Ischemic Optic Neuropathy - PowerPoint Presentation

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Ischemic Optic Neuropathy

Ophthalmology & Neuro-ophthalmology Dr. Omer Y. Bialer

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Disclosure

No conflict of interestsI have nothing to discloseION = Ischemic Optic Neuropathy

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Presentation’s outline

Introduction Terminology and NosologyNonarteritic anterior ischemic optic neuropathyArteritic IONPerioperative IONRadiation optic neuropathy“Take home massage” summary

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Introduction

ION is the most common acute optic neuropathy > age 502nd most common optic neuropathy after glaucomaRelatively common neuro-ophthalmological disorderVisual loss is often severeNo effective treatment or prevention

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Introduction

ION is due to:

poor blood flow to the optic nerve Acute occlusion of the feeding arteries

Short posterior ciliary arteries

Ophthalmic artery

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Terminology & Nosology

ION

Nonarteritic Anterior ION (NAION)with swollen optic disc

Nonarteritic Posterior ION (NA-PION) with normal optic disc

Arteritic ION (vasculitis)

Nonarteritic ION (cardiovascular risk factors)

Arteritic Posterior ION (APION) with normal optic disc

Arteritic Anterior ION (AAION)with swollen optic disc

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Terminology & Nosology

ION

Nonarteritic Anterior ION (NAION)with swollen optic disc

Nonarteritic Posterior ION (NA-PION) with normal optic disc

Arteritic ION (vasculitis)

Nonarteritic ION (cardiovascular risk factors)

Arteritic Posterior ION (APION) with normal optic disc

Arteritic Anterior ION (AAION)with swollen optic disc

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Idiopathic ION

Radiation optic neuropathy

Perioperative ION

GCA

Other

vasculitides

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NAION

(Nonarteritic Anterior Ischemic Optic Neuropathy)

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NAION is the most common ION

~ 90% of IONIncidence: 1 / 10,000 / year (> 50 y.o) 0.5/ 100,000 / year (overall)Mean age at onset 57-65Presentation: acute painless monocularvisual field loss ± visual acuity loss

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The most important risk factor is a crowded optic disc

“

disc at risk” = small optic disc + minimal cup

crowded

normal

glaucoma

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More risk factors for NAION

Hypertension (50%)Diabetes mellitus (25%)Obstructive sleep apnea (55%)HyperlipidemiaIschemic heart diseaseObesityTobacco useHigh intraocular pressure

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Several meds are associated with NAION

Erectile dysfunction drugs Amiodarone VasoconstrictorsCocaine

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(e.g. Viagra, Cialis

)

(e.g

. nasal decongestants

)

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The pathogenesis of NAION differs from IHD or CVA

decrease in blood flow

Edema of optic disc

Blockage of axonal flow

Compression of axons and blood vessels

Necrosis and demyelination of nerve fibers

Cardiovascular risk factors

Crowded optic disc

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Eye Exam

visual acuity & color vision can be normalA relative afferent pupillary defectNormal anterior segmentOptic disc edemaCrowded optic disc (fellow eye)

Peripapillary

hemorrhages

Nerve fiber layer edema

Obscured borders

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The most common visual field defect is a superior or inferior scotoma

Inferior altitudinal defect

Superior arcuate defect

Combined superior & inferior defect

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NAION is a clinical diagnosis

Elderly patient +/- cardiovascular risk factorsAcute painless optic neuropathy + disc edema + crowded optic disc in fellow eye Rule out arteritic AION Do Humphrey visual fields Imaging is not in indicatedFrequent follow-up

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There is no proven treatment for NAION

IONDT = ION decompression trialA multicenter randomized controlled clinical trialno efficacy for optic nerve fenestrationIntravitreal steroids (triamcinolone acetate)Intravenous noradrenalineWarfarin TPALevodopa + carbidopa

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There is no proven treatment for NAION

Oral prednisone 40-60mg daily – may hasten resolution of disc edemaSome evidence for anti-VEGF intravitreal injections

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Prophylaxis

Control of cardio-vascular risk factorsAspirin 100 mg daily – limited evidence for second eye prophylaxis

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Disc edema resolves in 1 month

Optic atrophy

Optic atrophy with cupping

cup

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Significant improvement is rare

~40% experience partial improvementImprovement may take up to 6 months15% risk for fellow eye involvement in 2 years< 5 % recurrent AION (the same eye)A significant visual field defect persists

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Arteritic ION

And Giant Cell Arteritis (GCA)

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>50% of Arteritic ION are d/t Giant Cell Arteritis

Other etiologies include:Systemic Lupus ErythematosusWegener’s granulomatosisBehcet’s diseaseChurg StraussPolyarteritis Nodosa

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GCA* - key facts

Large vessel vasculitisPredilection for the aortic arch Incidence 20 / 100,000 / year (> age 50) 20% of GCA patients experience severe visual lossAION is the most common ophthalmic manifestation of GCAA-AION is an ophthalmic emergency !

* GCA = Giant Cell Arteritis (Temporal arteritis)

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Arteritic ION presents like any ION, but . . .

75% have typical systemic symptoms 30% have preceding transient visual loss 54% have visual acuity of count-fingers  No light perception>50% second eye ION within hours -weeks

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(“amaurosis fugax”)

(vs

26% in

NAION)

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There are specific funduscopic findings

The involved swollen optic disc is acutely pale

NAION

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There are specific funduscopic findings

Branch Retinal Artery Occlusion

Central Retinal Artery Occlusion

Cherry red spot

Ischemic retina

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There are specific funduscopic findings

Choroidal hypoperfusion indicates multifocal ischemia on Fluorescein angiography

n

ormal choroid

Lack of

choroidal

perfusion

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The workup of suspected Arteritic ION

GCA Symptoms / signs ?

