Unilateral Optic Disc Swelling: Differential Diagnosis & - - PowerPoint PPT Presentation

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Unilateral Optic Disc Swelling: Differential Diagnosis & - - PowerPoint PPT Presentation

Jan 01, 2024 358 likes •774 views

Unilateral Optic Disc Swelling: Differential Diagnosis & Evaluation Karl C. Golnik, MD, MEd Professor & Chairman Department of Ophthalmology University of Cincinnati & the Cincinnati Eye Institute Objectives List the

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Unilateral Optic Disc Swelling: Differential Diagnosis & Evaluation

Karl C. Golnik, MD, MEd Professor & Chairman Department of Ophthalmology University of Cincinnati & the Cincinnati Eye Institute

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Objectives

  • List the differential diagnosis of

unilateral optic disc swelling.

  • Outline the diagnostic approach to

the patient with unilateral optic disc swelling.

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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

* This does not represent an exhaustive list of causes!

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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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Optic Neuritis

Nonspecific term describing optic nerve involvement by inflammation infection, or demyelination.

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  • Demyelinating, Idiopathic

Typical Optic Neuritis

  • Mild disc swelling (no exudate/hemorrhage)
  • Not steroid dependent

Atypical Optic Neuritis

  • Disc swelling may be severe (with

exudate/hemorrhage)

  • May be steroid dependent
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Inflammatory

  • Neuromyeltis Optica (Devics)
  • Sarcoid
  • Autoimmune Optic Neuropathy (AON)
  • Chronic Relapsing Inflammatory Optic Neuropathy

(CRION)

  • Other systemic (SLE, Wegeners)

Infectious

  • Syphilis, Lyme, Bartonella, TB

Chikungunya virus

Optic Neuritis Associated With Chikungunya Virus Infection in South India. Apoorva M. et al. Arch Ophthalmol 2007; 125:1381.

Atypical Optic Neuritis

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  • VA 20/200 OD

20/20 OS

  • Pupils RAPD OD
  • VF OD shown
  • VF OS normal

Diagnosis? Evaluation?

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spectrum of VF defects in the ONTT

Keltner et al. Arch Ophthalmol 1994;112:946.

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papillitis 35% retrobulbar neuritis 65%

Typical Optic Neuritis

(Demyelinating/Idiopathic)

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ONTT - Visual Outcome

Visual acuity 20/40 or better: placebo 94.3% IV steroids 93.7%

  • ral steroids 92.6%

IV only affects rate of recovery - not final vision!

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ONTT: Development of MS at 15 years

Arch Neurol 2008 65:727.

  • 50 % regardless of MRI
  • 25% if MRI normal at onset
  • 72% if ≥ 1 plaque at onset
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ANN NEUROL 2011; 69:292-302.

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  • VA 20/200 OD

20/20 OS

  • Pupils RAPD OD
  • VF OD shown
  • VF OS normal

Diagnosis? Evaluation?

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Inflammatory

  • Neuromyeltis Optica (Devics)
  • Sarcoid
  • Autoimmune Optic Neuropathy (AON)
  • Chronic Relapsing Inflammatory Optic Neuropathy

(CRION)

  • Other systemic (SLE, Wegeners)

Infectious

  • Syphilis, Lyme, Bartonella, TB

Chikungunya virus

Optic Neuritis Associated With Chikungunya Virus Infection in South India. Apoorva M. et al. Arch Ophthalmol 2007; 125:1381.

Atypical Optic Neuritis

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Atypical Optic Neuritis

  • Important to diagnose because they are

usually responsive to steroids and/or antibiotics and need treatment to improve vision.

  • May also be steroid dependent, relapsing

as steroid dose is reduced sometimes requiring immunosuppression for many years.

