Optic nerve disc edema unilateral

1. DISC EDEMA
D R . B H A R AT H M U T H U T H A N G A M P G
D E P T O F O P H T H A L M O LO GY
G H Q H K A N C H E E P U R A M

2. UNILATERAL DISC
EDEMA
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3. Introduction
The most common causes of unilateral optic disc edema are NAION, optic neuritis, and orbital
compressive lesions.
NAION, the disc typically has an edematous appearance that is often sectoral, and disc hemorrhages
are frequently present.
On the other hand, only one-third of patients with optic neuritis will have optic disc swelling, and,
when present, it is typically mild. Disc hemorrhages are uncommon with optic neuritis.
compressive lesions may lead to chronic disc edema, optociliary collateral vessels, and glistening
white bodies on the disc surface (pseudodrusen from extruded axoplasm).
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4. Vision loss generally is slowly progressive in patients with compressive lesions.
It is rapidly progressive with subsequent improvement in those with optic neuritis.
It is most often maximal at onset with minimal improvement in patients with NAION.
Both optic neuritis and compressive lesions generally produce central visual loss, whereas NAION
typically produces a nerve fiber bundle-type field defect
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5. Optic neuritis
Typical optic neuritis is an inflammatory optic neuropathy caused by demyelinating disease.
Visual loss in the affected eye typically occurs rapidly over several hours to a few days. Decreased
color vision and contrast sensitivity are characteristic.
Pain with eye movements precedes the vision loss in approximately 90% of cases.
The pain typically lasts 3–5 days; if it persists for longer than 7 days, optic neuritis should be
considered less likely.
Visual field defects commonly are present; they can be either diffuse or discrete scotomas and are
non- specific.
Fundus examination shows mild disc edema in approximately one-third of affected eyes, which is
typically less prominent than the disc swelling associated with papilledema.
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7. Typical optic neuritis
prognosis for recovery of vision generally is good but is in relation to the severity of the initial deficit.
Recovery typically begins within 1 month.
The likelihood of progression of optic neuritis to MS is best predicted by brain magnetic resonance
imaging (MRI) at the time of diagnosis.
In the Optic Neuritis Treatment Trial, the risk of developing MS within 15 years was 72% among
patients with one or more characteristic brain lesions, whereas it was 25% if the MRI was normal
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8. Atypical optic neuritis
Features that are atypical for optic neuritis
painless visual loss, severe disc edema, disc or peripapillary hemorrhages, and macular exudate.
The risk of developing MS is negligible.
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9. Treatment and follow up
The optic nerve usually demonstrates pallor, suggesting that axonal loss has accompanied the episode of
demyelination on follow up.
OCT usually demonstrates atrophy of the retinal nerve fiber and ganglion cell layers.
Treating optic neuritis with high-dose IV corticosteroids reduces the risk of developing MS over the
following 2 years.
In the long term, this acute treatment is unlikely to affect the likelihood of progression to MS. In addition,
IV corticosteroid treatment may hasten visual recovery but does not significantly affect longterm visual
outcomes
Because oral prednisone (1 mg/kg) may be associated with an increased risk of recurrence of optic neuritis
when not used in conjunction with high-dose IV corticosteroids, this therapy should be avoided
In addition to IV corticosteroids, studies support the early use of immunomodulating treatments for high-
risk patients to reduce the likelihood of progression to MS within 2–5 years
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10. Neuromyelitis optica
NMO, or Devic disease, is characterized by necrotizing demyelinating lesions of the optic nerves and
the spinal cord.
The spinal lesion characteristic of NMO often extends contiguously over three or more vertebral
segments.
NMO is a humorally mediated disease distinct from MS.
The majority of patients with NMO have serum antibodies that target aquaporin 4 (NMO-IgG, which
is a water channel that has a role in cell water homeostasis in the brain and spinal cord).
Minority have serum antibodies that target myelin oli- godendrocyte glycoprotein (MOG-IgG, which is
a cell membrane protein that has a role in myelin sheath construction and integrity.
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11. Management of NMO
Optimal treatment regimens for optic neuritis occurring in patients with underlying NMO have not
been established.
Plasmapheresis may be beneficial in the acute stage.
Treatment with rituximab (a chemother- apeutic monoclonal antibody that depletes B cells) may be
an effective disease-modifying agent
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12. Arteritic anterior Ischemic optic
neuropathy
Arteritic anterior ischemic optic neuropathy (AAION) is usually related to GCA, also referred to as
temporal or cranial arteritis.
Increases with age, with most patients being older than 70 years.
