This article is more than 5 years old. Information may no longer be current.
Elderly man presents with severe, intermittent left eye pain
Exam showed left-sided narrow angles, dilated retinal veins and attenuated retinal arteries.
An 80-year-old retired firefighter with a history of wet age-related macular degeneration since 1990 presented to the retinal specialist for new intermittent severe left throbbing eye pain over the past month. The pain woke him up from sleep and was so severe at times that he wanted to “pluck [his] eyeball out of the socket.” He denied flashes or floaters. He reported his vision was slightly dimmer at times in this eye. He was pseudophakic in both eyes, and he had been followed by his retinal specialist regularly for the past 28 years. He had a history of subretinal surgery with choroidal neovascularization removal in the left eye in 1995. Since then, his left eye had undergone no other treatment except cataract surgery. His right eye had developed wet AMD 10 years before the current presentation and was receiving regular anti-VEGF injections.
The patient’s medical history was significant for recently diagnosed adenocarcinoma of the lung, for which he started radiation treatment to his lungs 6 months ago. He also had laryngeal cancer approximately 30 years ago that was successfully treated with neck radiation. In addition, he had severe chronic obstructive pulmonary disease on 1 L of oxygen at baseline, bilateral carotid stenosis, hypertension, hyperlipidemia, coronary artery disease, early vascular dementia, depression, dysphagia and a recent bout of community-acquired pneumonia 2 months ago that resolved with IV antibiotics. His current medications included aspirin, albuterol inhaler, fluticasone/salmeterol inhaler, umeclidinium inhaler, amlodipine, hydrochlorothiazide, triamterene, verapamil, sertraline, mirtazapine, vitamin B12, vitamin D3 and Ocuvite (Bausch + Lomb). He had mild adverse side effects to lisinopril and erythromycin, which caused cough and itching, respectively. He had a 65+ pack-year smoking history and quit 6 months ago when he was diagnosed with lung cancer. He denied drinking or drug use. His family history was significant for cancer in his brother and son.
Source: Huan Mills, MD, and Jay S. Duker, MD
Examination
Visual acuity was 20/200 in the right eye and 20/400 in the left eye. IOP was 15 mm Hg in the right eye and 24 mm Hg in the left eye. His right eye was white and quiet with a clear cornea and a deep, quiet anterior chamber. His left eye had 1+ conjunctival injection, iris neovascularization, trace cell and 1+ flare. Gonioscopy of the right eye revealed a wide-open angle with ciliary body visible 360° with 2+ pigmentation. Gonioscopy of the left eye revealed significant angle closure with no angle structures visible and a few areas of angle neovascularization. Dilated fundus exam in both eyes revealed a clear vitreous. The optic nerve was pink and healthy in both eyes with a small cup-to-disc ratio. Retinal arteries and veins appeared to be of normal caliber in the right eye. Retinal veins appeared dilated in the left eye with narrowing and attenuation of left retinal arteries (Figure 1). There were disciform scars in the macula bilaterally. OCT of the left eye revealed subretinal hyperreflective material with retinal atrophy (Figure 2). There were no hemorrhages in either macula or peripheral retina bilaterally.
What is your diagnosis?
See answer on next page.
Left eye pain
The three most common causes of new neovascular glaucoma are proliferative diabetic retinopathy (PDR), central retinal vein occlusion (CRVO) and ocular ischemic syndrome (OIS). PDR and CRVO are each responsible for approximately one-third of cases of neovascular glaucoma. OIS is the third most common cause of neovascular glaucoma and is responsible for 13% of cases. Central retinal artery obstruction is responsible for less than 10% of cases. The patient did not have a history of diabetic retinopathy, which made PDR unlikely. The dilated veins and attenuated arteries can be seen with both CRVO and OIS, but CRVO usually presents with more tortuous veins and flame-shaped hemorrhages. Optic nerve edema is a common finding in CRVO that differentiates it from OIS. The patient also had decreased central retinal artery perfusion pressure, which suggests a diagnosis of OIS.
Workup and management
The test of choice to distinguish among OIS, CRVO and PDR is fluorescein angiography (FA). The patient showed classic FA findings of OIS (Figure 3). He manifested delayed, patchy choroidal filling. His choroid began to fill at 24 seconds; in normal patients, choroidal filling is usually complete by 10 seconds. In CRVO and PDR, choroidal filling is usually normal. There was also delayed retinal artery filling and delayed venous filling. Retinal arteries normally fill completely within approximately 12 seconds, and this patient had double the retinal artery filling time, with incomplete filling noted at 24 seconds. Another prominent finding that confirms the diagnosis of OIS is the leading edge dye, which is indicative of slow arterial filling. Retinal veins normally fill within 30 seconds, and our patient had significant delay of venous filling, with non-perfused veins still visible at 1 minute 26 seconds. Late staining of retinal arteries can also be observed at 4 minutes.
