Central Retinal Artery Occlusion

[Section Outline]

Description
Etiology

Author:

YasuharuOkuda

BradenHexom

Description

Obstruction of the central retinal artery associated with sudden painless loss of vision
Usually occurs in persons 50-70 yr of age
Ophthalmic artery is the first branch of carotid
Risk factors include HTN, atherosclerotic disease, sickle cell disease, vasculitis, valvular heart disease, lupus, trauma, and coronary artery disease
Incidence of 1-10/100,000
Often described as a “stroke of the eye”

Etiology

Embolic:
- Occlusion by intravascular material from a proximal source:
  - Atherosclerotic disease (majority)
  - Carotid artery stenosis
  - Valvular heart disease (cardiogenic emboli)
  - Atrial myxoma
  - Dissection of the ophthalmic artery
  - Carotid artery dissection
Thrombotic:
- Obstruction of flow from the rupture of a pre-existing intravascular atherosclerotic plaque
- Hypercoagulable states (sickle cell)
Inflammatory:
- Due to giant cell arteritis, temporal arteritis, lupus, vasculitis
Arterial spasm:
- Associated with migraine headaches
Decreased perfusion:
- Low-flow conditions such as in severe hypotension or high-pressure situations seen in acute angle-closure glaucoma or retrobulbar hemorrhage

Diagnosis ⬆ ⬇

[Section Outline]

Signs and Symptoms
Essential Workup
Diagnostic Tests & Interpretation
Differential Diagnosis

Signs and Symptoms

History

Sudden, painless, monocular loss of vision
Prior episodes of sudden visual loss:
- May last a few seconds to minutes (amaurosis fugax)
- Caused by transient embolic phenomena or decreased ocular blood flow

Physical Exam

Significantly decreased visual acuity
Afferent pupillary defect usually present
Retinal appearance:
- Emboli visualized within vascular tree of the retina
- Appears as glinting white or yellow flecks (Hollenhorst plaques) within the vessels
- Ischemic edema visible within 15-20 min of occlusion
- “Cherry-red spot” remains over the fovea (only area where there is very thin retina allowing the vascular choroids to show through)
- Affected arteries empty or showing dark red stationary or barely pulsatile segmented rouleaux (“box-carrying”)
- Within 1-2 hr opacification of the usually transparent, infarcting retinal nerve layer occurs
Partial field deficits:
- Occur only if branch of central retinal artery involved

Essential Workup

Visual acuity and visual field testing
Fundoscopic exam
Intraocular pressure measurements
Emergent ophthalmologic consultation

Diagnostic Tests & Interpretation

Lab

Directed toward evaluating underlying etiology of occlusion:

CBC with differential and platelet count
PT/PTT
Electrolytes, BUN/creatinine, glucose
Electronic spin resonance for giant cell arteritis (in patients >55 yr old)
ANA, RF, CRP, ESR
Rapid plasma reagin (RPR)
Hemoglobin electrophoresis
Serum protein electrophoresis

Imaging

Directed toward evaluating underlying etiology of occlusion:

Carotid artery US/Doppler
Possibly echocardiography
Fluorescein angiography or electroretinography to confirm the diagnosis

Differential Diagnosis

Acute angle-closure glaucoma
Central retinal vein occlusion
Giant cell arteritis (temporal arteritis)
Optic neuritis
Retinal detachment

Treatment ⬆ ⬇

[Section Outline]

ED Treatment/Procedures
Medication

ALERT

Initiate treatment immediately because irreversible visual loss occurs at 90 min:

Only immediate treatment may help to salvage or restore sight to the affected eye
Goals of therapy include dislodging or dissolving the embolus, arterial dilation to improve forward flow, and reduction of intraocular pressure to improve the perfusion gradient
Currently, there is not enough evidence to support any of the listed treatments, and no treatment has been found to be better or worse than observation, though several studies are ongoing