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Neuromyelitis Optica (Devic)

  • Idiopathic inflammatory demyelinating CNS

disease

  • Unilateral or Bilateral optic neuritis
  • Transverse myelitis
  • Monophasic or polyphasic course
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Neuromyelitis Optica - Diagnosis

  • Serum Biomarkers – NMO-IgG
  • Targets aquaporin-4, dominant water channel protein
  • Specificity reported 95-100%
  • Diagnostic Criteria:
  • Optic neuritis and acute myelitis + 2/3 of the

following:

  • Spinal cord lesion extending >3 vertebral segments
  • Brain MRI findings not satisfying MS criteria
  • NMO-IgG seropositive

Wingerchuk et al. Neurology 2006; 66(1), 1485-1489

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NMO Treatment

  • IV Corticosteroids
  • Potentially decreases attack severity and speeds recovery
  • Therapeutic Plasmapheresis
  • Effective rescue treatment if steroid unresponsive
  • Chronic Immunosuppressive Treatment (if recurrent)
  • Azathioprine ± prednisone – most common treatment
  • Mitoxantrone
  • Mycophenolate Mofetil
  • Rituximab
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Sarcoid can cause optic neuropathy with or without disc

  • swelling. Check angiotensin converting enzyme, CXR if disc

swelling is more than mild.

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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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Neuroretinitis - optic disc swelling and macular star of exudate. cat scratch disease, idiopathic, toxoplasmosis, tuberculosis, syphillis NOT MS!

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Syphilis Cases

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Infectious Optic Neuropathy

Summary

  • Presents like any subacute/acute optic neuropathy
  • Must know disease prevalence in your region
  • Prognosis probably related to treatment timing
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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Lebers? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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22-yo-wm c/o blurry vision OS x 1 month. VA: 20/15 OD, 20/200 OS, HRR: 10/10 OD, 0/10 OS P: L-RAPD

2 wks later OD 20/200 Lebers Hereditary Optic Neuropathy

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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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Anterior Ischemic Optic Neuropathy (AION)

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Non-Arteritic AION (NAION)

Age, HTN, DM, cholesterol Disc-at-Risk Nocturnal Hypotension? Not carotid stenosis 15% sequential (5yrs) 50% no change 43% improve (some) 7% worsen cup to big

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Giant Cell (Temporal) Arteritis

  • Always need to consider & ask the

right questions:

  • jaw claudication, scalp tenderness,

HA, fatigue

  • Check ESR, CRP, CBC/platelets

pallid swelling suspicious

Arteritic (AAION) vs. Non-Arteritic (NAION)

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AION - Causes

Sildenafil (Viagra)?

Pomeranz et al. J Neuro-Ophthalmol 2005; 25:9-13.

  • 7 patients from 1999-2003 at 1 institution
  • men, age 50-69
  • onset within 36 hrs
  • all had hypertension, dm, hypercholesterolemia
  • 4/7 disc-at-risk, 3/7 not specified
  • 7 other previously reported men, age 48-62
  • onset within 12 hrs, 5/7 with risk factors
  • 3/7 disc-at-risk, 4/7 not specified
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AION - Mimic

Amiodarone? (Cordone,

Pacerone)

From: Chen D, Hedges DR. Sem Ophthalmol 2003;18:169-73. Based

  • n review of 73 patients.
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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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Infiltration

leukemia lymphoma

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Unilateral Optic Disc Swelling*

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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Retinal-Choroidal Collaterals

  • ld CRVO

meningioma COAG idiopathic

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Chorioretinal Folds tumor Graves’ papilledema idiopathic

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Summary - Unilateral Optic Disc Swelling

Inflammation

  • Typical optic neuritis (idiopathic,

demyelinating)

  • Atypical Optic Neuritis

(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)

Infection

  • Syphilis, Lyme, Bartonella, TB, HZV,

CMV, sinusitis

Hereditary – Leber? Ischemia

  • NAION
  • AAION
  • Hypoperfusion (anemia, blood

loss, hypotension, DM)

Infiltration

  • Lymphoma, Leukemia, other

Compression

  • Tumor, Graves, ICP, drusen

Miscellaneous

hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO

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Thank-you for your attention.