3. COLOBOMA OF THE OPTIC DISC
results from failure in closure of the embryonic
Fissure
4. Congenital and developmental disorders
1. Anomalies of the optic disc
Include crescents, congenital pigmentation,
coloboma, drusen and hypoplasia of the optic disc
2. Anomalies of the nerve fibres
Like medullated (opaque) nerve fibres
3. Anomalies of vascular elements
such as persistent hyaloid artery, congenital
tortuosity of retinal vessels
5. MEDULLATED NERVE FIBRES
also known as opaque nerve fibres
represent myelination of nerve fibres of the
retina
Normally medullation of optic nerve proceeds
from brain downwards to the eyeball and stops at
the level of lamina cribrosa
6.
7. PERSISTENT HYALOID ARTERY
Congenital remnants of the hyaloid arterial system
persist in different forms
Bergmester’s papilla refers to flake of
glial tissue projecting from the optic disc
Vascular loop or a thread of obliterated
vessel sometimes is seen running forward into
the vitreous
8.
Mittendorf dot represents remnant of the
anterior end of hyaloid artery, attached to the
posterior lens capsule
9. OPTIC NEURITIS
includes inflammatory and demyelinating
disorders of optic nerve
Etiology
◦ Idiopathic.
◦ Hereditary optic neuritis (Leber’s disease)
◦ Demyelinating disorders multiple sclerosis,
neuromyelitis optica
10. Parainfectious optic neuritis is associated with
measles, mumps, chickenpox, whooping cough
and glandular fever
Infectious optic neuritis sinus related (with
acute ethmoiditis) or associated with cat scratch
fever, syphilis (during primary or secondary
stage), lyme disease and cryptococcal meningitis
in patients with AIDS
Toxic optic neuritis
11. Clinical profile
Anatomical types. three types:
Papillitis. refers to involvement of optic disc
Neuroretinitis refers to combined involvement
of optic disc and surrounding retina in the macular
area
Retrobulbar neuritis characterized by
involvement of optic nerve behind the eyeball
12. Symptoms.
Asymptomatic or associated with following
symptoms:
Visual loss. Sudden, progressive and profound
visual loss is the hallmark
Dark adaptation is lowered
Impairment of colour vision
14. Signs are as follows:
Visual acuity reduced markedly
Colour vision severely impaired
Pupil shows ill-sustained constriction to light
Marcus Gunn pupil sign
15.
16. 4. Ophthalmoscopic features.
hyperaemia of the disc and blurring of the
margins
Retinal veins are congested and tortuous
Splinter haemorrhages and fine exudates on the
disc
inflammatory cells in the vitreous
17. Retrobulbar neuritis fundus appears
normal
Visual field changes.
Common relative central or centrocaecal
scotoma
Contrast sensitivity is impaired
Visually evoked response (VER) shows reduced
amplitude and delay in the transmission time
18. Differential diagnosis
papilloedema and pseudo-papilloedema
Acute retrobulbar neuritis must be differentiated
from malingering, hysterical blindness, cortical
blindness etc.
19. Treatment
Efforts made to find out and treat the underlying
cause
Corticosteroid therapy shorten the period of
visual loss, but will not influence ultimate level of
visual recovery in patients with optic neuritis
20. LEBER’S DISEASE
Hereditary optic neuritis
affects males around age of 20 years
transmitted by the female carriers
characterised by progressive visual failure
fundus is initially normal or in the acute stage disc
may be mildly hyperaemic with telangiectatic
microangiopathy
Eventually bilateral primary optic atrophy ensues
21. TOXIC AMBLYOPIAS
includes conditions wherein visual loss results
from damage to optic nerve fibres due to effects of
exogenous or endogenous poisons
Tobacco amblyopia
men who are generally pipe smokers, heavy
drinkers and have a diet deficient in proteins and
vitamin B complex
hence also labelled ‘tobacco-alcohol-amblyopia
23. Clinical features.
men between 40 and 60 years
characterised by bilateral gradually progressive
impairment in central vision
fogginess and difficulty in doing near work
bilateral centrocaecal scotomas
Fundus examination essentially normal or slight
temporal pallor of disc
24. Treatment.
complete cessation of tobacco and alcohol
consumption
Hydroxycobalamine 1000 μg intramuscular injections
weekly for 10 weeks and care of general health and
nutrition
Prognosis.
