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Current Endovascular Treatment Options for Central Retinal Arterial Occlusion: a Review

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Current Endovascular Treatment Options for Central Retinal Arterial Occlusion: a Review Neurosurg Focus 36 (1):E7, 2014 ©AANS, 2014 Current endovascular treatment options for central retinal arterial occlusion: a review NITIN AGARWAL, B.S.,1 NIHAR B. GALA, M.D.,1 REZA J. KARIMI, M.D.,1 ROGER E. TURBIN, M.D.,2 CHIRAG D. GandHI, M.D.,1,3 and CHARLES J. PRESTIGIacOMO, M.D.1,3,4 Departments of 1Neurological Surgery, 2Ophthalmology and Visual Science, 3Radiology, and 4Neurology and Neuroscience, Rutgers New Jersey Medical School, Newark, New Jersey Central retinal artery occlusion, although relatively rare, is an ophthalmological emergency. If left untreated, complete blindness will ensue. Conventional therapies have not significantly improved outcomes compared with the natural history of the disease. Several case series of more recent endovascular approaches, such as intraarterial fibrinolysis, report successful outcomes. Still other studies regarding intraarterial fibrinolysis do not demonstrate any significantly better outcomes, with some even indicating increased complication rates. Therefore, the authors present a review of the current endovascular treatment options for central retinal artery occlusion. (http://thejns.org/doi/abs/10.3171/2013.11.FOCUS13331) KEY WORDS • central retinal arterial occlusion • treatment • endovascular • fibrinolysis ENTRAL retinal artery occlusion (CRAO) is an oph- high association of CRAO with atherosclerosis, diabetes, thalmological emergency that can result in com- and systemic hypertension.16 plete blindness in the affected eye if untreated.3,5,6, C17,29,38,41,42,45 It is a result of sudden cessation of circulation Epidemiology, Etiology, and Presentation to the inner retinal layer, which is considered to be a part of the CNS. This condition was first described in 1859 The true incidence of CRAO in the population is by Albrecht von Graefe, a famous German ophthalmolo- unknown. One report estimated CRAO to occur in 1 per gist known for his contributions in glaucoma and cataract 10,000 outpatient visits.10 The department of ophthalmol- treatment.44 Since then, there has been an abundant accu- ogy at the Western Galilee–Nahariya Medical Center in mulation of literature regarding the disease. Nahariya, Israel, estimated an incidence of acute CRAO Although the pathophysiological features of CRAO (with a less than 48-hour onset) at that institution to be ap- are well described in the literature, the natural history of proximately 0.85 per 100,000 per year or 1.13 per 10,000 this disease is poorly understood. Prolonged retinal ische- outpatient visits.38 Of these patients, 1%–2% present with mia is usually irreversible, suggesting a grim prognosis if bilateral involvement. Men are more frequently affected the occlusion is not corrected in a timely manner.38 In a than are women, and the average age at presentation is in matter of hours cellular hypoxia will ensue, with subse- the early 60s, with rare cases as early as in the 30s.1,38,40,42 quent necrosis if circulation is not restored. The central Patients suffering from central retinal artery embolism retinal artery is usually occluded by a thrombotic em- have a 56% mortality rate over 9 years, compared with bolus (of internal carotid artery or cardiac origin, 15.5% 27% for age-matched individuals without emboli.18 Life of cases); a calcified embolus (commonly of diseased car- expectancy is 5.5 years postdiagnosis for patients with diac valve origin, 10.5% of cases); or cholesterol embolus CRAO compared with 15.4 years for age-matched indi- (74.5% of cases) (Table 1).2,27,32 Other causes include sud- viduals without CRAO. den narrowing of the arterial wall (hemorrhage into an Diagnosis is usually made based on clinical history atheromatous plaque) and inflammatory processes (arte- and physical examination. Ninety percent of patients pre- ritic CRAO) secondary to temporal arteritis.17 There is a sent with a complaint of acute, persistent, painless loss of vision. Some patients may report a history of amaurosis Abbreviations used in this paper: CRAO = central retinal artery fugax lasting anywhere from seconds to several hours. occlusion; EAGLE = European Assessment Group for Lysis in the Ophthalmoscopic examination may reveal diminished Eye; IAF = intraarterial fibrinolysis; IOP = intraocular pressure; blood vasculature in the retina, retinal edema, pale optic rt-PA = recombinant tissue plasminogen activator. disc, and a cherry red spot. Approximately 18.7% of the Neurosurg Focus / Volume 36 / January 2014 1 Unauthenticated | Downloaded 09/25/21 01:58 PM UTC N. Agarwal et al. TABLE 1: Known causes of CRAO* 1. systemic hypertension 2. embolism 3. thrombotic calcified cholesterol bacterial 4. atherosclerotic changes 