Retinal Artery Occlusion (RAO)

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Retinal Artery Occlusion (RAO)

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Painless loss of monocular vision is the usual presenting symptom of retinal artery occlusion (RAO). Ocular stroke commonly is caused by embolism of the retinal artery, although emboli may travel to distal branches of the retinal artery, causing loss of only a section of the visual field. Retinal artery occlusion represents an ophthalmologic emergency, and delay in treatment may result in permanent loss of vision.

Immediate intervention improves chances of visual recovery, but, even then, prognosis is poor, with only 21-35% of eyes retaining useful vision. Although restoration of vision is of immediate concern, retinal artery occlusion is a harbinger for other systemic diseases that must be evaluated immediately.

Blood supply to the retina originates from the ophthalmic artery, the first intracranial branch of the internal carotid artery that supplies the eye via the central retinal and the ciliary arteries. The central retinal artery supplies the retina as it branches into smaller segments upon leaving the optic disc. The ciliary arteries supply the choroid and the anterior portion of the globe via the rectus muscles (each rectus muscle has 2 ciliary arteries except the lateral rectus, which has 1).

Anatomical variants include cilioretinal branches from the short posterior ciliary artery, giving additional supply to part of the macular retina. A cilioretinal artery occurs in approximately 14% of the population.

Typical funduscopic findings of a pale retina with a cherry red macula (ie, the cherry red spot) result from obstruction of blood flow to the retina from the retinal artery, causing pallor, and continued supply of blood to the choroid from the ciliary artery, resulting in a bright red coloration at the thinnest part of the retina (ie, macula). These findings do not develop until an hour or more after embolism, and they resolve within days of the acute event. By this time, visual loss is permanent and primary optic atrophy has developed. In those with a cilioretinal artery supplying the macula, a cherry red spot is not observed.

See the image below.

An embolism, atherosclerotic changes, inflammatory endarteritis, angiospasm, or hydrostatic arterial occlusion may occlude the retinal artery. The mechanism of obstruction may be obvious from comorbid systemic disease or physical findings. Atrial fibrillation and ipsilateral carotid stenosis are more commonly associated with prolonged visual disturbances.

Animal studies have shown that a retina with completely occluded circulation has irreversible ischemic damage at 105 minutes but may recover at 97 minutes. Complete occlusion of retinal artery circulation in humans is rare with retinal artery disease; thus, retinal recovery is possible even after days of ischemia.

Branch retinal artery occlusion (BRAO) occurs when the embolus lodges in a more distal branch of the retinal artery. BRAO typically involves the temporal retinal vessels and usually does not require ocular therapeutics unless perifoveolar vessels are threatened. The central retinal artery is affected in 57% of occlusions, the branch retinal artery is involved in 38% of occlusions, and cilioretinal artery obstructions occur in 5% of occlusions. [1]

United States

Recent estimates put the incidence of retinal artery occlusion at 0.85 per 100,000 per year, with a 10-year cumulative incidence of retinal emboli of 1.5%. [2]

Patients with visualized retinal artery emboli, whether or not obstruction is present, have 56% mortality over 9 years, compared to 27% for an age-matched population without retinal artery emboli. Life expectancy of patients with central RAO (CRAO) is 5.5 years, compared to 15.4 years for an age-matched population without CRAO.

RAO is associated with smoking and cardiovascular disease, with an increased incidence of stroke in patients who have suffered RAO.

Both eyes have an equal incidence of disease, with bilateral involvement in 1-2%.

Men are affected slightly more frequently than women.

The mean age of presentation of retinal artery occlusion is early in the seventh decade of life, although a few cases have been reported in patients younger than 30 years.

The etiology of occlusion changes, depending on the age at presentation.

Recovery of useful vision is related directly to the rapidity of treatment and presenting visual acuity.

Studies report that 21% of patients exhibited visual improvement of 6 gradients of visual acuity, 35% exhibited improvement of 3 gradients of visual acuity, while 26% showed no improvement in visual acuity.

Patients that showed improvement had presenting visual acuity of counting fingers and a mean duration of visual loss of 21.1 hours; those that did not improve had presenting visual acuity of hand movement and a mean duration of visual loss of 58.6 hours.

The longest delay to treatment that has been associated with significant visual recovery is approximately 72 hours.

Presence of a cilioretinal artery with foveolar sparing increases improvement of visual acuity.

Branch retinal artery occlusions (BRAOs) are associated with a higher recovery rate (80% of eyes improve to 20/40 or better) than central retinal artery occlusions (CRAOs).

Patients must understand that the prognosis for visual recovery is poor and that the visual changes are usually a result of a systemic process that needs treatment.

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Fraser SG, Adams W. Interventions for acute non-arteritic central retinal artery occlusion. Cochrane Database Syst Rev. 2009 Jan 21. CD001989. [Medline].

