OPTIC NERVE

+
OPTIC NERVE
LESIONS

Robert William B. King, MD

May 09, 2015

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ANATOMY
Begins:
Optic disc (Anatomically)
Ganglion cells of retina (physiologically)

ANATOMY
First part of ON
Approx. 1.0-1.2 M ganglion cells

Axons travers the sclera

through lamina cribrosa (200300 channels)

1mm
Sheathed with dura and

myelin coating once posterior

the sclera
3

ANATOMY
Intraorbital
Extends approx. 25-30mm to

the optic canal

Intracanalicular
Approx 4-10mm

Intracranial
10-20mm

LESIONS

PAPILLEDEMA
Swelling of the optic disc from increased intracranial

pressure

Headache is usual (worse in morning)

An alarming sign
Results from axoplasmic flow stasis of the optic nerve

head

Can result in loss of axons, and eventual atrophy

Develops anywhere from <24hrs to weeks
6

PAPILLEDEMA CAUSES
Space occupying lesion (tumor, hemorrhage)
Decreased CSF drainaige or absorption (obstructive

hydrocephalus / infection, hemorrhage, thrombosis)

Idiopathic intracrainial hypertension (Pseudotumor

cerebri)

Increased CSF production (choroid plexus tumor)

Decreased skull volume (craniosynostosis)

Must be distinguished from:

Edema of other cause
Pseudo papilledema (disc drusen)
Papillitis

DIAGNOSIS
Fundus exam - noting disc swelling (usually bilateral

unless one is atrophic)

Fluorescein Angiography
Perimetry (enlarged blind spots)
CT or MRI (preferred) (can be noted as flattening of

the sclera near optic nerve + raised area at disc

head)

Lumbar puncture
10

11

 Fast-spin echo T2-weighted axial imaging demonstrated reversed optic

nerve cupping in the right eye with posterior scleral flattening and
protrusion of optic nerve papilla into the globe (arrow)

12

PAPILLITIS
Inflammation or infarct of the optic nerve head
Usually unilateral
(+) Visual abnormalities (color, VA)
Optic nerve head swelling
Disc hemorrhages
Causes:
Optic neuritis
Multiple sclerosis
Infarct or nerve head
13

PAPILLEDEMA VS PAPILLITIS
Papilledema

Papillitis

Definition

Due to raised intracranial

pressure

Inflammation of the optic

nerve head

Laterality

Bilateral

Unilateral

Cause

Increase in ICP

Optic neuritis, multiple

sclerosis, infarct

Vision loss

Not usual

COMMON (cardinal
symptom)

Color perception

Unchanged

Depressed

RAPD

No

Yes

Hemorrhages

Around disk

Periphery of disc or on disc

Treatment

Decrease ICP (depending

on cause)

Usually high dose steroids

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WHICH IS WHICH?
Papillitis

Papilledem
a

16

OPTIC DISC DRUSEN

Pseudopapilledema
Consist of refractive, hyaline-like calcified nodules located within

the optic nerve head

May be due to slowed axoplasmic flow

Prevalence 0.4-3.7%
Males = Females
Whites > Blacks
>85% bilateral, but asymmetric
Familial Autosomal dominant inheritance
17

OPTIC DISC DRUSEN

Composed of small proteinaceous material that become

calcified with advancing age.

May lead to an elevated disc (and therefore

pseudopapilledema).

Usually asymptomatic, but may lead to a loss of visual field or,

in rare cases, central acuity.

18

 Elevated optic disc, with small contour

Indistinct and irregular disc margins
Anomalous vascular branching pattern (tortousity, optociliary shunt vessels)
Drusen seen as round, white/yellow refractile bodies on the surface of the nerve or buried

beneath it

Nasal margin is most common site of drusen

Spontaneous venous pulsations often seen
Afferent pupillary defect if there is asymmetric nerve involvement

19

TREATMENT
No treatment necessary (normal physiologic variant)
Visual prognosis for is generally good
Currently no effective treatment for patients that have gradual loss of

visual fields

20

TRAUMATIC OPTIC
NEUROPATHY (TON)
Most common cause: Indirect injury to the optic nerve
85% of patients are male, ave age of 34 (International Optic Nerve Trauma Study)
Symptoms:
Decreased central visual acuity
Depressed color vision
RAPD
Visual field deficits

Signs
The optic nerve head will appear normal initially, but optic atrophy can be seen

3-6 weeks after the initial traumatic event.