It is good, if complete abstinence from tobacco and
alcohol is maintained
Visual recovery is slow and may take several weeks
to months
26. Pathogenesis.
Oxidised into formic acid and formaldehyde
They cause oedema followed by degeneration of
the ganglion cells of the retina, resulting in
complete blindness due to optic atrophy
Clinical features.
Headache, dizziness, nausea, vomiting,
abdominal pain, delirium, stupor and even death
characteristic odour due to excretion of
formaldehyde in the breath or sweat is a helpful
diagnostic sign
27. Ocular features.
Patients are usually brought with almost complete
blindness, which is noticed after 2-3 days, when
stupor weans off.
Fundus examination in early cases mild disc
oedema and markedly narrowed blood vessels,
Finally bilateral primary optic atrophy
28. Treatment
1. Gastric lavage
2. Administration of alkali Soda bicarb given
orally or intravenously (500 ml of 5% solution)
3. Ethyl alcohol. given in early stages. Is given in
small frequent doses, 90 cc every 3 hours for 3
days
29.
4. Eliminative treatment by diaphoresis in the form
of peritoneal dialysis
5. Prognosis is usually poor; death may occur due
to acute poisoning.
Blindness occurs in those who survive
30.
Quinine amblyopia
Clinical features. near total blindness
Deafness and tinnitus may be associated
pupils fixed and dilated
Fundus examination retinal oedema, marked pallor
of the disc and extreme attenuation of retinal vessels
Visual fields markedly contracted
31. Ethambutol amblyopia
Antitubercular drug
toxicity usually occurs in patients who have associated
alcoholism and diabetes
Clinical features.
optic neuritis with typical central scotoma
Optic chiasma bitemporal hemianopia
reduced vision & colour vision during antitubercular
treatment
Fundus examination papillitis
Recovery occurs following cessation
32. ANTERIOR ISCHAEMIC OPTIC
NEUROPATHY
(AION)
refers to segmental or generalised infarction of
anterior part of optic nerve
Etiology. results from occlusion of short posterior
ciliary arteries
Depending upon etiology it may be typified as
follows:
Idiopathic AION.
33. Arteritic AION.
occurs in association with giant cell arteritis
AION due to other causes.
associated with severe anaemia, collagen
vascular disorders, following massive
haemorrhage, papilloedema, migraine and
malignant hypertension
34. Clinical features.
Visual loss is usually marked and sudden
Fundus examination during acute stage reveal
segmental or diffuse oedematous, pale or
hyperaemic disc, usually associated with splinter
haemorrhages
Visual fields altitudinal hemianopia
35. Treatment.
Immediate treatment heavy doses of
corticosteroids (80 mg prednisolone daily) and
tapered by 10 mg weekly
Steroids in small doses (5 mg prednisolone)
continued for a long time (3 months to one year)
36. PAPILLOEDEMA
‘papilloedema’ passive disc swelling associated
with increased intracranial pressure which is
almost always bilateral although it may be
asymmetrical
‘disc oedema or disc swelling’ includes all
causes of active or passive oedematous swelling
of the optic disc
39. Etiopathogenesis of papilloedema
Causes.
secondary to raised intracranial pressure which
may be associated with following conditions:
Congenital conditions aqueductal stenosis and
craniosynostosis
Intracranial space-occupying lesions (ICSOLs).
40.
Intracranial infections such as meningitis and
encephalitis
Intracranial haemorrhages.
Obstruction of CSF absorption via arachnoid villi
which have been damaged previously
Tumours of spinal cord
41.
