Central Retinal Artery Occlusion

Authors: Joe Walter, MD (@joewalter9999, EM/Hyperbaric Attending

Physician, Hennepin County Medical Center/ Healthpartners), R. Eric
Minnihan, MD (EM Attending Physician, Hyperbaric Medicine fellow,
Mayo Clinic Health System, Hennepin County Medical Center), Tom
Masters, MD (EM/Hyperbaric Attending Physician, Hennepin County
Medical Center), Chris Logue, MD (EM/Hyperbaric Attending
Physician, Hennepin County Medical Center), Bjorn Westgard, MD
(EM/Hyperbaric Attending Physician, Hennepin County Medical
Center/ Healthpartners), Stephen Hendriksen, MD (EM/Hyperbaric
Attending Physician, Hennepin County Medical Center) // Edited by:
Alex Koyfman, MD (EM Attending Physician, UT Southwestern Medical
Center / Parkland Memorial Hospital, @EMHighAK) & Justin Bright, MD
(EM Attending, Henry Ford Hospital, @JBright2021)

Case:

An 82 year-old man with a history of dyslipidemia, hypertension, and

CAD s/p stenting was driving home from the grocery store when he
experienced sudden vision loss in his right eye around 2:30 pm. He has
no previous history of vision problems and is puzzled but eventually
concerned. By the time he presents to the ED he has light perception
only in his right eye. He cannot not recognize motion. Central retinal
artery occlusion (CRAO) is first in the differential.

Introduction:

Central Retinal Artery Occlusion is characterized by a sudden painless

loss of vision in one eye. It occurs when there is a blockage of the
central retinal artery causing ischemia and infarction to the retina.
Incidence is 1-10/100,0001 with a mean age of 60-65 and over 90% of
cases occurring in those over 40.2,3,4 Providers are increasingly
recognizing this as a cerebral vascular accident that shares the same
risk factors commonly associated with stroke: hypertension,
hyperlipidemia, diabetes, and tobacco use1. Recent studies have
shown that acute cerebral infarcts often accompany CRAO and a
marked incidence in stroke and acute myocardial infarction occur in
the month following a CRAO.5,6

Pathophysiology/Anatomy:

The first branch off of the internal carotid artery is the ophthalmic
artery which splits into the posterior ciliary and central retinal arteries
which supply the eye. In CRAO there is an occlusion of the central
retinal artery causing a profound vision loss. In some cases the ciliary
arteries are able to perfuse the periphery of the retina and maintain
perfusion to the central portion of the retina. Research has shown that
the retina can only survive 90-100 minutes of ischemia prior to
permanent damage.7,8 However, cases with visual recovery beyond
this timeframe have been reported, potentially due to incomplete
occlusion, an intact cilioretinal artery, or collateral flow.

Etiology:

Although there are many etiologies for CRAO, carotid artery stenosis is
thought to be the most common cause and is present in up to 70% of
cases.2 Cardioembolic disease is another prevalent etiology and is
more likely in those under 40 and in those with a history of atrial
fibrillation or valvular disease. Giant cell arteritis, vasculitis, Sickle cell,
carotid artery dissection, Moyamoya, hypercoagulable states (SLE,
antiphospholipid, hematologic cancers), and iatrogenic causes
(injections, cerebral angiogram, carotid endarterectomy) are less
common causes of CRAO.

Presentation:

A patient typically presents with acute, profound, painless, monocular

vision loss with potentially a small amount of temporal sparing.
Approximately 20% of the population has a cilio-retinal artery which
can lead to some central sparing in case of a CRAO.7 On exam, patients
will demonstrate a complete or relative afferent pupillary defect,
though this need not be present. Funduscopic exam will show retinal
whitening (a “cherry red spot,” described in 90% of patients with acute
CRAO) or “box-carring” (vascular attenuation with stacking of red
cells within the vasculature, only seen in 15% of patients with acute
CRAO).9

The particular history of past and present illness is important in each

patient, as additional symptoms may lead to a specific etiology.
Headache and/or temporal tenderness may suggest Giant Cell Arteritis
while neck pain or recent cervical trauma may suggest dissection. It is
also important to remember the differential diagnosis, which includes
occipital stroke, retinal detachment, complex or atypical migraine, and
other ischemic optic neuropathies.