ED Treatment/Procedures

Immediate global massage in an attempt to dislodge the embolus:
- Lay patient flat and apply digital global massage bolus
- On closed eyelid, apply constant pressure for 15 s and remove for 15 s. Repeat for 5 cycles
Initiate high-flow oxygen via 100% nonrebreather:
- Consider transfer to a facility capable of providing hyperbaric oxygen (HBO) if <24 hr from symptom onset
- May use inhaled carbogen (a mixture of carbon dioxide and oxygen gas) if available
Administer IV acetazolamide to decrease intraocular pressure
Apply topical timolol maleate to reduce intraocular pressure
Administer aspirin and IV heparin for prevention of clot propagation
Obtain emergent ophthalmology consultation for:
- Anterior chamber paracentesis to help reduce intraocular pressure
- Possible intra-arterial fibrinolysis for clot lysis
Administer high-dose systemic steroids in suspected cases of inflammatory arteritis

Medication

First Line

Acetazolamide: 500 mg IV/PO
Carbogen: Inhalation of 95% oxygen and 5% carbon dioxide mixture
Heparin: 80 U/kg IV bolus then 18 U/kg/hr continuous infusions (rate adjusted based on PTT level)
Timolol maleate 0.5% solution: 1 drop topically to affected eye

Second Line

Methylprednisolone: 250 mg IV in suspected cases of inflammatory arteritis
Aspirin: 325 mg PO
Mannitol
Sublingual nitroglycerin

Follow-Up ⬆ ⬇

[Section Outline]

Disposition
Follow-up Recommendations

Disposition

Admission Criteria

Required for workup of proximal cause in acute cases (source of embolism, thrombosis, or inflammatory)

Discharge Criteria

Chronic retinal artery occlusion with no evidence of active disease can be worked up as an outpatient

Issues for Referral

All suspected cases warrant emergent ophthalmology consultation

Follow-up Recommendations

Most cases will require carotid US to exclude atherosclerotic disease

Pearls and Pitfalls ⬆ ⬇

Amaurosis fugax (transient, possibly resolved retinal artery occlusion) is a sentinel event and may lead to complete occlusion or stroke. Do not ignore these symptoms and urgent workup is required
Retinal artery occlusion is a medical emergency requiring immediate treatment to prevent loss of the eye
It is important to document a full eye exam including visual acuity and evaluation of the optic fundus

Additional Reading ⬆ ⬇

DattiloM, BiousseV, NewmanNJ. Update on the management of central retinal artery occlusion . Neurol Clin. 2017;35(1):83-100.
FraserSG, AdamsW. Interventions for acute non-arteritic central retinal artery occlusion . Cochrane Database Syst Rev. 2009;(1):CD001989.
Murphy-LavoieH, ButlerF, HaganC. Central retinal artery occlusion treated with oxygen: A literature review and treatment algorithm . Undersea Hyperb Med. 2012;39(5):943-953.
RudkinAK, LeeAW, AldrichE, et al. Clinical characteristics and outcome of current stand ard management of central retinal artery occlusion . Clin Exp Ophthalmol. 2010;38(5):496.
VortmannM, SchneiderJL. Acute monocular visual loss . Emerg Med Clin North Am. 2008;26(1):73-96.

See Also (Topic, Algorithm, Electronic Media Element)

Codes ⬆

ICD9

362.31 Central retinal artery occlusion

ICD10

H34.10 Central retinal artery occlusion, unspecified eye
H34.11 Central retinal artery occlusion, right eye
H34.12 Central retinal artery occlusion, left eye
H34.13 Central retinal artery occlusion, bilateral
H34.1 Central retinal artery occlusion

SNOMED

38742007 Central retinal artery occlusion

section name header

Basics ⬇

Diagnosis ⬆ ⬇

History

Physical Exam

Lab

Imaging

Treatment ⬆ ⬇

ALERT

First Line

Second Line

Follow-Up ⬆ ⬇

Admission Criteria

Discharge Criteria

Issues for Referral

Pearls and Pitfalls ⬆ ⬇

Additional Reading ⬆ ⬇

Codes ⬆