Do blood tests

but

ESR, CRP,

Hb, PLT, Fibrinogen

Urgent TAB*

TAB* in 1 w

Iv Solomedrol  Prednisone + aspirin

yes

no

IV

Solomedrol

 Prednisone + aspirin

until biopsy results

high

normal

NAION

* TAB = Temporal Artery Biopsy

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“Ophthalmic GCA” should be treated with IV steroids

Few studies evaluated treatment protocolsStudies in ophthalmology differ from rheumatology We recommend:IV methylprednisolone 1000mg/d for 3 days followed by a very slow taper of oral prednisone Aspirin 100mg dailyRheumatology consultation & follow-up

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Perioperative ION

(post operative AION and PION)

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ION is a rare surgical complication

ION is an uncommon but devastating complication after various types of surgeriesIntraocular surgeriesIntraocular injectionsNon-ocular surgeries ION may also occur after:renal dialysiscardiac catheterization

d/t Elevated intraocular pressure

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ION may complicate non-ocular surgeries

The 2 most “classic” are : CABGSpinal surgery Commonly bilateral There is often profound visual lossVisual loss may be immediate or delayed (days)

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(mostly AION, 0.06%)

(mostly

PION

,

0.2%)

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The differential diagnosis of post-operative visual loss includes

Ischemic optic neuropathyRetinal artery occlusionAngle closure glaucoma

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Unresponsive mid-dilated pupil

Hazy cornea

Red “angry” eye

Cherry red spot

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The differential diagnosis of post-operative visual loss includes

Cortical blindness Corneal erosion

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Epithelial

irregularity

Bilateral occipital stroke

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There is no prospective / controlled data regarding perioperative ION

Risk factors:Obesity Male genderProlonged surgical timeSurgery in the prone positionLarge fluid shifts / severe blood loss

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There is no effective treatment

Prognosis is poor – significant improvement in minority of patientsShould correct anemia, saturation & hypotension to improve perfusionNo evidence for efficacy of :AspirinAnti - coagulants ThrombolyticsAnti-glaucoma drops

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RON

(Radiation Optic Neuropathy)

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RON is a late complication

Prevalence ~ 0.5% Mean interval 18 monthsThe optic nerves must be in the radiation field

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(range: 3 months – 9 years)

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The risk factors are:

Radiation dosage AgeDiabetes mellitusPresence of compressive optic neuropathyConcomitant chemotherapyPrevious radiotherapy Multiple sclerosis

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(>total 50

Gy

or single dose > 10

Gy

)

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RON mostly presents as PION

May be monocular or binocular45% have visual acuity of no light perceptionDiagnosis is one of exclusion:Suspected Optic neuropathyPMH of radiotherapyNo other obvious explanationOptic nerve enhancement on MRI

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Isolated enhancement on MRI

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optic nerve enhancement

T1W with fat suppression + gadolinium

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There are few treatment options

Oral corticosteroids (prednisone 1mg/kg)Anticoagulants (heparin)AspirinHyperbaric oxygen (30-60min/day x 14-30 days)Intravenous Bevacizumab (2-4 cycles every 2 weeks)

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Suspected RON ?

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Onset < 48-72 hours ?

yes

VEP

Brain+orbits

MRI with gadolinium

normal

abnormal

Hyperbaric oxygen

Look for other etiologies

PO prednisone

Consider IV

Bevacizumab

Enhancement ?

yes

Other optic neuropathy

no

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Prognosis of RON is poor

Spontaneous recovery is rareTreatment is mostly ineffective85% visual acuity ≤ 20/200Optic atrophy appear in 6-8 weeksEnhancement on MRI resolves after several months

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Conclusions

(the “take home massage”)

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ION is an ophthalmic emergency

 Patients with GCA+ION are in danger of catastrophic, irreversible, bilateral blindness that may be prevented by prompt treatment with corticosteroidsAny patient > 50 presenting with ION  an immediate workup to rule out GCA

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ION is not “another type of CVA”

Although considered a “stroke of the optic nerve” and shares many risk factors with cerebrovascular disease, It cannot be directly compared to cerebral infarction, and therefore the evaluation should not be similar to that of cerebral infarction.

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There is no effective treatment for ION

there are no class I studies showing benefit from any medical or surgical treatments

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Steroids

Aspirin

Anti VEGF

Decompression surgery

Hyperbaric oxygen

Levodopa

Erythropoietin

Noradrenalin

Heparin

TPA

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Limited efficacy for prophylaxis

Aspirin 100mg dailyControl of cardiovascular risk factorssuspect GCA !!!Avoid prolonged surgical time and dramatic shifts in body perfusion during surgrey Consider routine serial brain MRIs after brain radiotherapy to detect RON early

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Thank you

For listening

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Acknowledgments

Based on the chapter: Optic nerve: Ischemic.Bialer OY, Bruce BB, Biousse V, Newman NJ.Oxford textbook in Neuro-ophthalmologyOxford textbook in clinical neurologyEditor: Bremner F. Publisher: Oxford University PressGratitude to : Dr. Karin Mimoni Dr. Hadas Kalish-Stiebel Dr. Beau B. Bruce Dr. Nancy J. Newman Dr. Valérie Biousse

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Visit my website to download the presentation: www.dr-bialer.com

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