Bilateral Optic Disc Edema (Grand Rounds)

From EyeWiki

Bilateral non-arteritic anterior ischemic optic neuropathy (NAION).

Financial Disclosure

  • Dr.Diana Beselga states that she has no financial interest, affiliation, or other relationship with the manufacture of any commercial project discussed or with the manufacture of any competing commercial project.


  • History of Present Illness
    • 46-year-old caucasian male with no complaints, comes to a routine Ophthalmology consultation.
  • Review of Systems: frequent headaches
  • Past Medical History: Ulcerative colitis, Migraine
  • Allergies: None
  • Medications: Mesalazine


  • BCVA: 20/20 bilaterally
  • Pupils: normal pupillary reflexes, no afferent pupillary defect
  • Colour vision: The patient was able to read Ishihara plates with each eye separately and without any errors
  • Tonometry: 18 mmHg RE and 19 mmHg LE
  • Extraocular Motility: without alterations
  • Visual Fields: enlarged blind spot bilaterally
  • External Exam: no alterations
  • Normal blood pressure and pulse
  • Slit Lamp Findings: without conjuntival hyperemia, transparent cornea, no anterior chamber reaction, clear lens bilaterally
  • Dilated Fundus Exam Findings: bilateral papilledema with an hemorrhagic component; macula and peripheral retina without changes


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Laboratory Testing

  • Normal blood count, ESR and CRP. No coagulation abnormalities. Analytical infectious and autoimmune evaluation negative.
  • Lumbar puncture showed no increased fluid pressure and no cytochemical changes.


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Imaging Testing

  • Normal optic nerve/NFL OCT Brain and orbits magnetic resonance without significant changes
  • Carotid Doppler US : without significant stenosis or atherosclerotic disease

Differential Diagnosis

  • Pseudotumor cerebri
  • Mesalazine causing intracranial hypertension
  • Non arteritic anterior ischemic optic neuropathy
  • Infiltrative disease of the optic nerve


  • Bilateral non arteritic anterior ischemic optic neuropathy


Non arteritic anterior ischemic optic neuropathy (NAION)

  • NAION is an infarction of the optic nerve head caused by compromised blood flow in one or more of the posterior ciliary arteries.
  • Bilateral NAION is a well-documented entity that may be seen in 12-19% patients and it usually occurs sequentially instead of simultaneously.
  • Patients with migraine may develop NAAION as early as the second or third decade of life.

  • Vasculitides
    • Polyarteritis nodosa
    • Systemic lupus
    • Buerger disease
    • Allergic vasculitis
    • Postviral vasculitis
    • Postimmunization
    • Syphilis
    • Radiation necrosis
  • Systemic vasculopathies
    • Hypertension
    • Atherosclerosis
    • Diabetes mellitus
    • Migraine
    • Takayasu disease
    • Carotid occlusive disease
  • Hematologic
    • Polycythemia vera
    • Sickle cell disease (trait)
    • Acute hypotension (shock)
    • Glucose-6-phosphate dehydrogenase deficiency (G-6-PD)
  • Ocular
    • Postcataract (possibly)
    • Low-tension glaucoma
  • Medications
    • Ex: phosphodiesterase type 5 (PDE-5) inhibitors, amiodarone


  • Swollen optic disc, either diffuse or sectoral. Flame-shaped hemorrhages are present at or near the optic disc margin.
  • Most patients have either altitudinal or arcuate visual field defects.
  • Relative afferent pupillary defect is invariably present unless coexistent optic neuropathy in the fellow eye balances the impaired pupillary response in the affected eye.


  • Typically presents with a sudden, painless loss of visual accuity.
  • In most patients there are no premonitory symptoms.
  • Approximately 50% of patients have visual acuity better than 20/100, so the presence of normal visual acuity does not rule out NAION

Differential Diagnosis

  • Arteritic anterior ischemic optic neuropathy
  • Optic Neuritis
  • Compressive optic neuropathy
  • Optic nerve inflammation, such as those related to syphilis or sarcoidosis
  • Infiltrative optic neuropathies
  • Idiopathic forms of optic disc edema, which include diabetic papillopathy and papillophlebitis.


  • The treatment of anterior ischemic optic neuropathy to a large degree depends on its etiology in the individual patient.
  • Most of the literature on the treatment of NAION consists of retrospective or prospective case series and anecdotal case reports.
  • Various medical therapies for NAION have been investigated including systemic corticosteroids, anticoagulants, antiplatelet agents, diphenylhydantoin, and levodopa. None of these treatments have been found to be effective.
  • Although numerous practitioners recommend steroids to treat acute NAION, this practice is not based on any level I evidence.

Follow Up

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  • Hayreh SS, Zimmerman MB. Nonarteritic anterior ischemic optic neuropathy: natural history of visual outcome. Ophthalmology 2008; 115:298.
  • Hayreh SS, Zimmerman MB. Optic disc edema in non-arteritic anterior ischemic optic neuropathy. Graefes Arch Clin Exp Ophthalmol 2007; 245:1107.
  • PreechawatP Bruce BB Newman NJ Biousse V. Anterior ischemic optic neuropathy in patients younger than 50 years. Am J Ophthalmol 2007; 144:953.
  • Boone MI, Massry GG, Frankel RA, et al. Visual outcome in bilateral nonarteritic anterior ischemic optic neuropathy. Ophthalmology 1996, 103:1223.
  • Atkins EJ, Bruce BB, Newman NJ, Biousse V. Treatment of nonarteritic anterior ischemic optic neuropathy. Surv Ophthalmol 2010; 55:47. 1996; 103:1223.


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