GCA typically affects the extracranial medium- to large-caliber arteries because they possess elastic
lamina, which is the initial site of inflammation in this disorder.
Condition is associated with polymyalgia rheumatica, consisting of proximal muscle ache, arthralgia,
and stiffness, as well as with jaw claudication, fever, malaise, and scalp tenderness.
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14. The diagnosis is suggested by an elevated erythrocyte sedimentation rate and C-reactive protein and
is confirmed by evidence of giant cells and endovascular inflammation on temporal artery biopsy.
Vision loss is the presenting symptom in up to 60% of cases and is generally more severe than in
NAION. In approximately 25% of cases, vision is limited to hand motion perception or worse. In
suspected cases, treatment with corticosteroids should not be delayed.
IV corticosteroids may help delay the progression of visual loss and decrease the likelihood of fellow
eye involvement.
The prognosis for recovery in the affected eye, however, is poor despite treatment.
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15. The optic disc in AAION typically has a chalky-white edematous appearance, and disc hemorrhages
are likely to be present .Coexisting retinal ischemia with cotton-wool spots is very suggestive of
AAION.
Fluorescein angiography reveals choroidal hypoperfusion.
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16. Arteritic posterior ischemic optic
neuropathy
Occasionally, the ischemic insult is to the retrobulbar portion of the optic nerve and causes visual loss
without disc edema; this condition is termed arteritic posterior ischemic optic neuropathy.
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17. Non arteritic anterior ischemic
optic neuropathy
NAION is the most common cause of unilateral optic nerve edema in adults older than 50 years and is
commonly associated with vascular risk factors such as diabetes or hypertension.
Other risk factors include a congenitally crowded optic nerve head, obstruc- tive sleep apnea, and
nocturnal hypotension, possibly precipitated by antihypertensive therapy.
Swelling of a crowded optic nerve within the scleral canal may produce a compartment syndrome,
provoking further vascular compression, ischemia, and swelling.
typical features of NAION include nerve fiber hemorrhages, altitudinal visual field defects, moderate to
severe disc edema, and the absence of pain.
Because the optic nerve head is supplied by an end-arterial system of short posterior ciliary arteries and
the circle of Zinn-Haller, sectoral disc edema is common. NAION may follow ocular surgery
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19. NAION will have a stable deficit, although a minority may experience stepwise visual loss progressing
over 1 month. Spontaneous improvement may occur in the first 6 months, although in many patients,
this reflects improved ability with eccentric fixation.
In 15%–20% of patients, subsequent involvement of the fellow eye occurs, and this rate is increased
by the presence of vascular risk factors and younger age, especially if there are crowding optic disc
anomalies such as optic disc drusen and cogenitally small optic disc.
Recurrence of NAION in an affected eye, however, is rare, possibly because optic nerve atrophy
following the initial event decompresses the nerve. There does not appear to be a significantly higher
rate of stroke in patients with NAION, suggesting that its pathophysiology may differ from
atherosclerotic disease
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20. Foster Kennedy syndrome
When the second eye is affected in NAION, optic atrophy has already developed in the first eye, and
acute disc edema is present in the fellow eye; this clinical presentation is called the pseudo-Foster
Kennedy syndrome.
A true Foster Kennedy syndrome is characterized by optic atrophy due to compression, typically from
an expanding tumor, and papilledema in the fellow eye secondary to increased ICP.)
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21. Other causes
Inflammation
Infections
Mass lesions
Radiation
Paraneoplastic syndromes
LHON
TON
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22. Inflammation
Inflammatory conditions cause subacute optic neuritis
Optic nerve involvement is common in neurosarcoidosis, which can be accompanied by anterior uveitis or
posterior segment vitritis. Visual loss in this condition is often steroid responsive.
Optic neuropathy and retinal involvement may also occur with other inflammatory conditions, such as
systemic lupus erythematosus and Sjögren disease. Occasionally, optic nerve infiltration produces optic disc
edema without affecting visual function, but more often, there is a decrease in visual acuity and visual field
loss.
steroid-dependent, indicating that long- term immunomodulatory treatment is necessary to prevent
relapsing symptoms; this clinical entity has been called chronic relapsing inflam- matory optic neuropathy,
proportion of patients with this entity have NMO-IgG or MOG-IgG serum antibod- ies
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24. Infections
Neuroretinitis, in which optic neu- ropathy coexists with characteristic peripapillary or macular exudates,
should be distinguished from acute demyelinating optic neuritis.
Characteristic macular star of neuroretinitis will appear within 1–2 weeks, establishing the diagnosis. The
distinction is critical because neuroretinitis has no association with an increased risk of MS.