Schrag M, Youn T, Schindler J, Kirshner H, Greer D. Intravenous Fibrinolytic Therapy in Central Retinal Artery Occlusion: A Patient-Level Meta-analysis. JAMA Neurol. 2015 Oct. 72 (10):1148-54. [Medline].

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Hattenbach LO, Kuhli-Hattenbach C, Scharrer I, Baatz H. Intravenous thrombolysis with low-dose recombinant tissue plasminogen activator in central retinal artery occlusion. Am J Ophthalmol. 2008 Nov. 146(5):700-6. [Medline].

Nowak RJ, Amin H, Robeson K, Schindler JL. Acute Central Retinal Artery Occlusion Treated with Intravenous Recombinant Tissue Plasminogen Activator. J Stroke Cerebrovasc Dis. 2012 Feb 18. [Medline].

Cohen JE, Moscovici S, Halpert M, Itshayek E. Selective thrombolysis performed through meningo-ophthalmic artery in central retinal artery occlusion. J Clin Neurosci. 2012 Mar. 19(3):462-4. [Medline].

Atebara NH, Brown GC, Cater J. Efficacy of anterior chamber paracentesis and Carbogen in treating acute nonarteritic central retinal artery occlusion. Ophthalmology. 1995 Dec. 102(12):2029-34; discussion 2034-5. [Medline].

Augsburger JJ, Magargal LE. Visual prognosis following treatment of acute central retinal artery obstruction. Br J Ophthalmol. 1980 Dec. 64(12):913-7. [Medline].

Beiran I, Reissman P, Scharf J, et al. Hyperbaric oxygenation combined with nifedipine treatment for recent-onset retinal artery occlusion. Eur J Ophthalmol. 1993 Apr-Jun. 3(2):89-94. [Medline].

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Butz B, Strotzer M, Manke C, et al. Selective intraarterial fibrinolysis of acute central retinal artery occlusion. Acta Radiol. 2003 Nov. 44(6):680-4. [Medline].

Cella W, Avila M. Optical coherence tomography as a means of evaluating acute ischaemic retinopathy in branch retinal artery occlusion. Acta Ophthalmol Scand. 2007 Nov. 85(7):799-801. [Medline].

Ffytche TJ, Bulpitt CJ, Kohner EM, et al. Effect of changes in intraocular pressure on the retinal microcirculation. Br J Ophthalmol. 1974 May. 58(5):514-22. [Medline].

Hayreh SS, Kolder HE, Weingeist TA. Central retinal artery occlusion and retinal tolerance time. Ophthalmology. 1980 Jan. 87(1):75-8. [Medline].

Hertzog LM, Meyer GW, Carson S, et al. Central retinal artery occlusion treated with hyperbaric oxygen. J Hyperbaric Med. 1992. 7:33-42.

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Magargal LE, Goldberg RE. Anterior chamber paracentesis in the management of acute nonarteritic central retinal artery occlusion. Surg Forum. 1977. 28:518-21. [Medline].

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Miyake Y, Horiguchi M, Matsuura M, et al. Hyperbaric oxygen therapy in 72 eyes with retinal arterial occlusion. 9th International Symposium on Underwater and Hyperbaric Physiology. 1987. 949-53.

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Benjamin Feldman, MD Associate Partner, Emergency Medicine Physician, The Permanente Medical Group; Clinical Professor, Touro University College of Osteopathic Medicine

Benjamin Feldman, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Pascal SC Juang, MD Medical Director, ED Information Systems, Department of Emergency Medicine, Hoag Memorial Hospital Presbyterian

Pascal SC Juang, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Bruce M Lo, MD, MBA, CPE, RDMS, FACEP, FAAEM, FACHE Medical Director, Department of Emergency Medicine, Sentara Norfolk General Hospital; Professor and Assistant Program Director, Core Academic Faculty, Department of Emergency Medicine, Eastern Virginia Medical School

Bruce M Lo, MD, MBA, CPE, RDMS, FACEP, FAAEM, FACHE is a member of the following medical societies: American Academy of Emergency Medicine, American Association for Physician Leadership, American College of Emergency Physicians, American College of Healthcare Executives, American Institute of Ultrasound in Medicine, Emergency Nurses Association, Medical Society of Virginia, Norfolk Academy of Medicine, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Assaad J Sayah, MD, FACEP Senior Vice President and Chief Medical Officer, Cambridge Health Alliance

Assaad J Sayah, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians, Massachusetts Medical Society

Disclosure: Nothing to disclose.

Robert E O’Connor, MD, MPH Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O’Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Heart Association, American Medical Association, National Association of EMS Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Neil Jain, MD Staff Physician, Yale University School of Medicine, Department of Surgery, Section of Emergency Medicine

Neil Jain, MD is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Kilbourn Gordon III, MD, and Enoch Huang, MD, to the development and writing of this article.

Retinal Artery Occlusion (RAO)

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