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ISCHAEMIC OPTIC NEUROPATHY
HAYREH, S. S. (2009). ISCHEMIC OPTIC NEUROPATHY. PROGRESS IN RETINAL AND EYE
RESEARCH, 28(1), 34-62.
Ade Wijaya, MD – December 2017
INTRODUCTION
 One of the major causes of blindness or seriously
impaired vision among the middle-aged or elderly
population
 The term ION first used at 1974
 Anterior vs posterior
 Most common: NA-AION
ANATOMY (VASCULARIZATION)
• Posterior ciliary
artery (PCA)
• Non-arteritic vs
arteritic
AION
• Multiple sources
non-PCA
PION
ANATOMY
FACTORS INFLUENCING THE BLOOD FLOW IN
THE OPTIC NERVE HEAD
 Blood flow
 Autoregulation
 Arterial blood pressure
 Intraocular pressure
NON-ARTERITIC
ISCHAEMIC OPTIC NEUROPATHY
(NA-ION)
PATHOGENESIS
 Due to transient non-perfusion or hypoperfusion of
the ONH circulation.  most common
 Due to embolic lesions of the arteries/arterioles
feeding the ONH.
PATHOGENESIS
Optic nerve
ischaemia /
hypoxia
Axoplasmic flow
stasis in the
optic nerve
fibers
Axonal swelling
Asymtopmatic
optic disc
edema
compression of
the intervening
capillaries
when there is no cup or
only a small cup, the
swollen axons are
crowded in a restricted
space in
the optic disc
RISK FACTORS
 Predisposing
- systemic
- ocular
 Precipitating  nocturnal arterial hypotension
 Drugs: phosphodiesterase inhibitors, amidarone
 Familial
SYSTEMIC PREDISPOSING RISK FACTORS
 Arterial hypertension
 Nocturnal arterial
hypotension,
 Diabetes mellitus
 Ischemic heart disease
 Hyperlipidemia
 Atherosclerosis
 Arteriosclerosis
 Sleep apnea
 Embolism
 Migraine
 Defective
cardiovascular
autoregulation
 Type A personality
 Carotid dissection
OCULAR PREDISPOSING RISK FACTORS
 Absent or small cup in the optic disc
 Raised IOP
 Optic disc edema
 Location of the watershed zone of the PCAs in
relation to the optic disc
 Vascular disorders
 Optic disc drusen
 Cataract extraction
HISTOPATHOLOGY
EPIDEMIOLOGY
 Incidence: 2.30 – 10,20 per 100,000 population >
50 years old in the US
 White > black or hispanic
 Middle-aged / elderly
 Men > women
SIGNS & SYMPTOMS
 Sudden painless deterioration of vision
 Usually discovered on waking in the morning
 Visual field defects
 Photophobia
 Normal visual acuity doesn’t rule out NA-ION
 Might have improvement up to 6 months
 RAPD
 Acute: disc edema ; Chronic: disc pallor
 Simultaneous bilateral onset of NA-AION is
extremely rare, except in patients who develop
sudden, severe arterial hypotension, e.g. during
hemodi-alysis or surgical shock.
MANAGEMENT
 Optic nerve sheath decompression.
 Aspirin
 Systemic corticosteroid therapy
 Intravitreal triamcynolone
 Intravitreal bevacizumab
 Reduction risk factors
ARTERITIC
ISCHAEMIC OPTIC NEUROPATHY
(A-ION)
PATHOGENESIS
 Causes: mostly GCA, other types of vasculitis, e.g.,
polyarteritis nodosa, systemic lupus erythematosus,
and herpes zoster.
 T-cells dependent disease affecting PCA
 Genetic predisposition  caucasians
 Late middle-aged and elderly
SIGNS & SYMPTOMS
 Amaurosis fugax
 Visual loss
 Visual field defects
 GCA symptoms
 Extraocular motility disorders
 RPD
 Optic disc changes. ODE, compared to NA-AION,
usually has a diagnostic appearance in A-AION, i.e.
chalky white color
 High ESR and CRP
OPTIC DISC APPEARANCES
 Chalky white color
 Retinal cotton wool spots
 CRAO
 Cilioretinal artery occlusion
 Choroidal ischaemic lesions
 Ocular ischaemia
MANAGEMENT
 Management of A-AION is actually management of
GCA
 Prime medical emergency
 STEROID!