5. temporal arteritis 6. hypercoagulable state 7. collagen vascular disease 8. cardiac valvular disease 9. cardiac anomalies 10. oral contraceptives 11. diabetes mellitus 12. polycythemia 13. polyarteritis nodosa 14. Behçet disease FIG. 1. Fluorescein angiographic photograph of CRAO. Note the de- layed and segmented arteriolar filling (a) in each of the major arteriolar 15. syphilis trunks distal to the central occlusion, and a small segment of an inferior 16. sickle cell disease temporal arteriole (b) that is strongly enhancing and may represent a 17. migraine fragment of distal embolization from the central blockage. Venous tran- sit is severely prolonged, and the typical laminar flow is nearly absent 18. glaucoma (http://www.rvscny.com/retinalarteryocclusion.html). Copyright Retina 19. prolonged orbital pressure Vi treous Surgeons of Central New York. Published with permission. * According to Fraser and Siriwardena. plete; there remains some residual blood flow distal to the occlusion.12,29 This allows a window for treatment before population has some macular circulation from a cilioreti- 28 significant morbidity occurs. It has been postulated that nal artery, which is spared in CRAO. These patients will treatment is only beneficial if commenced within 8 hours show finger-like areas of normal retina with patent arteri- of occlusion.17 However, beneficial effects were seen with oles that are not connected to the occluded stalk. In 20% 18 treatment given up to 24 hours after the onset of symp- of cases, the embolus can be directly visualized. Fluores- toms.37 Furthermore, significant visual improvement was cein angiography may show delayed filling or occlusion reported with treatment given after 48 hours of ischemia.5 during the arterial phase (11–12 seconds) (Fig. 1). Nevertheless, shorter latency to treatment results in better Due to the high probability of permanent visual loss, 38 7,11,35,38 outcomes. It is therefore important to review all treat- immediate correction of the occlusion is a priority. ment options that aim to restore circulation to the ische- In less than 10% of cases, spontaneous recanalization of 38,40 mic retina. the occlusion may occur. In eyes with presenting visual Reports of spontaneous recovery are inconsistent. Cer- acuity of finger counting or worse, visual acuity deterio- 31,39 21 tain authors report no spontaneous recovery, where as rated in 12% of cases. Nevertheless, medical treatment other case studies report some spontaneous recov ery.3,25,43 should be sought out on an emergency basis. Several ther- A recent study of the natural history of CRAO concludes apeutic options exist, ranging from noninvasive medical that classification of disease is important and spontane- options to the controversial thrombolysis treatment. This ous recovery can be determined by several factors, such as review of the literature discusses all the endovascular ther- presenting symptoms and etiology of occlusion.24 apeutic options available following the onset of CRAO, with a focus on intraarterial fibrinolysis (IAF), with con- Treatment Options current recommendations. The patient may begin treatment prior to arrival in 16 Natural History the emergency room by massaging the affected eye. Me- chanical force on the central retinal artery may dislodge Central retinal artery occlusion is an ophthalmologi- the embolus and restore circulation. This technique will cal emergency that can lead to permanent visual loss if only work if the occlusion is due to an embolus. The pa- left untreated. Restoration of circulation distal to the oc- tient may also breathe expired air using a paper bag. The clusion can result in complete recovery if recanalization subsequent hypercapnia will cause vasodilation of the ret- occurs rapidly. The monkey retina can tolerate complete inal arteries, with an increase in circulation. Carbogen, a ischemia for up to 105 minutes before irreversible dam- fixed mixture of 95% oxygen and 5% carbon dioxide, may age occurs,23 and up to 4 hours in the presence of residual be used if available.13,39 Retinal arteries may also dilate af- blood circulation.22 In humans CRAO is usually not com- ter sublingual isosorbide dinitrate is administered.38 Sys- 2 Neurosurg Focus / Volume 36 / January 2014 Unauthenticated | Downloaded 09/25/21 01:58 PM UTC Central retinal arterial occlusion treatments temic vasodilation may occur, causing decreased blood sion criteria included presence of branched retinal artery pressure. occlusion, cilioretinal artery, elevated IOP greater than 30 Immediate reduction of intraocular pressure (IOP) mm Hg, or severe general medical disease. Patients were may cause an increase in ocular perfusion pressure and randomly assigned into IAF treatment or control groups. therefore circulation as well. This may be attempted with Patients in the treatment group received a total of 50 mg intravenous
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