe coronary artery disease previously treated with cardiac bypass and restrictive lung disease. He was a former smoker. Ocular medications included dorzolamide-timolol in both eyes. Systemic medications included insulin, simvastatin, aspirin, hydrochlorothiazide, lisinopril and metoprolol.

Examination

At presentation, the patient’s best corrected visual acuity was counting fingers at 3 feet in the right eye and 20/25 in the left eye. The right pupil was slightly irregular but reactive to light with a trace relative afferent pupillary defect. The left pupil was round and reactive to light (Figure 1). IOP was 36 mm Hg in the right eye and 15 mm Hg in the left eye. Extraocular motility was normal. Right anterior segment examination demonstrated corneal edema, mild anterior chamber cellular reaction, neovascularization of the iris (NVI) and neovascularization of the angle (NVA). Dilated fundus examination showed multiple cotton wool spots (Figure 2). Fundus fluorescein angiography of the right eye demonstrated delayed vascular filling, areas of non-perfusion and late staining of the vessels (Figure 3). Of note, 2 months before this presentation, BCVA was 20/30 in the right eye, and there were no cotton wool spots or diabetic retinopathy seen on routine examination.

Figure 1. Color external photos after pharmacologic dilation demonstrate a poorly dilating pupil in the right eye with diffuse conjunctival injection and iris neovascularization. The left pupil dilates normally.

Images: Liu E, Salz D and Baumal C

Figure 2. Color fundus photograph showing cotton wool spots along the temporal arcades and macula in the right eye. The left eye is normal.

Figure 3. Fundus fluorescein angiogram of the right eye shows delayed vascular filling at approximately 1 minute of fluorescein transit. A late phase fluorescein angiogram at approximately 6 minutes shows staining of retinal vessels and patchy areas of poor perfusion. The foveal avascular zone appears enlarged. Areas of fluorescein blockage along the superior and inferior arcades correspond to cotton wool spots. The image quality is reduced due to corneal edema.

What is your diagnosis?

Unilateral loss of vision

The differential diagnosis for this individual with unilateral loss of vision, retinal ischemia and neovascular glaucoma includes vascular, neoplastic and inflammatory causes.

Differential diagnosis

Vascular causes include central retinal vein occlusion (CRVO), central retinal artery occlusion (CRAO), diabetic retinopathy and ocular ischemic syndrome (OIS). CRVO typically has both tortuous and dilated retinal veins as well as retinal hemorrhages in all four retinal quadrants. The onset of neovascularization of the iris secondary to CRVO classically occurs 3 months after the primary event. CRAO presents with whitening of the inner retina, and patients are at risk for developing neovascularization. Both CRVO and CRAO would be expected to present with acute loss of vision at least several weeks before the development of neovascular glaucoma, making these diagnoses less likely in our patient. Diabetic retinopathy can cause cotton wool spots and dot-blot retinal hemorrhages, but findings tend to be bilaterally symmetric. OIS typically presents unilaterally or asymmetrically with retinal veins that are dilated but not tortuous, dot-blot retinal hemorrhages that are often equatorial and cotton wool spots. Fluorescein angiography may demonstrate delayed transit time, delayed vascular filling, staining of the vessels and occasionally capillary dropout in the midperiphery. The most specific sign of OIS is a leading edge of the dye in the retinal arteries, and the most sensitive finding is prolonged arteriovenous transit time.

Neoplastic syndromes such as multiple myeloma or lymphoma may contribute to hyperviscosity syndrome, predisposing to retinal vascular abnormalities. Takayasu’s arteritis, also known as aortic arch syndrome, is due to idiopathic inflammation resulting in progressive obliteration of vessels arising from the aortic arch. It can present with bilateral retinal arterial narrowing, neovascularization of the disc and retina, and peripheral microaneurysms. Takayasu’s is most commonly found in patients of Asian descent and female gender with decreased or absent upper extremity pulses. Additional inflammatory entities such as giant cell arteritis and Wegener’s can be excluded by history, age and serologic studies.

Diagnosis and management

The patient’s neovascular glaucoma was treated with brimonidine, latanoprost and oral acetazolamide. He was continued on dorzolamide-timolol that he took for his long-standing history of mixed-mechanism glaucoma. He received an intravitreal Avastin (bevacizumab, Genentech) injection for the NVI/NVA and was started on cyclopentolate and prednisolone acetate drops for the intraocular inflammation. The next day, his pain had improved and IOP decreased to 23 mm Hg. The NVI/NVA started to regress, although gonioscopy revealed peripheral anterior synechiae throughout the angle. He subsequently underwent panretinal photocoagulation.

A carotid ultrasound was ordered, given the concern for OIS. The ultrasound revealed 80% to 99% stenosis in the right carotid artery, which was determined to be causing ocular ischemia in the patient’s right eye.

Discussion

OIS is rare, with a reported incidence of 7.5 cases per million population per year, and will occur in 4% to 18% of patients with hemodynamically significant carotid artery stenosis. This may be an underestimation due to misdiagnosis as other retinovascular disorders.

The majority of OIS cases are secondary to carotid artery stenosis. Ocular symptoms are frequently the first manifestation of carotid artery disease; 30% to 40% of patients with carotid occlusive disease will have episodes of transient vision loss or visual obscurations. In OIS, anterior segment examination often shows anterior chamber inflammation, neovascularization of the iris and/or angle, and cataract. Ectropion uveae may be observed in severe cases. Posterior segment evaluation of both eyes is often asymmetric (as opposed to diabetic retinopathy) and may reveal attenuated retinal arteries, retinal veins that are dilated but not tortuous, cotton wool spots, dot-blot hemorrhages in the midperiphery and neovascularization of the retina. Ischemia, in addition to elevated IOP, likely plays a role in the cause of ocular pain because only 30% of those with pain have IOP greater than 25 mm Hg.

Visual prognosis is poor. Around 97% of eyes with neovascularization of the iris end with vision of counting fingers or worse. Mortality is 40% at 5 years, most often due to myocardial infarction or cerebrovascular accident.

Treatment of OIS is focused on IOP reduction, treatment of ocular neovascularization and inflammation, and management of systemic risk factors. Intravitreal anti-VEGF agents can be used to treat neovascularization. Panretinal laser photocoagulation may be used in eyes with retinal ischemia and secondary neovascularization. Trabeculectomy for neovascular glaucoma has high failure rates due to inflammation and neovascularization; thus, aqueous shunt implants are the preferred management.

Most importantly, systemic treatment should be considered. Patients should be evaluated by an internist for consideration of anticoagulation, carotid recanalization or possible endarterectomy for those who meet treatment criteria. Careful monitoring of IOP after endarterectomy is critical because IOP may markedly increase after ocular blood flow is improved.

Follow-up

At 3 weeks’ follow-up, the patient’s vision was stable and his pain had resolved. There was complete regression of the NVI/NVA although the angle remained closed. IOP remained elevated above 30 mm Hg despite maximal medical therapy.

The patient underwent carotid endarterectomy 3 weeks after presentation and is currently recovering from Ahmed valve placement (New World Medical). His vision is stable, and IOP improved to 7 mm Hg.