II. Definitions

  1. Central Retinal Artery Occlusion (CRAO)
    1. Central Retinal artery is occluded affecting all Visual Fields
  2. Branch Retinal Artery Occlusion (BRAO)
    1. Branches of central Retinal artery are occluded, with segmental Vision Loss

III. Epidemiology

  1. Annual Incidence: One per 100,000 (U.S.)
  2. Age (mean): 60 years old

IV. Pathophysiology

  1. Typically due to embolism to the Retinal artery
    1. May also occur due to thrombosis, inflammation or Eye Trauma
  2. Arteritis (e.g. Temporal Arteritis) accounts for <5% of cases

VI. Causes: Ophthalmic artery Occlusion

  1. See Transient Ischemic Attack
  2. Cholesterol emboli
  3. Thrombotic emboli
  4. Vasculitis
  5. Hypoperfusion
    1. Hemodialysis
    2. Severe shock
    3. Nocturnal artery Hypotension (awake with Vision Loss)
      1. Associated with antihypertensives taken near bedtime

VII. Symptoms

  1. See Transient Monocular Blindness (Amaurosis Fugax)
  2. Painless acute unilateral Vision Loss
    1. More than half of patients have only hand motion and light Perception
    2. CRAO causes Vision Loss over entire Visual Field, while BRAO results in focal Vision Loss
    3. May be preceded by prior episode of Amaurosis Fugax
  3. May be associated with other focal neurologic deficits
    1. See Transient Ischemic Attack
    2. Affects ipsilateral Carotid Artery circulation

VIII. Signs

  1. Visual Acuity severely reduced to light Perception only
    1. Branch Retinal Artery Occlusion may present with Visual Field cut
    2. Perform full Visual Field testing (differentiates CRAO from BRAO)
  2. Relative Afferent Pupillary Defect
    1. Pupil dilated with slow reaction
  3. Fundoscopic exam
    1. Retina appears pale-gray due to Retinal edema
    2. Macula with cherry-red spot on white-yellow background (collateral circulation from Choroid)
    3. Constricted arterioles
    4. Box-Carring of Retinal vessels
      1. Retinal vessels with interrupted columns of blood appear as train box cars
      2. Blood cells separate from serum
    5. Hollenhorst Plaques (white punctate Cholesterol emboli)
      1. "Glistening orange yellow flakes"
      2. Represent fragmented emboli at arteriole bifurcations
  4. Neck Exam
    1. Carotid Bruit

IX. Exam

  1. See Eye Vital Signs
  2. Visual Fields and Visual Acuity testing drive management and prognosis

X. Differential Diagnosis

XII. Diagnostics

XIII. Imaging: Obtain after acute stabilization (see management below)

  1. See Transient Ischemic Attack
  2. Evaluate as Transient Ischemic Attack or CVA (depending on deficits and timing)
    1. MRI Brain with Diffusion Weighted Imaging (CT misses "TIA" lesions) AND
    2. Evaluate Carotid Arteries: Carotid Ultrasound or Head and Neck CT Angiogram or MR Angiogram
  3. Echocardiogram
    1. Evaluate as performed in CVA and TIA

XIV. Precautions

  1. Central Retinal Artery Occlusion (CRAO) is a stroke of the eye
  2. Evaluate as Cerebrovascular Accident

XV. Management

  1. Immediate, emergent Ophthalmology Consultation without delay
    1. Irreversible Vision Loss begins in the first 90-120 minutes
    2. Manage in similar fashion to a stroke protocol
  2. Lower Intraocular Pressure or dislodge Occlusion
    1. Lie patient supine with both Eyelids closed
    2. Ballot the eye: Apply intermittent pressure to eyeball
      1. Emergently perform as soon as possible
        1. Offers benefit within 6 hours (possibly up to 24 hours)
      2. Massage the globe with index fingers or each hand, then release suddenly
      3. Apply pressure in repeated cycles of 5-10 seconds on and 5 seconds off
      4. Perform for 20 cycles total or from 5-30 minutes
      5. Goal is to dislodge a thrombus
        1. Aqueous outflow increases with eye pressure
        2. Retinal perfusion increases with release of eye pressure
    3. Ocular Paracentesis
      1. Ophthalmologist aspirates 0.1 to 0.4 ml anterior chamber fluid via 27-30 gauge needle
      2. Goal to reduce Intraocular Pressure and shift the embolism distally
      3. May offer benefit up to 24 hours after onset
    4. Consider Hypercarbia
      1. Patient rebreathes into a paper bag for 10 minutes of each hour OR
      2. Inhalation of mix of 5% carbon dioxide and 95% oxygen
      3. Goal is to result in eye vessel vasodilation due to increased carbon dioxide concentrations
    5. Consider Aqueous Humor production strategies
      1. Mannitol 1 g/kg IV for 1 dose AND Acetazolamide 500 mg IV for 1 dose OR
      2. Acetazolamide 500 mg orally for 1 dose
    6. Consider hyperbaric oxygen
      1. Murphy-Lavole (2012) Undersea Hyperb Med 39(5): 943-53 +PMID: 23045923 [PubMed]
    7. Other measures that have been used (discuss with ophthalmology)
      1. Timolol maleate (0.5%) one drop topically
      2. Pilocarpine drops to eye
      3. Oral Nitroglycerin
      4. Pentoxifylline (Trental) three 600 mg tablets daily
      5. Laser arteriotomy
      6. Embolectomy
    8. Experimental or insufficient evidence to support
      1. Intraarterial Thrombolysis
        1. Page (2018) Front Neurol 9:76 [PubMed]
  3. Cerebrovascular Management
    1. Approach as Transient Ischemic Attack
    2. Evaluate patients age <50 years old for Hypercoagulable state causes (e.g. Antiphospholipid Antibody Syndrome)
  4. Temporal Arteritis (ESR or CRP meet criteria)
    1. Start empiric Corticosteroids
    2. Temporal artery biopsy or Doppler Ultrasound

