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Ischemic Optic Neuropathy. Ophthalmology & Neuro-ophthalmology Dr. Omer Y. Bialer. Disclosure. No conflict of interests I have nothing to disclose ION = I schemic O ptic Neuropathy. Presentation’s outline. Introduction Terminology and Nosology

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Ischemic optic neuropathy

Ischemic Optic Neuropathy

Ophthalmology & Neuro-ophthalmology

Dr. Omer Y. Bialer


Disclosure
Disclosure

  • No conflict of interests

  • I have nothing to disclose

    ION = Ischemic Optic Neuropathy


Presentation s outline
Presentation’s outline

  • Introduction

  • Terminology and Nosology

  • Nonarteritic anterior ischemic optic neuropathy

  • Arteritic ION

  • Perioperative ION

  • Radiation optic neuropathy

  • “Take home massage” summary


Introduction
Introduction

  • ION is the most common acute optic neuropathy > age 50

  • 2nd most common optic neuropathy after glaucoma

  • Relatively common neuro-ophthalmological disorder

  • Visual loss is often severe

  • No effective treatment or prevention


Introduction1
Introduction

  • ION is due to:

    • poor blood flow to the optic nerve

    • Acute occlusion of the feeding arteries

Ophthalmic artery

Short posterior ciliary arteries


Terminology nosology
Terminology & Nosology

ION

Nonarteritic ION (cardiovascular risk factors)

Arteritic ION

(vasculitis)

NonarteriticAnterior ION (NAION)

with swollen optic disc

NonarteriticPosterior ION (NA-PION) with normal optic disc

Arteritic Anterior ION (AAION)

with swollen optic disc

Arteritic Posterior ION (APION)

with normal optic disc


Terminology nosology1
Terminology & Nosology

ION

Nonarteritic ION (cardiovascular risk factors)

Arteritic ION

(vasculitis)

NonarteriticAnterior ION (NAION)

with swollen optic disc

NonarteriticPosterior ION (NA-PION) with normal optic disc

Arteritic Anterior ION (AAION)

with swollen optic disc

Arteritic Posterior ION (APION)

with normal optic disc

GCA

Other vasculitides

Idiopathic ION

Perioperative ION

Radiation optic neuropathy


Naion
NAION

(Nonarteritic Anterior Ischemic Optic Neuropathy)


Naion is the most common ion
NAION is the most common ION

  • ~ 90% of ION

  • Incidence: 1 / 10,000 / year (> 50 y.o)

    0.5/ 100,000 / year (overall)

  • Mean age at onset 57-65

  • Presentation: acute painless monocular

    visual field loss ± visual acuity loss


The most important risk factor is a crowded optic disc
The most important risk factor is a crowded optic disc

  • “disc at risk” =

    small optic disc + minimal cup

crowded

normal

glaucoma


More risk factors for naion
More risk factorsfor NAION

  • Hypertension (50%)

  • Diabetes mellitus (25%)

  • Obstructive sleep apnea (55%)

  • Hyperlipidemia

  • Ischemic heart disease

  • Obesity

  • Tobacco use

  • High intraocular pressure


Several meds are associated with naion
Several meds are associated with NAION

  • Erectile dysfunction drugs

  • Amiodarone

  • Vasoconstrictors

  • Cocaine

(e.g. Viagra, Cialis)

(e.g. nasal decongestants)


The pathogenesis of naion differs from ihd or cva
The pathogenesis of NAION differs from IHD or CVA

Edema of optic disc

Cardiovascular risk factors

decrease in blood flow

Compression of axons and blood vessels

Crowded optic disc

Blockage of axonal flow

Necrosis and demyelination of nerve fibers


Eye exam
Eye Exam

  • visual acuity & color vision can be normal

  • A relative afferent pupillary defect

  • Normal anterior segment

  • Optic disc edema

  • Crowded optic disc

    (fellow eye)

Peripapillary hemorrhages

Obscured borders

Nerve fiber layer edema


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

Combined superior & inferior defect

Inferior altitudinal defect

Superior arcuate defect


Naion is a clinical diagnosis
NAION is a clinical diagnosis inferior scotoma

  • Elderly patient +/- cardiovascular risk factors

  • Acute painless optic neuropathy

    + disc edema

    + crowded optic disc in fellow eye

  • Rule out arteritic AION

  • Do Humphrey visual fields

  • Imaging is not in indicated

  • Frequent follow-up


There is no proven treatment for naion
There is no proven treatment for NAION inferior scotoma