Causes - cat scratch disease (Bartonella henselae), syphilis (Treponema pallidum), Lyme disease (Borrelia
burgdorferi), Bartonella infection, human immunodeficiency virus (HIV) and opportunistic infections
including toxoplasmosis, cytomegalovirus, and cryptococcosis.
Optic neuropathy due to sinusitis and mucocele should be considered in patients who have clinical
evidence of optic neuritis with seemingly atypical features, particularly in elderly patients with severe sinus
disease, a history of fevers, ophthalmoplegia, or progression of vision loss beyond 2 weeks. Presentations
after dental infection also occur.
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26. Compressive mass lesions
Important causes of compressive optic neuropathy include
neoplasm (including optic nerve sheath or skull base meningioma, pituitary adenoma,
craniopharyngioma, dermoid and epidermoid tumors, and metastases).
sinus lesions
bony processes (such as fibrous dysplasia)
enlarged extraocular muscles (such as in thyroid eye disease),
aneurysms
meningiomas of the optic nerve
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27. Compressive lesions causes progressive optic neuropathy.
The optic disc is edematous in cases of intraorbital compres- sion,
Retro-orbital compression, disc edema typically only occurs if ICP is elevated.
Chronic disc edema due to compressive lesions may be accompanied by optociliary collateral
vessels and glistening white bodies on the disc surface.
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29. Primary optic nerve neoplasms
Benign juvenile pilocytic glioma in children.
Juvenile pilocytic astrocytoma is often associated with neurofibromatosis type 1 and may be
managed conservatively with frequent ophthalmological examination through adolescence .When
clinical or radiographic progression is detected, chemotherapy should be first-line therapy, followed
by radiation and, rarely, surgery.
Malignant glioblastoma
Malignant optic nerve glioblastoma is much rarer, affects adults, and has a poor prognosis.
Other neoplastic conditions include lymphoma, leukemia, carcinomatous meningitis, and optic nerve
metastasis.
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31. Radiotherapy induced optic
neuropathy
It can occur with or without disc edema and can sometimes be difficult to distinguish from tumor
recurrence.
Radiation optic neuropathy is suggested by exposure (typically at least 50-Gy dosage), char- acteristic
6- to 24-month time lag to symptoms, and accompanying radiation changes in proximal tissues.
Progression occurs over weeks to months and spontaneous recovery is rare.
Several treatments, including corticoste- roids, hyperbaric oxygen, and vascular endothelial growth
factor inhibitors, have been reported in occasional cases to halt progression of visual loss.
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32. Paraneoplastic optic neuropathy
or retinopathy
Paraneoplastic optic neuropathy - the antibody most commonly identified is directed toward collapsin
response mediator protein 5 (CRMP5).
Paraneoplastic retinopathies - include CAR - antibodies to recoverin protein.
Melanoma- associated retinopathy - antibodies to rod ganglion cells.
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33. LHON
LHON is a subacute, sequential, maternally inherited optic nerve dis- order in which 80%–90% of affected
persons are males in the second or third decade of life.
The optic disc may appear hyperemic and mildly edematous in the acute phase, although fluorescein
angiography does not demonstrate capillary leakage because true disc edema is not present.
Circumpapillary telangiectatic vessels, present in the peripapillary nerve fiber layer, are an important clue
to the diagnosis.
These early funduscopic changes may be noted in presymptomatic eyes. As the condition progresses, the
discs become atrophic.
Fibers mediating the pupillary light reflex may be selectively spared, the light reflex may be preserved
despite significant visual loss.
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35. Genetic diagnosis of LHON is based on the identification of mitochondrial DNA mutations.
Most patients have permanent visual loss, although a minority will experience some recovery.
The prognosis depends on the mutation harbored - patients with mtDNA mutation 14484 are more
likely to have spontaneous recovery than patients with mutations 11778 or 3460.
At present, no effective treatment for this condition is available.
Idebenone has been studied and might promote some improvement of vision
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36. TON
Direct TON may include nerve avulsion or transection and is easily recognized by the history of injury.
Fundus examination may reveal extensive intraocular hemorrhages.
Posterior indirect TON will present with visual loss in the absence of fundus abnormalities; it may
result from shearing forces and subsequent edema within the optic canal.
Up to half of these patients may improve spontaneously .
Treatments, including corticosteroids and surgical decompression, remain controversial and mostly
ineffective
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38. Bilateral Optic Disc
Edema
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39. Papilledema
The term papilledema refers specifically to optic disc edema secondary to increased ICP.
