1. Ischemic Optic Neuropathy
Ophthalmology & Neuro-ophthalmology
Dr. Omer Y. Bialer

2. ION = Ischemic Optic Neuropathy
Disclosure
No conflict of interests
I have nothing to disclose
ION = Ischemic Optic Neuropathy

3. Presentation’s outline
Introduction
Terminology and Nosology
Nonarteritic anterior ischemic optic neuropathy
Arteritic ION
Perioperative ION
Radiation optic neuropathy
“Take home massage” summary
אנחנו נדבר רק על הסטנדרט אוף קר בעינים כיום ולא על התפתחויות חדשות ומחקרים
הוסף SUMMARY

4. 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

5. Introduction ION is due to: poor blood flow to the optic nerve
Acute occlusion of the feeding arteries
Ophthalmic artery
Short posterior ciliary arteries

6. Terminology & Nosology
ION
Nonarteritic ION (cardiovascular risk factors)
Arteritic ION
(vasculitis)
Nonarteritic Anterior ION (NAION)
with swollen optic disc
Nonarteritic Posterior ION (NA-PION) with normal optic disc
Arteritic Anterior ION (AAION)
with swollen optic disc
Arteritic Posterior ION (APION)
with normal optic disc

7. Terminology & Nosology
ION
Nonarteritic ION (cardiovascular risk factors)
Arteritic ION
(vasculitis)
Nonarteritic Anterior ION (NAION)
with swollen optic disc
Nonarteritic Posterior ION (NA-PION) with normal optic disc
Arteritic Anterior ION (AAION)
with swollen optic disc
Arteritic Posterior ION (APION)
with normal optic disc
הוסף ION תחת "אידיופאתי"
Idiopathic ION
Perioperative ION
Radiation optic neuropathy
GCA
Other vasculitides

8. NAION
(Nonarteritic Anterior Ischemic Optic Neuropathy)

9. NAION is the most common ION
~ 90% of ION
Incidence: / 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
Non arteritic posterior ischemic optic neuropathy is exceedingly rare and is a diagnosis of exclusion.

10. The most important risk factor is a crowded optic disc
“disc at risk” =
small optic disc + minimal cup
crowded
normal
glaucoma

11. More risk factors for NAION
Hypertension (50%)
Diabetes mellitus (25%)
Obstructive sleep apnea (55%)
Hyperlipidemia
Ischemic heart disease
Obesity
Tobacco use
High intraocular pressure
למעשה השכיחות של OSA ב NAION כפולה מהאוכלוסיה הכללית.

12. Several meds are associated with NAION
Erectile dysfunction drugs
Amiodarone
Vasoconstrictors
Cocaine
(e.g. Viagra, Cialis)
(e.g. nasal decongestants)

13. 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

14. 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

15. The most common visual field defect is a superior or inferior scotoma
Inferior altitudinal defect
Superior arcuate defect
Combined superior & inferior defect

16. NAION is a clinical diagnosis
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
נבצע גם את השאלון סקירה ל OSA (BERLIN QUESTIONNARE)

17. There is no proven treatment for NAION
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

18. There is no proven treatment for NAION
Oral prednisone 40-60mg daily –
may hasten resolution of disc edema
Some evidence for anti-VEGF
intravitreal injections

19. Prophylaxis Control of cardio-vascular risk factors
Aspirin 100 mg daily – limited evidence
for second eye prophylaxis

20. Disc edema resolves in 1 month
cup
Optic atrophy with cupping
Optic atrophy

21. Significant improvement is rare
~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

22. Arteritic ION
And Giant Cell Arteritis (GCA)

23. >50% of Arteritic ION are d/t Giant Cell Arteritis
Other etiologies include:
Systemic Lupus Erythematosus
Wegener’s granulomatosis
Behcet’s disease
Churg Strauss
Polyarteritis Nodosa

24. * GCA = Giant Cell Arteritis (Temporal arteritis)
GCA* - 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)

25. 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
(“amaurosis fugax”)
(vs 26% in NAION)