XVI. Prognosis

  1. Vision Loss risk increases after 90 minutes (and esp. after 4 hours) of arterial Occlusion
  2. Spontaneous visual improvement may occur in first 7 days after onset
  3. Final Visual Acuity in affected eye <20/400

XVII. References

  1. Hartmann (2016) Crit Dec Emerg Med 30(6): 3-11
  2. Sales, Patel and Patel (2019) Crit Dec Emerg Med 33(12): 3-13
  3. Werner and St Peter in Herbert (2021) 21(9): 13-4
  4. Beatty (2000) J Accident Emerg Med 17:324-9 [PubMed]
  5. Biousse (2018) Ophthalmology 125:1597-607 [PubMed]
  6. Gelston (2020) Am Fam Physician 102(9):539-45 [PubMed]
  7. Pokhrel (2007) Am Fam Physician 76:829-36 [PubMed]

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Related Studies

Ontology: Branch Retinal Artery Occlusion (C0006123)

Concepts Disease or Syndrome (T047)
MSH D015356
ICD9 362.32
ICD10 H34.23
SnomedCT 50821009
English Retinal arterial branch occlusion, BRAO - Branch ret art occlus, Branch ret artery occlusion, BRAO, branch retinal artery occlusion, branch retinal artery occlusion (physical finding), occlusion of retinal arterial branch, occlusion of retinal arterial branch (diagnosis), Arterial branch occlus, Retinal artery branch occlusion, Arterial retinal branch occlusion, BRAO - Branch retinal artery occlusion, Branch retinal artery occlusion, Arterial retinal branch occlusion (disorder), occlusion; artery, retina (partial) (branch), artery; occlusion, retina (partial) (branch), Retinal Arterial Branch Occlusion, Branch Retinal Artery Occlusion
Dutch retina arteriële tak occlusie, afsluiting; arterie, retina (partieel) (tak), arterie; afsluiting, retina (partieel) (tak), Occlusie van netvliesarterietak
French Occlusion d'une branche de l'artère rétinienne, Occlusion de branche artérielle rétinienne, Occlusion d'une branche de l'artère centrale de la rétine, Occlusion d'une branche de l'artère de la rétine
German partieller Verschluss eines Netzhautarterienschenkels, Netzhautarterienastverschluß
Italian Occlusione di ramo arterioso retinico, Occlusione di una branca dell'arteria retinica
Portuguese Oclusão de ramo arterial da retina, Oclusão do Ramo da Artéria Retiniana
Spanish Oclusión de una rama arterial de la retina, Oclusión de la Arteria de la Rama Retiniana, oclusión de la rama arterial retiniana (trastorno), oclusión de la rama arterial retiniana
Japanese 網膜動脈分枝閉塞, モウマクドウミャクブンシヘイソク
Czech Okluze větve retinální arterie
Hungarian Retinalis arteria ágelzáródás
Norwegian Okklusjon av retinaarteriegren, Okklusjon i retinal arteriegren, Retinaarteriegrenokklusjon, Retinal arterieokklusjon, i gren, Netthinnearteriegrenokklusjon

Ontology: Central Retinal Artery Occlusion (C0007688)

Concepts Disease or Syndrome (T047)
MSH D015356
ICD9 362.31
ICD10 H34.1
SnomedCT 38742007
English CRA - Centr ret art occlusion, CRAO - Central ret art occln, Central ret artery occlusion, CRAO, central retinal artery occlusion (physical finding), central retinal artery occlusion, occlusion of central retinal artery (diagnosis), occlusion of central retinal artery, Cent retina artery occlu, Central retinal artery occlusion, CRA - Central retinal artery occlusion, CRAO - Central retinal artery occlusion, Central retinal artery occlusion (disorder), obstruction; artery, retina, central, occlusion; artery, retina, central, occlusion; retina, central retinal artery, retina; occlusion central retinal artery, artery; occlusion, artery, retinal, central retinal artery, Central Retinal Artery Occlusion
Dutch centrale retina arterie occlusie, afsluiting; arterie, retina, centraal, afsluiting; retina, arteria centralis retinae, arterie; afsluiting, arterie retina, a.centralis retinae, obstructie; arterie, retina, centraal, retina; afsluiting arteria centralis retinae, Afsluiting van arteria centralis retinae, Occlusie van centrale netvliesarterie
German Verschluss der zentralen Netzhautarterie, Zentraler Netzhautarterienverschluß, Verschluss der A. centralis retinae
Portuguese Oclusão da artéria central da retina, Oclusão da Artéria Retiniana Central
Spanish Oclusión de la arteria central de la retina, Oclusión de la Arteria Retiniana Central, oclusión de la arteria retiniana central (trastorno), oclusión de la arteria retiniana central
Japanese 網膜中心動脈閉塞, モウマクチュウシンドウミャクヘイソク
Italian Occlusione dell'arteria centrale della retina, Occlusione dell'arteria retinica centrale
French Occlusion de l'artère centrale de la rétine, Thrombose de l'artère centrale de la rétine
Czech Uzávěr centrální retinální arterie
Korean 중심 망막 동맥 폐쇄
Hungarian Arteria centralis retinae elzáródás
Norwegian Sentral retinaarterieokklusjon, Sentral retinal arterieokklusjon