OPTIC ATROPHY

Anatomy
Afferent fibres. The optic nerve

carries approximately 1.2 million

afferent nerve fibres which originate
in the retinal ganglion cells.
Most of these synapse in the lateral
geniculate body, although some reach
other centres, notably the pretectal
nuclei in the midbrain.
Nearly one-third of the fibres
subserve the central 5 of the visual
field.

Surrounding sheaths
a The innermost sheath is
the delicate vascular pia mater.
b The outer sheath comprises
the arachnoid mater and the
tougher dura mater which is
continuous with the sclera; optic
nerve fenestration involves
incision of this outer sheath.
The subarachnoid space is
continuous with the cerebral
subarachnoid space and contains

DEFINITION:
It refers to degeneration of

the optic nerve, which occurs

as an end result of any
pathologic process that
damages axons in the anterior
visual system i.e from retinal
ganglion cells to the lateral
geniculate body

CLASSIFICATION:
Primary versus secondary

optic atrophy
Ophthalmoscopic
classification
Ascending versus
descending optic atrophy

PRIMARY VERSUS SECONDARY:

PRIMARY OPTIC ATROPHY:

- it refers to simple
degeneration of the nerve
fibres without any complicating
process within the eye eg:
syplilitic optic atrophy of tabes
dorsalis.

Tabetic optic
atrophy:
Tabes degeneration was due to

chronic inflammation of the pia

which caused a secondary
degeneration of the nerve fibres
commencing in the optic nerve
near the chiasma.
slowly progressive and
prognosis is bad
But with antisyphilitic treatment
disease is rare.

SECONDARY OPTIC ATROPHY:

-it occurs following

any pathologic process which
produces optic neuritis or
papilloedema

OPHTHALMOSCOPIC
CLASSIFICATION
Classified based on

ophthalmoscopic appearance as
follows
1. Primary (simple) optic atrophy
2. Consecutive optic atrophy
3. Glaucomatous optic atrophy
4. Post - neuritic optic atrophy
5. Vascular (ischaemic) optic
atrophy

ASCENDING VERSUS
DESCENDING OPTIC
ATROPHY
Ascending atrophy follows damage to

ganglion cells or nerve fibre layer due

to disease of the retina or optic disc.
In it nerve fibre degeneration
progresses (ascends) from the eyeball
towards the geniculate body
Descending or retrograde optic atrophy
proceeds from the region of the optic
tract, chiasma or posterior portion of
the optic nerve towards the optic disc.

ETIOLOGICAL CLASSIFICATION:
CONSECUTIVE ATROPHY:

-secondary to retinal
disease and destruction of
ganglion cells
post inflammatory: diffuse
chorioretinitis
degenerative: primary pigmentary
and systemic degeneration

PRESSURE AND TRACTION ATROPHY:

- glaucomatous
- postpapilledema, due to swelling and
pressure at disc
-arterial, sclerosed and frequently calcified
artery pressing
upon nerve
- aneurysms of internal carotid
- bony pressure at optic foramen (osteitis
deformans,oxycepaly)
- tumors (optic nerve sheaths, orbit,
cranium)
- imflammatory adhesions (basal
arachnoiditis)
-swelling in nerve (neurofibromatous
degeneration of
v.recklinghausn disease)

CIRCULATORY ATROPHY:

- occlusion of central retinal artery

- post haemorrhagic
- arteriosclerotic, producing ischemic
degeneration
POST INFLAMMATORY ATROPHY:

Optic neuritis,
perineuritis
disseminated sclerosis
neuromyelitis optica
herpes zoster
tabes

TOXIC ATROPHY:

Endogenous toxins: diabetes,

anemia, malignant tumors,
hyperthyroidism
Exogenous toxins: tobacco, alcohol,
arsenic, lead
TRAUMATIC ATROPHY: mechanical
injury
secondary to fracture of skull
ATROPHY OF UNKNOWN ORIGIN:
lebers disease

PATHOLOGICAL FEATURES:
ACUTE NECROSIS:

-occurs following acute trauma or

surgical division of nerve
- at site of injury axons are
destroyed and medullary sheaths
fragmented
- degeneration of all elements of
nerve
- then there is proliferation of
astrocytes and development of
neuroglial scar formation.

SECONDARY DEGENERATION:

- following lesions of optic

nerve there is destruction of
nerve fibres and development of
optic atrophy
-it may be ascending or
decending atrophy

REGENERATION OF OPTIC NERVE:

- fibres grow from both ends of

nerve provided ganglion cells of
retina are intact.
- no successful union of
regenerating fibres due to
absence of neurilemma sheaths.
- after several weeks these
fibres which grow into scar tissue
degenerate.

Degeneration of optic nerve fibres

is associated with attempted but

unsuccessful regeneration which is
characterised by proliferation of
astrocytes and glial tissue.
Ophthalmoscopic appearance of
the atropic disc depends upon
balance between loss of nerve
tissue and gliosis

Following three
situations may occur:
1) Degeneration of the nerve fibres may

be associated with excessive gliosis.

