Ischemic Optic Neuropathy

Dr. Sriniwas Atal

MD Resident

Ophthalmology
BPKLCOS, IOM
 LAYOUT
 INTRODUCTION

 ANTERIOR ISCHEMIC OPTIC NEUROPATHY

 BLOOD SUPPLY OF OPTIC NERVE HEAD

 NA-AION

 A-AION

 PION

 Case Report

 REFERENCES
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 Introduction
● Acute, painless optic neuropathy due to circulatory
insufficiency.

● > 50 years of age

● Most frequently at the optic nerve head (intraocular

insufficiency), Anterior Ischemic Optic Neuropathy (AION)

● Less frequently behind the optic neve head (intraorbital

insufficiency), Posterior Ischemic Optic Neuropathy (PION)

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 Anterior Ischemic Optic Neuropathy
● Most common cause of acute optic neuropathy in older age
groups.

● Rapid onset of painless, unilateral visual loss.

● Decreased visual acuity, visual field, or both.

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1. Non-Arteritic Anterior Ischemic Optic Neuropathy
(NAAION)

2. Arteritic Anterior Ischemic Optic Neuropathy (AAION)

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 Non-arteritic Anterior Ischemic Neuropathy
(NAAION)
● 90-95% of AION

● Mean age - 60 yrs

● Less severe visual loss compared to Arteritic AION.

● Arcuate and altitudinal visual field defects.

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 RISK FACTORS
● Crowded optic disc
● “Disc at risk”
● Small optic disc plus small or absent cup
● Axonal crowding and compression

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Blood supply

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 Blood flow
Theblood flow in the ONH is calculated by using the following
formula (Hayreh et al):

Perfusion pressure = Mean BP – Intraocular pressure (IOP)

(Mean BP = Diastolic BP + 1/3 Pulse pressure)

From this formula, it is evident that the blood flow depends upon
(a) Resistance to blood flow, (b) BP and (c) IOP.

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 Autoregulation
● Helps compensate for any decrease in the blood flow.

● Operates only over a critical range of perfusion pressure.

● Rise or fall of perfusion pressure beyond the critical range

leads to autoregulation failure.

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 RISK FACTOR-MULTIFACTORIAL

HTN/DM

ABSENT CUP

H’RGIC
CARDIAC DS
SHOCK
H’RGIC SHOCK

SMALL
OCULAR RAISED
CUP
IOP
SYSTEMIC
COLLAGEN
VASULAR DS VASOSPASTIC
DISORDER PROLONG
PRESSURE
COLLAGEN ON EYEBALL
VASOSPASTIC
VASCULAR
DISORDER
DS

THYROID DS

 Noctural arterial hypotension play very important role

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Drugs - Sildenafil, Amiodarone, Cocaine
Pathophysiology

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 Clinical findings
● Typically presents as acute painless monocular loss of
visual acuity or visual field, or both, frequently upon
awakening.

● A relative afferent pupillary defect.

● Normal anterior segment.

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● Optic disc edema (sectoral or entire disc)

● Nerve fibre layer hemorrhages associated with the disc

edema

● Crowded optic disc in fellow eye.

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Visual Field Defects

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 Differential Diagnosis
● Arteritic AION

● Optic Neuritis

● Infiltrative Optic Neuropathy

● Compressive Optic Neuropathy

● Diabetic Papillopathy

● Neuroretinitis

● Disk swelling associated with retinal disorders, such as

central retinal vein occlusion (CRVO).
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 INVESTIGATIONS
● Routine investigations

● Lipid profile

● Cardiac evaluation

● ESR (rule out AAION)

● MRI (rule out Optic Neuritis, Compressive ON)

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 Treatment
● None proven

● Aspirin

● Levodopa or carbidopa

● Hyperbaric O2

● Topical - Brimonidine

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 Surgical treatment
● Ischemic Optic Nerve Decompression Trial (IONDT)

● Optic nerve sheath fenestration, with drainage of perineural

subarachnoid cerebrospinal fluid.

● No effect of surgery on visual outcome.

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 Prognosis

● Improvement may take up to 6 months

● ~15% risk for fellow eye involvement in 2 years
● < 5 % risk for recurrent AION (the same eye)
● A significant visual field defect persists
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 Goal of Management
● Eliminating other potential treatable optic neuropathies such
as optic neuritis or a compressive optic neuropathy.

● Identifying any potential modifiable vascular risk factors.

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 Arteritic Anterior Ischemic Optic Neuropathy
● 5-10% of AION

● Short posterior ciliary artery vasculitis Infarction of

optic nerve head

● Commonly seen in elderly, mean age 70 yrs

● Severe visual loss in majority (<6/60)

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● >50% caused by Giant Cell Arteritis (GCA)

● Other etiologies include:

Systemic Lupus Erythematosus
Wegener’s Granulomatosis
Behçet's disease
Churg Strauss Syndrome
Polyarteritis Nodosa

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 Giant Cell Arteritis
● Granulomatous necrotizing arteritis

● Mainly involves large and medium-size arteries :

Major aortic branches
Superficial temporal artery(STA)
Ophthalmic artery
Posterior ciliary arteries
Proximal vertebral arteries.

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● Headache (most common)

● Jaw claudication (pathognomic)

● Temporal artery or scalp tenderness

● Malaise, Anorexia, Weight loss, Fever

● Superficial temporal arteritis is characterized by pulseless,

thickened, tender, inflamed and nodular arteries.

