Optic Atrophy

OPTIC ATROPHY
Presenter- Dr. Anmol Mahajan

Optic atrophy refers to the death of the retinal ganglion cell axons that comprise
the optic nerve with the resulting picture of a pale optic nerve on fundoscopy.
Optic atrophy is an end stage that arises from myriad causes of optic nerve
damage anywhere along the path from the retina to the lateral geniculate. Since
the optic nerve transmits retinal information to the brain, optic atrophy is
associated with vision loss. Optic atrophy is somewhat of a misnomer as
atrophy implies disuse, and thus optic nerve damage is better termed optic
neuropathy.
Etiology
Anything that can compromise ganglion cell function can cause (over time)
optic atrophy (and more broadly optic neuropathy). Optic atrophy can occur due
to damage within the eye (glaucoma, optic neuritis, papilledema, etc.), along the
path of the optic nerve to the brain (tumor, neurodegenerative disorder, trauma,
etc.), or it can be congenital (Leber’s hereditary optic atrophy, autosomal
dominant optic atrophy).
Risk Factors
Risk factors run the gamut from increased intraocular pressure (glaucoma),
ischemia, compression (tumors), inflammation, infection, etc. See differential
diagnosis below.
General Pathology
The optic nerve is a bundle of 1.2 million axons of retinal ganglion cells that
carries visual information from the retina to the brain. The optic nerve is
myelinated by oligodendrocytes that do not regenerate after damage. In optic
nerve atrophy there is loss of axons and shrinkage of myelin leading to gliosis
and widening of the optic cup.
Primary prevention
Optic atrophy is the end stage of a process causing damage to the optic nerve.
Medical practice is currently unable to return function (regrow axons) to an
atrophic optic nerve, and at best is able to stabilize whatever function remains.
Primary prevention (removal of the process causing the damage) is the goal to
prevent loss of axons and optic atrophy (neuropathy).
Diagnosis
Since the optic nerve is the conduit for information from the retina to the brain,
a damaged optic nerve will result in vision loss. Subtle damage might not affect
acuity but may lead to a loss of contrast or color vision. Severe damage may
lead from legal blindness to no light perception. Damage to a part of the optic
nerve results in loss of vision in the corresponding visual field. Occasionally if
the process causing damage is removed before apoptosis occurs (as for instance
removal of a pituitary tumor compressing the chiasm or reducing inflammation
in sarcoid) some improvement in visual function may be noted. A complete
diagnosis is based on optic nerve appearance, tests of visual function (visual
field, contrast, color, acuity), identifying the causative factor of the damage, and
ruling out other causes for vision loss (such as retinal causes).
Certain disc appearances can help to determine the cause for the optic nerve
damage. Sector disc pallor in an older individual could have been caused by
NAION. Severe optic atrophy with gliosis again in an elderly person could have
been due to giant cell arteritis. Damage from papilledema may leave retinal
folds and sometimes glistening bodies in the optic nerve head. Cupping is
suggestive of glaucoma.
Optical Coherence Tomography has become a valuable tool to verify the status
of the nerve fiber layer/ganglion axons. Quantification of the nerve fiber layer
height and comparison with normative data can document axon loss and
differentiate between optic nerve and retinal disease as a cause for vision loss.
History
History is critical in the diagnosis of optic atrophy since the physician needs to
know how the eye arrived at this juncture. A careful history with attention to
past medical history including all medications, time course of vision loss,
associated symptoms etc is critical for arriving at a correct diagnosis.
Physical examination
A complete eye exam including visual field, assessing color and contrast vision,
intraocular pressures, looking for afferent pupil defect, and fundoscopy should
be done.
Signs
Optic atrophy is a sign and typically is noted as optic nerve pallor. This is the
end stage of a process resulting in optic nerve damage. Because the optic nerve
fiber layer is thinned or absent the disc margins appear sharp and the disc is
pale, probably reflecting absence of small vessels in the disc head.
Symptoms
The main symptom of optic atrophy is vision loss. Any other symptoms are
attributable to the underlying process that caused the disc damage (such as pain
with angle closure glaucoma).
Clinical diagnosis
Optic atrophy is usually not difficult to diagnose (characteristic pale optic disc)
but the cause for the optic atrophy may be difficult to ascertain. Sometimes the
cause of vision loss may be difficult to differentiate between subtle optic
neuropathy and disease of the retina (or both). Electrophysiology can be helpful
(ERG, mERG) and OCT to assess the thickness of the nerve fiber layer may be
helpful in such cases.
Characteristic visual field patterns include papillomacular defect (cecocentral
scotoma), arcuate defect (include altitudinal) or temporal wedge defect (nasal
fibers) for prechiasmal, bitemporal (superior) field defects for chiasmal lesions,
and hemianopsia for post-chiasmal lesions.
The following work up should be considered for patients presenting with