21

 History:
A history consistent with TON
Vision loss after blunt or penetrating trauma that could not be
explained by slit lamp or dilated fundus findings.
Often, patients complain of acute unilateral decrease in vision, color
vision deficits, or visual field deficits.

22

PATHOPHYSIOLOGY OF TON
The optic nerve dura is continuous with the orbital periosteum, leaving the

optic nerve susceptible to transmission of force from blunt head trauma.

Indirect TON - result from shearing injury to the intracanalicular portion of

optic nerve, which can cause axonal injury or disturb the blood supply of
the optic nerve.

Direct TON - tissue disruption secondary to foreign body or bony fragments

impacting on the optic nerve.

23

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PROGNOSIS
Visual acuity improvement of >3 lines was seen in 57% of patients
Use of steroids did not result in significant difference in outcome

25

ARTERITIC ANTERIOR
ISCHEMIC OPTIC
NEUROPATHY
(AAION)
Acute and painless optic neuropathy

Decreased visual acuity - tipically severe (<20/200 in over

60% of the patients)

May occur as ocular manifestation of giant cell arteritis (5-10%

of cases)

Predominantly in patients over 70 years old

26

PATHOGENESIS
Inflammatory and thrombotic involvement of the short

posterior ciliary arteries (SPCAs) with resultant optic nerve

head infarction

Ischemia occurs at the head of the optic nerve in relation with

structural crowding of the nerve fibers and reduction of the

vascular supply impairing perfusion and producing optic disc
edema.

27

DIAGNOSIS
Severe visual acuity loss
(+) RAPD
Pallor of the optic disc, which may be severe, chalky-white is the hallmark

of AAION

Narrowed peripapillary arterioles

Diffuse disc swelling
Extremely poor or absent filling of the choroid has been depicted as a

characteristic of AAION

Elevated ESR and/or CRP

28

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TREATMENT
Early treatment is essential.
High dose systemic corticosteroid are standard.
IV methylprednisolone at 1g/day for the first 3 days has been

recommended for severe cases.

Oral prednisone in the range of 60-100mg/day may be used initially and for

follow up to intravenous pulse therapy.

Treatment is usually continued at a high dose for a several months before

beginning taper.

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PROGNOSIS
Without treatment, visual loss occurs in 54-95%, typically

within 4 months. Reduced to 13% if with corticosteroid

therapy.

Despite treatment, visual recovery of the affected eye is poor

with a 15-34% improvement rate.

31

NON-ARTERITIC ANTERIOR ISCHEMIC

OPTIC NEUROPATHY (NAION)
Acute painless visual loss
Most common cause of acute optic neuropathy in patients over the age of

50

Pathology is unclear, but it is presumed to result from a circulatory

insufficiency

Leads to a compartment syndrome from axonal edema in a structurally

crowded optic disc

Up to 97% of patients with NAION have small optic discs with small or

absent optic cups

32

 Signs: Some or all of the signs of an optic neuropathy

Decreased visual acuity (NLP very rare)
Dyschromatopsia
RAPD
Swollen optic nerve
Splinter Hemorrhages
Visual field defects (altitudinal field loss [inferior most common], central

scotoma)

33

DIAGNOSTICS
ESR
CRP
Temporal artery biopsy is recommended to rule out GCA and AAION

34

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TREATMENT
There is no effective treatment for NAION

36

PROGNOSIS
Vision can worsen over 2 weeks after initial presentation
Natural history of visual outcome showed approximately 50% of

patients had a visual acuity of 20/30 or better and nearly onequarter were 20/200 or worse.