Disc edema in papilledema results from blockage of axoplasmic flow in nerve fibers, increasing the
volume of axoplasm in the optic disc.
Papilledema can be divided into four stages:
early,
fully developed (acute),
chronic, and
atrophic.
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40. Fields and VA
The most common visual field defects with early or acute papilledema are
enlargement of the physiological blind spot,
arcuate visual field defects (typically inferonasal), and
concentric constriction.
Decreased acuity due to metamorphopsia - fluid typically extends from the peripapillary region to
macula.
Edema is most prominent at the superior and inferior poles of the optic disc where the nerve fiber
layer is thickest.
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41. Early and Acute disc changes -
papilledema
The retinal veins may distend slightly, and the disc may appear mildly hyperemic.
Edema causing compression of capillaries and venules, leading to venous stasis and dilation,
formation of microaneurysms, and finally flame-shaped disc and peripapillary hemorrhages.
Concentric retinal folds may be seen in the peripapillary region, and radial retinal folds may be seen
extending into the macula.
Retinal cotton-wool spots may occur secondary to infarction of the nerve fiber layer
Spontaneous venous pulsations (SVPs) usually are absent.
Usually bilateral
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42. FFA
Fluorescein angiography acute papilledema :
Absent fluorescence during the retinal arterial phase as a resultof delayed circulation caused by disc
swelling.
Dilated capillaries and microaneurysms may appear in the arteriovenous phase.
Fluorescein may leak from dilated capillaries in the venous phase.
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43. Chronic disc changes -
papilledema
Usually after weeks to months.
The nerve fiber layer may appear pale and take on a gliotic appearance as a result of optic atrophy
and astrocytic proliferation .
Hemorrhages are less prominent or resolved completely.
The disc takes on a “champagne cork” appearance in which small, glistening white bodies
(pseudodrusen) are present owing to extruded axoplasm after prolonged stasis.
Collateral vessels between the retinal and ciliary circulation may appear.
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45. Atrophic papilledema
If increased ICP and papilledema persist, optic nerve axons become damaged, and visual field loss
develops; optic disc edema lessens, and pallor develops (atrophic papilledema).
Finally, patients with endstage papilledema exhibit optic nerve atrophy (disc pallor) without
evidence of swelling.
Chronic atrophic papilledema, unlike the acute phase, is often characterized by loss of visual acuity
with severely constricted visual fields.
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46. Causes - papilledema
Expanding SOL
Stroke – cerebral edema
Increased ICP due to intracranial hemorrhage
Compression of ventricular system in fossa
Venous sinus thrombosis – pregnancy , hypercoagulability
Cryptococcal meningitis
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47. Idiopathic intracranial
hypertension
Also known as pseudotumor cerebri or benign intracranial hypertension can lead to disc edemaand
progressive visual loss.
The condition is most common in obesewomen, but modest weight gain (by 5%–15%) even in
nonobese womenis a risk factor for the disease.
Additional risk factors – tetracycline , vit A derivatives
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48. Treatment
Mild visual loss, treatment with acetazolamide can improve symptoms and reduce optic disc swelling.
In patients with more severe visual loss or a fulminant presentation - surgical interventions
optic nerve sheath fenestration
cerebrospinal fluid (CSF) shunting
transverse venous sinus stenting
indicated to reduce the possibility of permanent visual loss.
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49. Malignant Hypertension
May produce bilateral optic disc edema indistinguishable from papilledema.Peripapillary cotton-wool
spots and retinal hemorrhages are other prominent features in patients with malignant hypertension.
Encephalopathy and vision loss are common but not always present.
The changes associated with malignant hypertension can occur at lower blood pressures in patients
with renal failure.
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50. Diabetic Papillopathy/Incipient
NAION
Rare cause of bilateral disc edema in pt with diabetes
Visual loss is typically minimal,with the exception of an enlarged physiological blind spot.
Disc edema may be accompanied by marked capillary telangiectasias overlying the disc surface.
The pathogenesis is unclear,possibly due to mild impairment of blood flow causing disc edema
without infarction of the optic nerve head, as in the case of premonitory NAION, also called incipient
NAION.
In many cases, the optic disc edema resolves without residual visual deficit.
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52. Other causes
Anemia
Hyperviscousity states
Pickwickian syndrome
Hypotension
Severe blood loss
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53. Unilateral disc edema may
present bilaterally
Infiltrative lesions
Optic neuritis in children
GCA may present as bilateral simultaneous ischemic optic neuropathy in elderly >55yrs
LHOA
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54. Thank you
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