unexplained optic atrophy:
Check for afferent pupil
Visual fields 30-2,color vision
MRI of brain and orbit with contrast
CT with contrast (check bony disease, sinuses)
Blood pressure and check of cardiovascular health (carotids, etc.), Glucose
Screen for these if history or examination are suggestive:heavy metals, B12,
folate, FTA ABS, VDRL, ANA, homocysteine, ACE, Antiphospholipid
antibodies, TORCH panel [1]
Diagnostic procedures
Visual Field Testing (Humphrey 30-2, Tangent Screen) - to help localize the
location of the lesion.
Optical Coherence Tomography (OCT) - to assess the thickness of peripapillary
retinal nerve fiber layer and/or ganglion cell layer.
ERG, mERG - to rule out retinal disease.
Neuro-imaging (MRI, CT) – to asses for tumors, bone growth, sinusitis,
fractures, multiple sclerosis, and infections.
Laboratory test
As stated above, if history or examination are suggestive, it may be useful to
screen for: heavy metals, B12, folate, VDRL, ANA, homocysteine, ACE,
Antiphospholipid antibodies, TORCH panel.
Differential diagnosis
Optic atrophy is not usually difficult to diagnose but might be confused with
optic nerve hypoplasia, myelinated nerve fibers, myopic or scleral crescent, or
tilted disc.
The causes for optic atrophy include:
Compressive – secondary to papilledema, tumor, bony growth (fibrous

dysplasia, osteopetrosis), thyroid eye disease, chiasmal (pituitary etc), optic
nerve sheath meningioma, disc drusen, increased intraocular pressure
(glaucoma)
Vascular – arteritic and non-arteritic ischemic optic neuropathy, diabetes,

Inflammatory – sarcoid, systemic lupus, Behcet’s, demyelination (MS), etc.
Infectious – viral, bacterial, fungal infections - herpes, TB, bartonella, etc.
Toxic & nutritional – many medications such as ethambutol, amiodarone,
methanol, vitamin deficiency etc.
Metabolic – diabetes
Neoplastic – lymphoma, leukemia, tumor, glioma
Genetic – Autosomal dominant optic atrophy (OPA1), Leber’s hereditary
optic atrophy, Leber's hereditary optic neuropathy, as a late complication of
retinal degeneration.
Radiation optic neuropathy
Traumatic optic neuropathy
Management
The management goal is to intervene before optic atrophy is noted or to save
remaining function. This will depend on the underlying cause for the optic nerve
damage. For instance, intraocular pressure control in glaucoma, control of
inflammation in sarcoid, etc.
Prognosis
Studies in glaucoma (based on OCT nerve fiber layer measurements and other
methods) have shown that the optic nerve has some reserve (axons) before
vision loss is appreciated. After that reserve is depleted small changes in nerve
fiber loss lead to significant decrease in vision. Early detection is key sinceDr
we cannot replace dead axons.

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OPTIC ATROPHY

Presenter- Dr. Anmol Mahajan

The following work up should be considered for patients presenting with

Compressive – secondary to papilledema, tumor, bony growth (fibrous

Vascular – arteritic and non-arteritic ischemic optic neuropathy, diabetes,

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