  • IONDT = ION decompression trial

    • A multicenter randomized controlled clinical trial

    • no efficacy for optic nerve fenestration

  • Intravitreal steroids (triamcinolone acetate)

  • Intravenous noradrenaline

  • Warfarin

  • TPA

  • Levodopa + carbidopa


There is no proven treatment for naion1
There is no proven treatment for NAION inferior scotoma

  • Oral prednisone 40-60mg daily –

    may hasten resolution of disc edema

  • Some evidence for anti-VEGF

    intravitrealinjections


P rophylaxis
P inferior scotomarophylaxis

  • Control of cardio-vascular risk factors

  • Aspirin 100 mg daily – limited evidence

    for second eye prophylaxis


Disc edema resolves in 1 month
Disc edema resolves in 1 month inferior scotoma

cup

Optic atrophy with cupping

Optic atrophy


Significant improvement is rare
Significant improvement is rare inferior scotoma

  • ~40% experience partial improvement

  • Improvement may take up to 6 months

  • 15% risk for fellow eye involvement in 2 years

  • < 5 % recurrent AION (the same eye)

  • A significant visual field defect persists


Arteritic ion
Arteritic ION inferior scotoma

And Giant Cell Arteritis (GCA)


50 of arteritic ion are d t giant cell arteritis
>50% of Arteritic ION are d/t Giant Cell Arteritis inferior scotoma

  • Other etiologies include:

    • Systemic Lupus Erythematosus

    • Wegener’s granulomatosis

    • Behcet’s disease

    • Churg Strauss

    • PolyarteritisNodosa


Gca key facts
GCA inferior scotoma* - key facts

  • Large vessel vasculitis

  • Predilection for the aortic arch

  • Incidence 20 / 100,000 / year (> age 50)

  • 20% of GCA patients experience severe visual loss

  • AION is the most common ophthalmic manifestation of GCA

  • A-AION is an ophthalmic emergency !

* GCA = Giant Cell Arteritis (Temporal arteritis)


Arteritic ion presents like any ion but
Arteritic ION presents like any ION, but . . . inferior scotoma

  • 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

(“amaurosisfugax”)

(vs 26% in NAION)


There are specific funduscopic findings
There are specific funduscopic findings inferior scotoma

The involved swollen optic disc is acutely pale

NAION


There are specific funduscopic findings1
There are specific funduscopic findings inferior scotoma

Ischemic retina

Cherry red spot

Branch Retinal Artery Occlusion

Central Retinal Artery Occlusion


There are specific funduscopic findings2
There are specific funduscopic findings inferior scotoma

Lack of choroidal perfusion

normal choroid

Choroidalhypoperfusion indicates multifocal ischemia on Fluorescein angiography


The workup of suspected arteritic ion
The workup of suspected Arteritic ION inferior scotoma

GCA Symptoms / signs ?

Do blood tests but

yes

no

ESR, CRP, Hb, PLT, Fibrinogen

IV Solomedrol Prednisone + aspirin

until biopsy results

Iv Solomedrol Prednisone + aspirin

NAION

high

normal

Urgent TAB*

TAB* in 1 w

* TAB = Temporal Artery Biopsy


Ophthalmic gca should be treated with iv steroids
“Ophthalmic GCA” should be treated with IV steroids inferior scotoma

  • Few studies evaluated treatment protocols

  • Studies 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 daily

    • Rheumatology consultation & follow-up


Perioperative ion
Perioperative ION inferior scotoma

(post operative AION and PION)


Ion is a rare surgical complication
ION is a rare surgical complication inferior scotoma

  • ION is an uncommon but devastating complication after various types of surgeries

    • Intraocular surgeries

    • Intraocular injections

    • Non-ocular surgeries

  • ION may also occur after:

    • renal dialysis

    • cardiac catheterization

d/t Elevated intraocular pressure


Ion may complicate non ocular surgeries
ION may complicate non-ocular surgeries inferior scotoma

  • The 2 most “classic” are :

    • CABG

    • Spinal surgery

  • Commonly bilateral

  • There is often profound visual loss

  • Visual loss may be immediate or delayed (days)

(mostly AION, 0.06%)

(mostly PION, 0.2%)


The differential diagnosis of post operative visual loss includes
The differential diagnosis of post-operative visual loss includes