Vision in the affected eye will typically stabilize within two months
Progression or recurrence more than two months after initial

presentation should bring the diagnosis of NAION into question

Recurrence in the affected eye range from 3% to 8%

Involvement of the fellow eye ranges from 15% to 24% over 5

years

37

POSTERIOR ISCHEMIC OPTIC

NEUROPATHY
Acute
Retrobulbar portion of ON
Sever vision loss
(+) RAPD
Initially optic discs are normal
Diagnosis of exclusion (rare)
Prognosis: Poor
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DIABETIC PAPILLOPATHY
Occurs in both DM type 1 or 2
No symptoms or have nonspecific symptoms of"blurred vision'' or

"distortion" without pain

The optic nerve reveals hyperemic edema

VA and RAPD are variable
50% with marked dilation of the disc surface microvasculature appearing

similar to neovascularization of the disc (NVD)

The absence of DM retinopathy does not preclude a diagnosis of diabetic

papillopathy

39

40

PROGNOSIS
resolve slowly over 2-l 0 months. Optic atrophy occurs in 20% of cases, but

the visual

Prognosis often depends mostly upon the degree of accompanying diabetic

retinopathy.

Rarely, diabetic papillopathy progresses to AION, with residual pallor and

nerve fiber defects

41

LEBER HEREDITARY OPTIC

NEUROPATHY (LHON)
Males 10-30 y/o
Mitochondrial DNA
Acute, severe, painless, sequential vision loss (visual acuity, <20/200) and

central or cecocentral visual field impairment

Fundus Triad

(AAO 2015):

1. Hyperemia and elevation of the optic disc, with thickening of the peripapillary

retina; although the disc appears swollen, it does not leak on fluorescein
angiography ("pseudoedema'')
2. Peripapillary telangiectasia
3. Tortuosity of the medium-sized retinal arterioles

42

Hyperemic optic discs with blurred margins and moderately tortuous

vasculature.
43

TREATMENT
No effective treatment

44

PROGNOSIS
Unaffected eye becomes symptomatic within weeks to months
Vision loss is usually permanent
Recovery of vision occur in 10%-20% of cases over several

years

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AUTOSOMAL DOMINANT
OPTIC ATROPHY (ADOA)
Most common hereditary optic neuropathy (1:50,000)
Autosomal dominant
First decade of life
Insidious vision loss
Bilateral and symmetric
Color vision loss
No treatment available

46

OPTIC NERVE HYPOPLASIA

VA ranges from 20/15 to no light perception
BUT almost all have some degree of visual field loss
56-92% bilateral
The optic disc is small, usually one-half to one-third of normal diameter
Discs may be pale/grey or hyperemic (less common)
May be associated with endocrine abnormalities

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EXCAVATED OPTIC DISC

ANOMALIES
Optic pit
Depression of the optic disc surface

that is often gray or white,

Associated with a mild visual field

Serous detachment of the macula

develops in 25%-75%

49

EXCAVATED OPTIC DISC

ANOMALIES
Colobomas Result from incomplete closure of the

embryonic fissure

Usually occur inferiorly

Occasionally extend to the adjacent

choroid and retina.

May have VF defects and RAPD

depending on severity

Colobomas of other eye structures may

be present

50

EXCAVATED OPTIC DISC

ANOMALIES
Morning glory disc anomaly

Funnel-shaped staphylomatous excavation of

the optic nerve and peripapillary retina.

More common in females

Unilateral
(+) RAPD
Chorioretinal pigmentation surrounds the

excavation, and white glial tissue is present on

the central disc surface.

The characteristic feature: Radial spoke pattern

of retinal vessels

Visual acuity can be normal, but is often 20/200

or worse

Retinal detachment is common

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POSTERIOR OPTIC
NEUROPATHIES:
RETROBULBAR OPTIC
NEURITIS
Typically in young females (30s)

Retrobulbar form occurs in 65% of cases

Disc appears normal
Subacute monocular visual loss

Periorbital pain, particularly with eye movement, occurs in 92%

(+) RAPD unless bilateral
Visual field loss
Dyschromatopsia (especially for red)
Some improvement after 1 month
Usually due to an isolated demyelinating event
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TREATMENT
Corticosteroids failed to demonstrate long term benefit for visual acuity
Corticosteroids may speed up recovery by a few weeks