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● 20% of GCA patients experience severe visual loss

● AION is the most common ophthalmic manifestation of GCA

● 30% have preceding transient visual loss (amaurosis fugax)

● 54% have visual acuity of counting fingers - No perception of

light

● >50% second eye involved within hours - weeks

● When temporal arteritis is suspected as the cause of visual loss,

treatment should not be delayed for results of laboratory
investigations or biopsy to prevent blindness in fellow eye.
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 Fundus Examination
● Optic disc pallor (pallid, chalky optic disc edema)

● Choroidal ischemia may be seen - peripapillary pallor and

edema deep to retina

● Disc of fellow eye is normal

● Cotton wool spots

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● CRAO

● Anterior Ischaemic syndrome

● Ophthalmic artery occlusion

● Delay in choroidal perfusion or non-perfusion

● PION can occur

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 Fluorescein fundus angiography
In early stage shows filling defect in optic disc, peripapillary choroid or
choroidal watershed area 31
 INVESTIGATIONS
● Westergren sedimentation rate

● C-reactive protein (reliable indicator than ESR)

● Complete blood count

● Fluorescein angiography

● Temporal artery biopsy - ‘gold standard’

2-3 cm specimen taken to avoid skip lesion

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 Treatment
● Steroids

● Methotrexate

● Aspirin

● Rheumatology consultation & follow-up

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 Regimen
● IV Methylprednisolone 1 g/day for 3 days.

● Followed by oral Prednisolone 1-2mg/kg/day for 3 days.

● Then 50-60mg/day (not less than 0.75mg/kg/day) for 4 weeks

until symptom resolution and ESR/CRP normalisation.

● Then tapering by 10mg/day every 2 weeks until 20mg/kg.

● Tapering then titrated against ESR/CRP and symptoms.

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 Posterior Ischemic Optic Neuropathy(PION)
● Uncommon

● No disc edema

● The mechanism of ischemia is presumed to be insufficiency

of pial arterial vascular supply to the optic nerve distal to the
optic nerve head.

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 Diagnosis

● Rule out other types of retrobulbar optic neuropathies,

including traumatic, infiltrative, inflammatory, toxic,
radiation, and compressive types.

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 CLINICAL FEATURES
● Sudden, painless unilateral or bilateral vision loss.

● Relative afferent pupillary defect in unilateral cases as well as

in bilateral cases when the degree of vision loss is unequal
between the two eyes.

● Normal disc at presentation.

● Optic disc atrophy ensues in 4-6 weeks.

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 Subtypes (Sadda et al)
1. Arteritic PION (associated with temporal arteritis)

2. Non-arteritic PION

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 Subtypes

• Within hours to days

PERIOPERATIVE
• Mainly spinal, cardiac bypass
surgery
• Associated with GCA
ARTERITIC • Poor visual prognosis

• Same risk factor as NAION

NONARTERITIC • Not associated with crowded optic
disc

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 Pathogenesis

HYPOTENSION
LEADS TO
DECREASED
PERFUSION
DECREASED PRESSURE
OXYGEN INCREASED
CARRYING RESISTANCE TO
CAPACITY BLOOD FLOW

PION

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 Non-arteritic PION
● Occurs following a variety of surgical procedures or is
associated with systemic vascular disease.

● Spinal surgery, cardiac bypass surgery and heart or lung

transplantation surgery.

● Intraoperative risk factors -

hypotension, hypovolemia, hypoxia, anemia, and blood loss

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 INVESTIGATIONS
● Routine investigations

● ESR

● CT/MRI

● VEP - shows decreased amplitude

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 TREATMENT
● No effective treatment

● Steroid

● Aspirin

● IOP lowering agents

● Hemodynamic correction

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 Case of A-AION
 A 77-year-old Chinese man complained of bilateral,
simultaneous onset vision loss for 5 days, accompanied by
severe headache on right side and jaw pain. The visual acuities
were no light perception in both eyes.The bilateral superficial
temporal arteries were palpable and tenderness.

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Fundus photographs at presentation of the patient showing severe
bilateral optic disc swollen, with “chalky white” pallid appearance;
there are splinter hemorrhage and cotton wool spots on the retina in the
right eye. The choroid showing diffused atrophy around the optic disc
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The orbital fat-suppression T1-weighted magnetic resonance imaging with contract
showing the enhancement of the optic nerve sheath
in the right eye, (a):axial and (b): sagittal. The white arrows indicate the optic nerve
sheath; the MRA of the cerebral vascular is unremarkable (c)

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 The right temporal artery biopsy was performed and revealed
the occlusion of the luminal owing to the intimal proliferation
and infiltration

 Methylprednisolone 1 g/d was intravenousfor 3 days followed

by prednisone 1 mg/kg/d.

 The vision acuity maintained NLP bilateral after treatment,

whereas the headache and jaw pain disappeared. The oral
prednisone was weaned and methotrexate was added as the
immunosuppressive agent for long treatment.

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 Take Home Message
● 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.

● Thus, any patient > 50 presenting with ION an immediate

workup to rule out GCA.

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● There is no effective treatment for ION.

● 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.

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 References
 Walsh and Hoyt's Clinical Neuro-ophthalmology, 6th edition.
Volume 3.
 Myron Yanoff and Jay S. Duker. Ophthalmology, 5th edition
2019. Elsevier.
 Albert and Jakobiec’s, Miller, Azar, Bloo Principle and Practice
of Ophthalmology. Volume III. Elsevier.
 AAO. Basic and clinical science course. Neuro-
ophthalmology .Volume 5, 2019-20
 Tian et al. BMC Ophthalmology (2018) 18:282

https://doi.org/10.1186/s12886-018-0953-5

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