Occurs in consecutive and postneuritic optic atrophy
2) Degeneration and gliosis may be
orderly and the proliferating
astrocytes arrange themselves in
longitudinal columns replacing the
nerve fibres (columnar gliosis)
occurs in primary optic atrophy

3) Degeneration of the optic nerve

fibres may be
associated with negligible gliosis
- occurs due to progressive
decrease in blood
supply
- such pathological changes are
known as cavernous optic
atrophy
- occurs in glaucomatous and
ischaemic (vascular) optic atrophy

ETIOLOGY:
PRIMARY (SIMPLE) OPTIC

ATROPHY:
-occurs due to lesions
proximal to the optic disc
without antecedent
papilloedema.
-It may be caused by lesions
affecting the visual pathways
from the retrolaminar portion of

Causes: 1. multiple sclerosis

2. retrobulbar neuritis(idiopathic)
3. lebers and other hereditary
optic atrophies
4. intracranial tumors (pressing
directly on anterior visual
pathway)
5. traumatic severance or
avulsion of the optic nerve
6. toxic amblyopia (chronic

CONSECUTIVE OPTIC ATROPHY:

occurs following destruction of ganglion

cells secondary to degenerative or
inflammatory lesions of the choroid and /or
retina.
Causes: 1) diffuse chorioretinitis
2) retinal pigmentary dystrophies
(retinitis pigmentosa)
3) pathological myopia
4) occlusion of central retina
5) old vasculitis
6) retinal necrosis
7) excessive retinal photocoagulation.

POSTNEURITIC OPTIC ATROPHY:

- it is also known as secondary

optic atrophy 1
-develops as a sequelae to
long standing papilloedema or
papillitis
- Causes include chronic
papilloedema, anterior
ischaemic optic neuropathy and
papillitis.

GLAUCOMATOUS OPTIC ATROPHY:

results from the effect of long

standing raised intraocular pressure
VASCULAR (ISCHAEMIC) OPTIC

ATROPHY: results from the conditions

(other than glaucoma) producing disc
ischaemia.
Causes : 1) giant cell arteritis
2) severe haemorrhage
3) severe anaemia
4) quinine poisoning

CLINICAL FEATURES:
LOSS OF VISION : -sudden or

gradual(depend on cause)
- partial or total (depending on degree
of atrophy)
-ophthalmic signs cannot be correlated
with amount of vision
PUPIL:
semidilated and direct light
reflex is very sluggish
or absent
marcus gunn pupil on swinging
flash light test

VISUAL FIELD:- vary with the

distribution of the fibres that

have been damaged
- field loss is peripheral in
systemic infections,
central
in focal optic neuritis and
eccentric when nerve or
tracts compressed
OPHTHALMOSCOPIC APPEARANCE:
-varies with the type of optic
atrophy
- pallor of the disc and decrease
in no of small blood
vessels

PRIMARY OPTIC ATROPHY:

-disc is chalky white or white with

bluish hue
-edges sharply outlined
-slight recession of entire optic disc
in total atrophy and the temporal side
of disc in total optic atrophy
-lamina cribrosa is clearly seen at
the bottom of the physiological cup
-major retinal vessels and
surrounding retina normal

-Reduction in the number of small blood

vessels on the disc surface (Kestenbaum sign).

-Attenuation of peripapillary blood vessels

and thinning of the retinal nerve fibre layer.

-The atrophy may be diffuse or sectoral

depending on the cause and level of the lesion.

-Temporal pallor may indicate atrophy of

fibres from the papillomacular bundle, which
enters the optic nerve head on the temporal
side.
- Band atrophy caused by involvement of
the fibres entering the optic disc nasally and
temporally with sparing of the superior and
inferior portions occurs in lesions of the optic
chiasm or tract

POST-NEURITIC OPTIC ATROPHY:

-optic disc dirty white in colour

-due to gliosis edges are blurred,
physiological cup obliterated and lamina
cribrosa not visible
- retinal vessels are attenuated and
perivascular sheathing present
- hyaline bodies (drusen) may be
present on or about the disc

CONSECUTIVE OPTIC

ATROPHY:
-disc appears yellow waxy
-edges are not so sharply
defined as in optic
atrophy
-retinal vessels are
attenuated

GLAUCOMATOUS OPTIC

ATROPHY:
-deep and wide cupping of
the optic disc and nasal
shift of the blood vessels
ISCHAEMIC OPTIC ATROPHY:
- pallor of optic disc
associated with marked
attenuation of the vessels

DIFFERENTIAL DIAGNOSIS:
Pallor of the optic disc does not signify

optic atrophy unless there is

dmonstrable defect in vision or in the
visual field.
PATHOLOGICAL PALLOR OF OPTIC DISC:

hypoplasia
congenital pit
coloboma

NON-PATHOLOGICAL PALLOR OF

OPTIC DISC:
axial myopia
infants
elderly people with sclerotic
changes
temporal pallor associated with
large physiological cup

PROGNOSTIC FACTORS:
Atrophic cupping:

-if present and involves entire disc, visual

acuity is decreased and indicates bad
prognosis
Attenuation of arteries:
- is a sign of bad prognosis
- but transient narrowing (angiospasm)
does not
indicate poor prognosis
Papilledema combined with pallor of the
discs:
- indicates poor prognosis for vision

Glaucoma and optic atrophy:

- when glaucoma advanced to stage of

glaucomatous atrophy , poor prognosis seen
wheather ocular tension lowered or not
Optic atrophy and intracranial tumors, brain

abscess, arachnoiditis:
- pallor of disc and visual field defects
does not indicate poor prognosis
- in such cases if there is absence of
atrophic cupping and if retinal vessels are
not narrowed, operative treatment results in
good improvement

Optic atrophy and the

demyelinizing diseases :
-occurs in disseminated
sclerosis, encephalomyelitides
and neuromyelitis optica
- prognosis is good in these
conditions
Parenchymatous optic atrophy :

- is seen in tabes dorsalis

- normal fundus with pallor of

TREATMENT:
In case of partial optic

atrophy underlying cause

when treated helps in
preserving some vision.
Once complete atrophy sets
in, vision cannot be
recovered.

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