POSTERIOR
ISCHAEMIC OPTIC NEUROPATHY
(PION)
INTRODUCTION
 First described by Hayreh at 1981
 Much less common than AION
 A diagnosis of exclusion
 Classification: non-arteritic, arteritic, surgical
 Middle-age / elderly
 Women > men
 Clinical manifestation similar to AION with normal
funduscopic appearance
 In surgical PION  symptoms bilateral
DIAGNOSIS
 Sudden onset of visual deterioration with or without
deterioration of central visual acuity
 Optic nerve related visual field defects
 RAPD
 Normal fundus
 No other ocular, orbital or neurological abnormality
to explain the visual loss;
 Development of optic disc pallor, usually within 6–8
weeks
 Surgical PION: dramatic visual loss noticed as soon
as the patient is alert enough after a major surgical
procedure
MANAGEMENT & PROGNOSIS
 Similar to AION
 Prognosis:
- NA PION  good with steroid
- A PION  steroid could prevent further visual
deterioration
- Surgical PION  poor prognosis
SUMMARY
 Spectrum of several type of condition with its own
etiology, pathogenesis, and management
 Could cause blindness or severe visual impairment
 Therapy during early stage of the diseases might
be beneficial
THANK YOU

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Ischaemic Optic Neuropathy

  • 1. ISCHAEMIC OPTIC NEUROPATHY HAYREH, S. S. (2009). ISCHEMIC OPTIC NEUROPATHY. PROGRESS IN RETINAL AND EYE RESEARCH, 28(1), 34-62. Ade Wijaya, MD – December 2017
  • 2. INTRODUCTION  One of the major causes of blindness or seriously impaired vision among the middle-aged or elderly population  The term ION first used at 1974  Anterior vs posterior  Most common: NA-AION
  • 3. ANATOMY (VASCULARIZATION) • Posterior ciliary artery (PCA) • Non-arteritic vs arteritic AION • Multiple sources non-PCA PION
  • 5. FACTORS INFLUENCING THE BLOOD FLOW IN THE OPTIC NERVE HEAD  Blood flow  Autoregulation  Arterial blood pressure  Intraocular pressure
  • 7. PATHOGENESIS  Due to transient non-perfusion or hypoperfusion of the ONH circulation.  most common  Due to embolic lesions of the arteries/arterioles feeding the ONH.
  • 8. PATHOGENESIS Optic nerve ischaemia / hypoxia Axoplasmic flow stasis in the optic nerve fibers Axonal swelling Asymtopmatic optic disc edema compression of the intervening capillaries when there is no cup or only a small cup, the swollen axons are crowded in a restricted space in the optic disc
  • 9. RISK FACTORS  Predisposing - systemic - ocular  Precipitating  nocturnal arterial hypotension  Drugs: phosphodiesterase inhibitors, amidarone  Familial
  • 10. SYSTEMIC PREDISPOSING RISK FACTORS  Arterial hypertension  Nocturnal arterial hypotension,  Diabetes mellitus  Ischemic heart disease  Hyperlipidemia  Atherosclerosis  Arteriosclerosis  Sleep apnea  Embolism  Migraine  Defective cardiovascular autoregulation  Type A personality  Carotid dissection
  • 11. OCULAR PREDISPOSING RISK FACTORS  Absent or small cup in the optic disc  Raised IOP  Optic disc edema  Location of the watershed zone of the PCAs in relation to the optic disc  Vascular disorders  Optic disc drusen  Cataract extraction
  • 13. EPIDEMIOLOGY  Incidence: 2.30 – 10,20 per 100,000 population > 50 years old in the US  White > black or hispanic  Middle-aged / elderly  Men > women
  • 14. SIGNS & SYMPTOMS  Sudden painless deterioration of vision  Usually discovered on waking in the morning  Visual field defects  Photophobia  Normal visual acuity doesn’t rule out NA-ION  Might have improvement up to 6 months  RAPD  Acute: disc edema ; Chronic: disc pallor  Simultaneous bilateral onset of NA-AION is extremely rare, except in patients who develop sudden, severe arterial hypotension, e.g. during hemodi-alysis or surgical shock.
  • 15. MANAGEMENT  Optic nerve sheath decompression.  Aspirin  Systemic corticosteroid therapy  Intravitreal triamcynolone  Intravitreal bevacizumab  Reduction risk factors
  • 17. PATHOGENESIS  Causes: mostly GCA, other types of vasculitis, e.g., polyarteritis nodosa, systemic lupus erythematosus, and herpes zoster.  T-cells dependent disease affecting PCA  Genetic predisposition  caucasians  Late middle-aged and elderly
  • 18. SIGNS & SYMPTOMS  Amaurosis fugax  Visual loss  Visual field defects  GCA symptoms  Extraocular motility disorders  RPD  Optic disc changes. ODE, compared to NA-AION, usually has a diagnostic appearance in A-AION, i.e. chalky white color  High ESR and CRP
  • 19. OPTIC DISC APPEARANCES  Chalky white color  Retinal cotton wool spots  CRAO  Cilioretinal artery occlusion  Choroidal ischaemic lesions  Ocular ischaemia
  • 20. MANAGEMENT  Management of A-AION is actually management of GCA  Prime medical emergency  STEROID!
  • 21.
  • 23. INTRODUCTION  First described by Hayreh at 1981  Much less common than AION  A diagnosis of exclusion  Classification: non-arteritic, arteritic, surgical  Middle-age / elderly  Women > men  Clinical manifestation similar to AION with normal funduscopic appearance  In surgical PION  symptoms bilateral
  • 24. DIAGNOSIS  Sudden onset of visual deterioration with or without deterioration of central visual acuity  Optic nerve related visual field defects  RAPD  Normal fundus  No other ocular, orbital or neurological abnormality to explain the visual loss;  Development of optic disc pallor, usually within 6–8 weeks  Surgical PION: dramatic visual loss noticed as soon as the patient is alert enough after a major surgical procedure
  • 25. MANAGEMENT & PROGNOSIS  Similar to AION  Prognosis: - NA PION  good with steroid - A PION  steroid could prevent further visual deterioration - Surgical PION  poor prognosis
  • 26. SUMMARY  Spectrum of several type of condition with its own etiology, pathogenesis, and management  Could cause blindness or severe visual impairment  Therapy during early stage of the diseases might be beneficial