Discussion
OIS was first described by Thomas R. Hedges Jr., MD, in 1963. The pathogenesis is most commonly stenosis and occlusion of the common carotid or internal carotid arteries, leading to symptoms of transient ipsilateral vision loss with underlying chronic progressive hypoperfusion of the eye. The mean age of onset is 65 years, and men are twice as likely to be affected by OIS. The majority of cases are unilateral, but OIS can be bilateral in 20% of cases. The most common presenting symptom is vision loss (91%). Retinal hemorrhages, usually located in the mid-periphery, can also be seen in 80% of patients. Iris neovascularization occurs in 62% of patients. Approximately 40% of patients with OIS report pain, and it is thought that the pain is from ischemia of the eye or high IOP. Treatment of elevated IOP can be done topically with beta-blockers, alpha-agonists or carbonic anhydrase inhibitors. The development of neovascular glaucoma can be treated with trabeculectomy, tube shunt or cyclophotocoagulation (CPC). Panretinal photocoagulation (PRP) can be done in an attempt to induce regression of iris neovascularization, but it is effective in only 36% of cases because, although PRP treats retinal ischemia, it does not address the uveal ischemia that also occurs in OIS nor the complete angle closure. Anti-VEGF therapy can be used to treat neovascularization.
Definitive treatment of OIS is carotid endarterectomy when possible. The surgical risk of carotid endarterectomy is weighed against the perioperative risk of stroke or death. If there is symptomatic stenosis of 50% to 99%, surgery is indicated if the perioperative risk is less than 6%. If there is asymptomatic stenosis of 60% to 99%, the perioperative risk of stroke or death should be less than 3%. Carotid endarterectomy is most effective at treating OIS if done before the development of iris neovascularization or neovascular glaucoma. Carotid artery stenting is an alternative option for patients who have symptomatic severe stenosis with comorbidities that increase the risk of surgery. OIS is associated with a poor prognosis. The most common cause of death is myocardial infarction (67%), and the second most common cause of death is stroke (19%). The risk of stroke is approximately 4% per year. The presence of iris neovascularization is associated with a poor visual outcome, and 97% of patients with iris neovascularization have a final visual outcome of count fingers or worse 1 year after diagnosis.
Clinical course continued
On the day of presentation, the patient received an intravitreal injection of bevacizumab in the left eye. Several days later, PRP was performed in the left eye. Visual acuity in the left eye decreased from 20/400 to count fingers at the 1-week follow-up visit. He was referred to vascular surgery and underwent CT angiography, which showed significant narrowing of the left common carotid artery with 70% to 75% stenosis. He also had 40% stenosis of the proximal right internal carotid artery. The patient was deemed not a candidate for surgery due to current lung cancer, severe chronic obstructive pulmonary disease and prior neck radiation for laryngeal cancer. He was started on maximal topical pressure-lowering therapy in the left eye with dorzolamide and brimonidine. He was also given acetazolamide by mouth to try to lower the IOP, but he could not tolerate the medication due to dehydration. He is currently scheduled for a CPC procedure in the left eye.
- References:
- Brott TG, et al. Circulation. 2011;doi:10.1161/CIR.0b013e31820d8c98.
- Duker JS, et al. Ophthalmology. 1990;doi:10.1016/S0161-6420(90)32488-0.
- Hedges TR Jr. Am J Ophthalmol. 1963;doi:10.1016/0002-9394(63)90382-9.
- Mendrinos E, et al. Surv Ophthalmol. 2010;doi:10.1016/j.survophthal.2009.02.024.
- Paraskevas KI, et al. J Vasc Surg. 2012;doi:10.1016/j.jvs.2012.01.084.
- Rodrigues GB, et al. Int J Retina Vitreous. 2016;doi:10.1186/s40942-016-0051-x.
- Shazly TA, et al. Semin Ophthalmol. 2009;doi:10.1080/08820530902800801.
- Sivalingam A, et al. Int Ophthalmol. 1991;doi:10.1007/BF00150974.
- For more information:
- Huan Mills, MD, and Jay S. Duker, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Adam T. Chin, MD, and Omar Dajani, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.