  • Ischemic optic neuropathy

  • Retinal artery occlusion

  • Angle closure glaucoma

Cherry red spot

Hazy cornea

Unresponsive mid-dilated pupil

Red “angry” eye


The differential diagnosis of post operative visual loss includes1
The differential diagnosis of post-operative visual loss includes

  • Cortical blindness

  • Corneal erosion

Bilateral occipital stroke

Epithelial

irregularity


There is no prospective controlled data regarding perioperative ion
There is no prospective / controlled data regarding perioperative ION

  • Risk factors:

    • Obesity

    • Male gender

    • Prolonged surgical time

    • Surgery in the prone position

    • Large fluid shifts / severe blood loss


There is no effective treatment
There is no effective treatment perioperative ION

  • Prognosis is poor – significant improvement in minority of patients

  • Should correct anemia, saturation & hypotension to improve perfusion

  • No evidence for efficacy of :

    • Aspirin

    • Anti - coagulants

    • Thrombolytics

    • Anti-glaucoma drops


RON perioperative ION

(Radiation Optic Neuropathy)


Ron is a late complication
RON is a late complication perioperative ION

  • Prevalence ~ 0.5%

  • Mean interval 18 months

  • The optic nerves must be in the radiation field

  • (range: 3 months – 9 years)


The risk factors are
The risk factors are: perioperative ION

  • Radiation dosage

  • Age

  • Diabetes mellitus

  • Presence of compressive optic neuropathy

  • Concomitant chemotherapy

  • Previous radiotherapy

  • Multiple sclerosis

  • (>total 50 Gy or single dose > 10 Gy)


Ron mostly presents as pion
RON mostly presents as PION perioperative ION

  • May be monocular or binocular

  • 45% have visual acuity of no light perception

  • Diagnosis is one of exclusion:

    • Suspected Optic neuropathy

    • PMH of radiotherapy

    • No other obvious explanation

    • Optic nerve enhancement on MRI


Isolated enhancement on mri
Isolated enhancement on MRI perioperative ION

optic nerve enhancement

T1W with fat suppression + gadolinium


There are few treatment options
There are few treatment options perioperative ION

  • Oral corticosteroids (prednisone 1mg/kg)

  • Anticoagulants (heparin)

  • Aspirin

  • Hyperbaric oxygen (30-60min/day x 14-30 days)

  • Intravenous Bevacizumab (2-4 cycles every 2 weeks)


Suspected ron
Suspected RON ? perioperative ION

Onset < 48-72 hours ?

yes

no

VEP

Look for other etiologies

Brain+orbits MRI with gadolinium

normal

abnormal

Hyperbaric oxygen

yes

PO prednisone

Enhancement ?

Consider IV Bevacizumab

Other optic neuropathy


Prognosis of ron is poor
Prognosis of RON is poor perioperative ION

  • Spontaneous recovery is rare

  • Treatment is mostly ineffective

  • 85% visual acuity ≤ 20/200

  • Optic atrophy appear in 6-8 weeks

  • Enhancement on MRI resolves after several months


Conclusions
Conclusions perioperative ION

(the “take home massage”)


Ion is an ophthalmic emergency
ION is an ophthalmic emergency perioperative ION

  • Patients with GCA+ION are in danger of catastrophic, irreversible, bilateral blindness that may be prevented by prompt treatment with corticosteroids

  • Any patient > 50 presenting with ION 

    an immediate workup to rule out GCA


Ion is not another type of cva
ION is not “another type of CVA” perioperative ION

  • 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.


There is no effective treatment for ion
There is no effective treatment for ION perioperative ION

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

TPA

Steroids

Anti VEGF

Heparin

Aspirin

Levodopa

Erythropoietin

Decompression surgery

Noradrenalin

Hyperbaric oxygen


Limited efficacy for prophylaxis
Limited efficacy for prophylaxis perioperative ION

  • Aspirin 100mg daily

  • Control of cardiovascular risk factors

  • suspect 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


Thank you
Thank you perioperative ION

For listening


Acknowledgments
Acknowledgments perioperative ION

  • Based on the chapter:

    Optic nerve: Ischemic.

    Bialer OY, Bruce BB, Biousse V, Newman NJ.

    Oxford textbook in Neuro-ophthalmology

    Oxford textbook in clinical neurology

    Editor: Bremner F.

    Publisher: Oxford University Press

  • Gratitude to : Dr. Karin Mimoni

    Dr. HadasKalish-Stiebel

    Dr. Beau B. Bruce

    Dr. Nancy J. Newman

    Dr. ValérieBiousse



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