Management:

Recent painless vision loss is an ocular emergency that should prompt

immediate ophthalmology consultation. Concerns about vasculitis or
Giant Cell Arteritis deserve the addition of an ESR/CRP to the workup.
A complete stroke and cardiovascular work up is needed either in the
ED or during inpatient admission, as there is a high correlation with
additional stroke or myocardial infarction in the period immediately
following onset of a CRAO (Incident Rate Ratio of 14.0 (95% confidence
interval, 8.90-22.00)).5,6 Despite this only one-third of
ophthalmologists transfer patients with incident CRAO to an
emergency department for immediate evaluation.5

As this in an ophthalmologic emergency, management is usually in

coordination with ophthalmology and early consultation is
recommended. No clinical trials have demonstrated improvement with
any treatment compared with observation10 and historically CRAO has
an abysmal prognosis.10,11 Despite the unlikely chance of
improvement, maneuvers are usually attempted if a patient presents
within 24 hours including:10,12

Ocular massage
Ocular pressure lowering agents / maneuvers
Topical agents such as timolol
IV agents such as acetazolamide or mannitol
Anterior chamber paracentesis
Vasodilatory Agents
Nitroglycerin
Pentoxifylline
Isosorbide
 Carbogen
Breathing into a bag (to increase CO2 which causes
vasodilatation)

Again, there is little data to show that any of these methods improve
outcomes over control and there is some evidence to suggest that that
these interventions may actually be associated with worsened visual
outcomes and recovery rates.13

As CRAO is a vaso-occlusive phenomenon, there is a great deal of

interest in the use of tPA in its treatment. While there are published
reports of vision improvement after the administration of tPA,13 there
is debate about whether or not this improvement is greater than the
natural history of CRAO.11 Additionally, there is concern regarding
hemorrhage associated with the administration of tPA and as such, the
use of tPA in patients with CRAO is not currently considered standard
of care. As a result of this, the traditional “treatment” of CRAO has
been observation alone.11

Hyperbaric Oxygen Experience/Data:

Recently, the undersea and hyperbaric medicine society made a

recommendation for the consideration of hyperbaric oxygen therapy
(HBOT) in patients with a CRAO.14 While experiencing a CRAO, the
inner retinal layers become ischemic due to poor perfusion /
oxygenation. Animal models have shown that under hyperbaric
conditions, the collateral circulation from the choroid is capable of
supplying 100% of the retina’s oxygen needs.15,16 Additionally, as
mentioned before, approximately 20% of the population has cilioretinal
artery, which supplies blood to the area around the macula. This
ability to hyperoxygenate and meet the retina’s oxygen demands while
the central retinal artery re-cannulates is part of the rationale behind
the use of hyperbaric oxygen. Additional proposed mechanisms are
related to hyperbaric oxygen’s effect on edema reduction and its
ability to blunt ischemia-reperfusion injury after re-canalization
occurs.17

There are a number of clinical trials looking at the effect of hyperbaric

oxygen on patients affected by a CRAO. In a literature summary of 476
patients treated with hyperbaric oxygen, 306 (65%) experienced
vision improvement after their treatment.18 Overall, the American
Heart Association classification of evidence was considered IIB with
fair to good evidence with retrospective control case series, but no
prospective randomized controlled trials.18 Additionally noteworthy is
that therapy with hyperbaric oxygen is generally considered to be
benign and safe with proper patient selection and medical control.

To add to the existing evidence, Hennepin County Medical Center has

one of the largest single cohorts of CRAO patients treated with
hyperbaric oxygen therapy. Patients found to have a CRAO are being
treated with hyperbaric oxygen and seeing significant results. In
patients who are treated in <6 hours from time of onset, 83% are
seeing improvement in their vision, averaging 6 lines of improvement
on a Snellen eye chart. Overall, patients had 4.6 lines of improvement
when treated with HBOT.19 Though this is a small and promising study,
further investigation is needed. As with all strokes, it appears that time
is crucial.
Case Resolution:

Our patient is transferred to a hospital that has emergent hyperbaric

capabilities and is evaluated by the Emergency Staff, Neurology and
Ophthalmology. CRAO is confirmed and he is taken for hyperbaric
oxygen therapy at 9:30 that evening (7 hours after onset of vision
loss). During treatment, his vision improves to 20/200 (he can read the
big E), and after treatment examination shows that he has improved to
20/100. On examination the following day, he is able to count fingers
but is no longer able to read the eye chart. Immediately after his
second treatment, he is again 20/100. Ophthalmology evaluation later
that day shows a corrected vision of 20/20 -1. The patient reports
some small gray spots, but otherwise marked improvement. He is then
treated BID for a total of 10 treatments. During his hospitalization he
had a stroke work up including an MRI, CTA of the neck and an echo.
On reevaluation 6 weeks later, he continues to see 20/20 with
correction.