26. There are specific funduscopic findings
The involved swollen optic disc is acutely pale
NAION

27. There are specific funduscopic findings
Ischemic retina
Cherry red spot
Branch Retinal Artery Occlusion
Central Retinal Artery Occlusion

28. There are specific funduscopic findings
Lack of choroidal perfusion
normal choroid
Choroidal hypoperfusion indicates multifocal ischemia on Fluorescein angiography

29. The workup of suspected Arteritic ION
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
הרגישות המשולבת של ESR ו CRP היא מעל 97%
בביופסיה חייבים לוודא שנלקח ספסימן מתאים – כלומר באורך 2 ס"מ לפחות , עורק ולא וריד ולא נמעך. במקרים של חשד קליני גבוה נפנה לביופסיה של הצד השני
Urgent TAB*
TAB* in 1 w
* TAB = Temporal Artery Biopsy

30. “Ophthalmic GCA” should be treated with IV steroids
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
Tocilizumab (אנטי IL6) ומתוטרקסט לטיפול ב GCA

31. Perioperative ION
(post operative AION and PION)

32. ION is a rare surgical complication
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

33. ION may complicate non-ocular surgeries
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%)

34. 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

35. The differential diagnosis of post-operative visual loss includes
Cortical blindness
Corneal erosion
Bilateral occipital stroke
למחוק CORNEAL EROSION ולשים PITUITARY APOPLEXY עם תמונת MRI
Epithelial
irregularity

36. 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

37. There is no effective treatment
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
40 חווים איזה שהוא שיפור אך לא משמעותי.
הוספה המילה saturation &
הוספה השורה "anti glaucoma drops"

38. RON
(Radiation Optic Neuropathy)

39. RON is a late complication
Prevalence ~ 0.5%
Mean interval 18 months
The optic nerves must be in the radiation field
(range: 3 months – 9 years)

40. The risk factors are: Radiation dosage Age Diabetes mellitus
Presence of compressive optic neuropathy
Concomitant chemotherapy
Previous radiotherapy
Multiple sclerosis
(>total 50 Gy or single dose > 10 Gy)

41. RON mostly presents as PION
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

42. Isolated enhancement on MRI
optic nerve enhancement
T1W with fat suppression + gadolinium

43. There are few treatment options
Oral corticosteroids (prednisone 1mg/kg)
Anticoagulants (heparin)
Aspirin
Hyperbaric oxygen (30-60min/day x days)
Intravenous Bevacizumab (2-4 cycles every 2 weeks)

44. Suspected RON ? Onset < 48-72 hours ? yes no VEP
Look for other etiologies
Brain+orbits MRI with gadolinium
normal
abnormal
Hyperbaric oxygen
VEP היא בדיקת עזר טובה לאבחן פגיעה בעצב הראיה בשלב מוקדם מאד - אפילו לפני הופעת הירידה בראיה.
האפשרויות כוללות נוגדי קרישה (הפרין) סטרואידים , חמצן היפרבארי ואבסטין סיסטמי
חמצן היפרבארי - יש 27 מקרים מתוארים בספרות – טיפולים בני דקות של חמצן 100% בלחץ 2-3 אטמוספירות. יש להתחיל בטיפול תוך שעות.
yes
PO prednisone
Enhancement ?
Consider IV Bevacizumab
Other optic neuropathy

45. Prognosis of RON is poor
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

46. Conclusions
(the “take home massage”)

47. 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 corticosteroids
Any patient > 50 presenting with ION 
an immediate workup to rule out GCA

48. 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.
Non arteritic anterior ischemic optic neuropathy is not an embolic disorder, but a small vessel disease. Evaluation of the internal carotid artery is not routinely indicated. There is also no definite increased risk of stroke in patients with nonarteritic anterior ischemic optic neuropathy, but vascular risk factors are common and should be controlled.

49. There is no effective treatment for 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
Hyperbaric oxygen
Noradrenalin

50. Limited efficacy for prophylaxis
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

51. Thank you
For listening

52. Acknowledgments 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. Hadas Kalish-Stiebel
Dr. Beau B. Bruce
Dr. Nancy J. Newman
Dr. Valérie Biousse

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