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Ophthalmology & Neuro-ophthalmology Dr. Omer Y. Bialer 1 Slide 2 Disclosure No conflict of interests I have nothing to disclose ION = Ischemic Optic Neuropathy 2 Slide 3 Presentations outline Introduction Terminology and Nosology Nonarteritic anterior ischemic optic neuropathy Arteritic ION Perioperative ION Radiation optic neuropathy Take home massage summary 3 Slide 4 Introduction ION is the most common acute optic neuropathy > age 50 2 nd most common optic neuropathy after glaucoma Relatively common neuro-ophthalmological disorder Visual loss is often severe No effective treatment or prevention 4 Slide 5 Introduction ION is due to: poor blood flow to the optic nerve Acute occlusion of the feeding arteries Short posterior ciliary arteries Ophthalmic artery 5 Slide 6 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 Arteri t ic Anterior ION (AAION) with swollen optic disc 6 Slide 7 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 Arteri t ic Anterior ION (AAION) with swollen optic disc 7 Idiopathic ION Radiation optic neuropathy Perioperative ION GCA Other vasculitides Slide 8 (Nonarteritic Anterior Ischemic Optic Neuropathy) 8 Slide 9 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 9 Slide 10 The most important risk factor is a crowded optic disc disc at risk = small optic disc + minimal cup crowded normalglaucoma 10 Slide 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 11 Slide 12 Several meds are associated with NAION Erectile dysfunction drugs Amiodarone Vasoconstrictors Cocaine 12 (e.g. Viagra, Cialis) (e.g. nasal decongestants) Slide 13 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 13 Slide 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 Nerve fiber layer edema Obscured borders 14 Slide 15 The most common visual field defect is a superior or inferior scotoma Inferior altitudinal defect Superior arcuate defect Combined superior & inferior defect 15 Slide 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 16 Slide 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 17 Slide 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 18 Slide 19 Prophylaxis Control of cardio-vascular risk factors Aspirin 100 mg daily limited evidence for second eye prophylaxis 19 Slide 20 Disc edema resolves in 1 month Optic atrophy Optic atrophy with cupping cup 20 Slide 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 21 Slide 22 And Giant Cell Arteritis (GCA) 22 Slide 23 >50% of Arteritic ION are d/t Giant Cell Arteritis Other etiologies include: Systemic Lupus Erythematosus Wegeners granulomatosis Behcets disease Churg Strauss Polyarteritis Nodosa 23 Slide 24 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) 24 Slide 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 25 (amaurosis fugax) (vs 26% in NAION) Slide 26 There are specific funduscopic findings The involved swollen optic disc is acutely pale NAION 26 Slide 27 There are specific funduscopic findings Branch Retinal Artery Occlusion Central Retinal Artery Occlusion Cherry red spot Ischemic retina 27 Slide 28 There are specific funduscopic findings Choroidal hypoperfusion indicates multifocal ischemia on Fluorescein angiography normal choroidLack of choroidal perfusion 28 Slide 29 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 yesno IV Solomedrol Prednisone + aspirin until biopsy results highnormal NAION * TAB = Temporal Artery Biopsy 29 Slide 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 30 Slide 31 (post operative AION and PION) 31 Slide 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 32 Slide 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) 33 (mostly AION, 0.06%) (mostly PION, 0.2%) Slide 34 The differential diagnosis of post- operative visual loss includes Ischemic optic neuropathy Retinal artery occlusion Angle closure glaucoma 34 Unresponsive mid-dilated pupil Hazy cornea Red angry eye Cherry red spot Slide 35 The differential diagnosis of post- operative visual loss includes Cortical blindness Corneal erosion 35 Epithelial irregularity Bilateral occipital stroke Slide 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 36 Slide 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 37 Slide 38 (Radiation Optic Neuropathy) 38 Slide 39 RON is a late complication Prevalence ~ 0.5% Mean interval 18 months The optic nerves must be in the radiation field 39 (range: 3 months 9 years) Slide 40 The risk factors are: Radiation dosage Age Diabetes mellitus Presence of compressive optic neuropathy Concomitant chemotherapy Previous radiotherapy Multiple sclerosis 40 (>total 50 Gy or single dose > 10 Gy) Slide 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 41 Slide 42 Isolated enhancement on MRI 42 optic nerve enhancement T1W with fat suppression + gadolinium Slide 43 There are few treatment options 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) 43 Slide 44 Suspected RON ? 44 Onset < 48-72 hours ? yes VEP Brain+orbits MRI with gadolinium normalabnormal Hyperbaric oxygen Look for other etiologies PO prednisone Consider IV Bevacizumab Enhancement ? yes Other optic neuropathy no Slide 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 45 Slide 46 (the take home massage) 46 Slide 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 47 Slide 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. 48 Slide 49 There is no effective treatment for ION there are no class I studies showing benefit from any medical or surgical treatments 49 Steroids Aspirin Anti VEGF Decompression surgery Hyperbaric oxygen Levodopa Erythropoietin Noradrenalin Heparin TPA Slide 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 50 Slide 51 For listening 51 Slide 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. Valrie Biousse 52 Slide 53 Visit my website to download the presentation: www.dr-bialer.com 53