Key Points:

Presentation: Painless vision loss

Early ophthalmology consultation (true eye emergency)
Should be treated as a stroke
Traditionally poor prognosis overall
Promising HBO experience thus far. Consider early consultation with
an emergent hyperbaric treatment center if available
References / Further Reading

1. Leavitt JA, Larson TA, Hodge DO, Gullerud RE. The incidence of central
retinal artery occlusion in Olmsted County, Minnesota. Am J
Ophthalmol 2011; 152:820.
2. Yuzurihara D, Iijima H. Visual outcome in central retinal and branch
retinal artery occlusion. Jpn J Ophthalmol 2004; 48:490.
3. Park SJ, Choi NK, Seo KH, et al. Nationwide incidence of clinically
diagnosed central retinal artery occlusion in Korea, 2008 to 2011.
Ophthalmology 2014;121:1933–8.
4. Smit RL, Baarsma GS, Koudstaal PJ. The source of embolism in
amaurosis fugax and retinal artery occlusion. Int Ophthalmol 1994;
18:83.
5. Park et al. Risk and Risk Periods for Stroke and Acute Myocardial
Infarction in Patients with Central Retinal Artery Occlusion.
Ophthalmology. 2015 Nov;122(11):2336-2343.e2. doi:
10.1016/j.ophtha.2015.07.018. Epub 2015 Aug 19.
6. Lee et al. Co-occurrence of acute retinal artery occlusion and acute
ischemic stroke: diffusion-weighted magnetic resonance imaging
study. American Journal of Ophthalmology. June 2014;157(6):1231-
1238.
7. Brown GC, Shields JA. Cilioretinal arteries and retinal arterial occlusion.
Arch Ophthalmol 1979; 97:84.
8. Hayreh SS, Kolder HE, Weingeist TA. Central retinal artery occlusion
and retinal tolerance time. Ophthalmology 1980; 87:75.
9. Hayreh SS, Zimmerman MB. Fundus changes in central retinal artery
occlusion. Retina. 2007;27(3): 276-289.
10. Rudkin AK, Lee AW, Aldrich E, et al. Clinical characteristics and
outcome of current standard management of central retinal artery
occlusion. Clin Experiment Ophthalmol 2010; 38:496.
11. Hayreh SS. Ocular vascular occlusive disorders: Natural history of
visual outcomes. Progress in Retinal and Eye Research. 2014;41:1-25.
12. Tintinalli, Judith, Gabor Kelen, and J. Stephan Stapczynski. Emergency
 Medicine: A Comprehensive Study Guide -6th Edition. New York: The
McGraw-Hill Companies, Inc., 2004. Print.
13. Schrag M, Youn T, Schindler J, Kirshner H, Greer D. Intravenous
fibrinolytic therapy in central retinal artery occlusion. A patient-level
meta-analysis. JAMA Neurology. 2015;72(10):1148-1154.
14. Murphy-Lavoie H, Butler F, Hagan C. Arterial Insufficiencies: Central
Retinal Artery Occlusion. In: Weaver LK, ed. Hyperbaric Oxygen
Therapy Indications. 13th ed. North Palm Beach: Best Publishing
Company; 2014.
15. Patz A. Oxygen inhalation in retinal artery occlusion. American Journal
of Ophthalmology. 1955;40:789-795.
16. Li HK, Dejean BJ, Tang RA. Reversal of visual loss with hyperbaric
oxygen treatment in a patient with Susac Syndrome. Ophthalmology.
1996;103(12):2091-2098.
17. Buras JA, Garcia-Covarrubias L. Ischemia-reperfusion injury and
hyperbaric oxygen therapy. Basic mechanisms and clinical studies. In:
Neuman TS, Thom SR, eds. Physiology and Medicine of Hyperbaric
Oxygen Therapy. 1st ed. Philadelphia, PA: Saunders Elsevier; 2008.
18. Murphy-Lavoie H, Butler F, Hagan C. Central retinal artery occlusion
treated with oxygen: a literature review and treatment algorithm.
Undersea and Hyperbaric Medicine. September-October
2012;39(5):943-953.
19. Masters T, Westgard B, Hendriksen S, Walter J, Logue C. Central retinal
artery occlusion treated with hyperbaric oxygen. A retrospective
review. Paper presented at: UHMS Annual Scientific Meeting, 2015;
Montreal.

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