54

NEUROMYELITIS OPTICA
AKA Devic Syndrome
Optic neuritis and acute myelitis

Diagnostic Criteria (99% sensitivity 90% specificity):

Optic neuritis
Myelitis
At least 2 of the ff:
Contiguous spinal cord lesion on MRI involving 3 or more vertebral segments
Brain MRI nondiagnostic for MS
Positive NMO-IgG serology

55

TREATMENT
Mainstay treatment for acute period is high

dose steroids IV
IV Ig or plasmapheresis can be done on
patients who do not respond to steroids

56

PROGNOSIS
Visual prognosis for NMO patients are poorer vs MS

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OPTIC PERINEURITIS
Inflammation of optic nerve sheath
Acute painful vision loss
Female predilection
Patients are generally older >50 y/o
Vision loss and pain progresses over weeks unless

treate

58

DIAGNOSIS
Orbital MRI will show

enhancement of the dural

sheath rather than optic
nerve

59

TREATMENT
Responds rapidly to corticosteroids, but relapses are common

60

INTRAORBITAL COMPRESSIVE
OPTIC NEUROPATHY
Slow progressive vision loss
RAPD
Monocular visual field loss (central of diffuse)
Other signs of orbital disese
Proptosis
Ptosis
Retraction
Lag
Edema
Optic disc might be normal or with some edema

61

MOST COMMON CULPRITS

Optic nerve sheath meningioma
Optic nerve sheath glioma

62

DIAGNOSIS
MRI
Thin section CT scan

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OPTIC NERVE SHEATH

MENINGIOMA (ONSM)
Proliferation of meningioepithelial cells of intraorbital and

intracanalicular optic nerve

1/3 of primary optic nerve tumors

Adults aged 40-50
Women > Men
Diagnostic triad:
Painless slow monocular vision loss
Optic atrophy
Optociliary shunt vessels
64

 Optociliary shunt

vessel

preexisting optic disc

channels that dilate due to

chronic obstruction of
outflow through the central
retinal vein.

65

 Tram track sign

66

 Ring sign

67

TREATMENT
Fractionated radiation therapy is the modality

of choice for ONSM

Stability or improvement in vision up to 94.3% of

patients

68

OPTIC PATHWAY GLIOMA

(OPG)
Most common primary tumor of the optic

nerve
May involve optic nerve, chiasm or both
First decade of life
Men = Women

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DIAGNOSIS
Proptosis (94%)
Vision loss (87.5%)
Optic dic pallor (59%)
Disc edema (35%)
Strabismus (27%)
RAPD usually present
70

DIAGNOSIS
MRI shows diffuse enlargement and

enhancement of affected nerve

71

TREATMENT
Observation may be indicated for stable

lesions with a good VA

Chemotherapy for progression
Radiotherapy is inconclusive

72

PROGNOSIS
Blindness usually 2-4 months after start of

vision loss
Death usually within 6-12 months

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TOXIC OR NUTRITIONAL
OPTIC NEUROPATHY
Gradual progressive vision loss
Painless
Bilateral and symmetric
Central scotoma
Decrease in VA and color vision
Disc edema

74

COMMON MEDICATIONS
IMPLICATED
Ethambutol
Linezolid
Isoniazid
Chloramphenicol
Hydroxyquinolines
Penicillamine
Cisplatin
Vincristin
75

PROGNOSIS
Slow resolution of nerve edema over months of

discontinuation of substance

76

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INFILTRATIVE OPTIC
NEUROPATHY
Infiltration of ON with neoplastic or inflammatory cells
Progressive, sever vision loss
Often with pain
Unilateral or bilateral

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USUAL CAUSES
Leukemia
Lymphoma
Sarcoidosis
Syphilis
TB
Fungal
79

DIAGNOSIS
Neuro imaging
CSF evaluation

80

 Metastasis of

Leukemia to the
Optic nerve head
Note ill defined

yellowish
infiltrates

81

PROGNOSIS
Metastatic:
Poor even with aggressive therapy

Infectious/Inflammatory:
damage may be partially reversed

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