Optic atrophy is the degeneration of the optic nerve that occurs when the ganglion cells and axons in the retina are damaged. It can be classified clinically, etiologically, and anatomically. Clinically, it is classified as primary, secondary, consecutive, cavernous, or segmental based on ophthalmoscopic features. Causes include diseases of the visual pathways, swelling/congestion of the optic nerve head, destruction of ganglion cells, or axonal degeneration without glial proliferation. Etiologically, it can be hereditary, consecutive, vascular, toxic, metabolic, demyelinating, pressure-related, post-inflammatory, or traumatic. Anatomically it is classified
2. DEFINITION
• Optic atrophy is the degeneration of the optic nerve, which occurs
as a pathological end point of any disease that causes damage to
the ganglion cells and axons in the retina to the lateral geniculate
body (LGB)
6. Primary Optic Atrophy
• There is orderly degeneration of optic nerve fibers and is replaced by columns of
glial tissue without any alteration in the architecture of the optic nerve head
• It is caused by lesions affecting the visual pathways at any point from the
retrolaminar portion of the optic nerve to the lateral geniculate body
Lesions anterior to the optic chiasm result in unilateral optic atrophy
Posterior to optic chiasm and optic tract – bilateral optic atrophy
7. Ophthalmoscopic Features Of Primary Optic
Atrophy
a) Chalky white disk
b) Sharply defined margins
c) Lamina cribrosa is well seen
d) Surrounding retina and retinal vessels
and periphery are normal
a) Shallow, saucer shaped cup seen
b) Reduction in number of small blood vessels
on the disc surface - (Kestenbaum index < 6)
8. Causes Of Primary Optic Atrophy
a) Retrobulbar neuritis
b) Compressive lesions of optic nerve
pituitary tumors
meningiomas
gliomas
c) Traumatic optic atrophy
d) Demyelinating diseases
tabes dorsalis
multiple sclerosis
10. Secondary Optic Atrophy
• It is marked degeneration of optic nerve fibers with excessive proliferation of glial
tissue resulting in loss of entire architecture of optic nerve head and is preceded by
swelling or congestion of the optic nerve head.
11. Ophthalmoscopic Features Of Secondary
Optic Atrophy
• Gray or dirty gray pallor of the disk
• Poorly defined margins
• Physiological cup is obliterated and is
filled with proliferating fibroglial tissue
• Peripapillary sheathing and narrowing of arteries
• Veins are tortuous and sometimes narrowed
• Hyaline bodies and drusens in and around the disk
13. Consecutive Optic Atrophy
• It occurs due to destruction of ganglion cells secondary to degenerative or
inflammatory lesions of choroid and/or retina
14. Ophthalmoscopic Features
• Disk has a waxy pallor
• Normal disk margin
• Marked attenuation of arteries
• Associated retinal pathology may be seen
• Normal physiologic cup
15. Causes
• Degenerative—Retinitis Pigmentosa, cerebromacular degeneration, myopia
• Post inflammatory—Choroiditis, chorioretinitis
• Extensive Pan retinal photocoagulation (PRP)
• Long standing Retinal Detachment
16. Cavernous Optic Atrophy
• It is characterized by axonal degeneration without any proliferation of glial tissue
resulting in formation of caverns with marked excavation of the optic disk
• Also called as
• Glaucomatous optic atrophy
• Schnabel’s optic atrophy
17. Ophthalmoscopic Features
• Marked cupping along with vertical enlargement
of cup.
• Lamina cribrosa pores seen (laminar dot sign).
• Bayoneting and nasal shifting of the retinal vessels.
• Peripapillary halo and atrophy
19. a) Hereditary
a) Leber optic atrophy
b) Congenital or infantile optic atrophy (recessive or dominant form)
c) Behr hereditary optic atrophy (autosomal recessive)
b) Consecutive atrophy: due to diseases of the retina and/or choroid
a) Chorioretinitis
b) Pigmentary retinopathies
c) Extensive retinal laser photocoagulation/long standing retinal detachments
c) Vascular: ischemic optic neuropathy (arteritic or non-arteritic).
d) Toxic or drug-induced: tobacco, methyl alcohol, ethambutol, sulphonamides
20. e) Metabolic atrophy: nutritional amblyopia, juvenile diabetes mellitus and thyroid
ophthalmopathy
f) Demyelination: multiple sclerosis
g) Pressure atrophy: diseases such as glaucoma and papilledema.
h) Post-inflammatory: optic neuritis, perineuritis secondary to meningitis.
i) Traumatic optic neuropathy: optic nerve avulsion and transection, optic nerve
sheath hematoma, and optic nerve impingement from a penetrating foreign body
or bony fragment
22. 1. Ascending / antegrade optic atrophy
• Also called Wallerian degenration
• Degeneration begins in the retina and proceeds toward the lateral geniculate body
• Larger axons disintegrate more rapidly than smaller axons.
• (e.g., toxic retinopathy, chronic simple glaucoma)
2. Descending / retrograde optic atrophy
• Degeneration starts from the proximal portion of the axon and proceeds toward the optic disc
• (e.g. optic nerve compression by intracranial tumor).