Idiopathic intracranial hypertension (IIH)
Systemic conditions malignant hypertension,
pregnancy induced hypertension (PIH)
cardiopulmonary insufficiency, blood dyscrasias
and nephritis
Diffuse cerebral oedema from blunt head trauma
42. swelling due to ocular and orbital lesions is usually
unilateral
In majority of the cases with raised intracranial
pressure, papilloedema is bilateral
1. Foster-Kennedy syndrome
associated with olfactory or sphenoidal
meningiomata and frontal lobe tumours
There occurs pressure optic atrophy on side of
lesion and papilloedema on other side (due to
raised intracranial pressure)
43. Pseudo-Foster-Kennedy syndrome
characterised by occurrence of unilateral
papilloedema with raised intracranial pressure
(due to any cause) and a pre-existing optic
atrophy (due to any cause) on other side
44. Pathogenesis. It has been a confused and
controversial issue
Hayreh’s theory is the most accepted one
states that develops as a result of stasis of
axoplasm in the prelaminar region of optic disc,
due to an alteration in the pressure gradient
across the lamina cribrosa
45. Clinical features
[A] General features.
headache, nausea, projectile vomiting and
diplopia
Focal neurological deficit
[B] Ocular features.
history of recurrent attacks of transient blackout of
vision (amaurosis fugax)
Visual acuity and pupillary reactions : normal
Clinical features described under four stages:
early, fully developed, chronic and atrophic
46. 1. Early (incipient) papilloedema
Symptoms usually absent and visual acuity
normal
Pupillary reactions normal
Ophthalmoscopic features
Obscuration of disc margins
Absence of spontaneous venous pulsation at the
disc (appreciated in 80% of the normal individuals)
Mild hyperaemia of disc
Splinter haemorrhages.
Visual fields are fairly normal.
47.
48.
49.
2. Established (fully developed)
papilloedema
Symptoms.
history of transient visual obscurations in one or
both eyes, lasting a few seconds, after standing
Visual acuity is usually normal,
Pupillary reaction remain normal
50. Ophthalmoscopic features
Apparent optic disc oedema is seen as its
forward elevation above the plane of retina
Physiological cup of the optic disc is obliterated.
Disc becomes hyperaemic and blurring of the
margin is present all-around
Multiple soft exudates and superficial
haemorrhages may be seen near the disc
Veins becomes tortuous and engorged
Visual fields enlargement
of blind spot
51.
52.
3. Chronic or long standing (vintage)
papilloedema
Symptoms.
Visual acuity is reduced ????
Pupillary reactions normal
Ophthalmoscopic features
acute haemorrhages and exudates resolve, and
peripapillary oedema is resorbed
Optic disc gives appearance of the dome of a
champagne cork
The central cup remains obliterated
Visual fields Blind spot is enlarged and the visual
fields begin to constrict
55. Ophthalmoscopic features
greyish white discoloration and pallor of the
disc due to atrophy of the neurons and
associated gliosis
Prominence of the disc decreases
Retinal arterioles are narrowed
Whitish sheathing develops around the vessels.
Visual fields Concentric contraction of
peripheral fields
56. Treatment and prognosis
neurological emergency and requires immediate
hospitalisation
As a rule unless causative disease is treatable
or cerebral decompression is done, the course of
papilloedema is chronic and ultimate visual
prognosis is bad
57. OPTIC ATROPHY
refers to degeneration of the optic nerve
occurs as an end result of any pathologic process
that damages axons
Classification
Primary optic atrophy refers to simple
degeneration of the nerve fibres without any
complicating process within the eye e.g., syphilitic
optic atrophy.
Secondary optic atrophy occurs following any
pathologic process which produces optic neuritis
or papilloedema
58. Ophthalmoscopic classification. It is more
useful
Common types are as follows:
Primary (simple) optic atrophy
Consecutive optic atrophy
Glaucomatous optic atrophy
Post-neuritic optic atrophy
Vascular (ischaemic) optic atrophy
59. Ascending versus descending optic atrophy.
Ascending optic atrophy follows damage to
ganglion cells or nerve fibre layer due to disease
of the retina or optic disc
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
60. Etiology
1. Primary (simple) optic atrophy.
multiple sclerosis, retrobulbar neuritis (idiopathic),
Leber’s and other hereditary optic atrophies
61. 2. Consecutive optic atrophy.
occurs following destruction of ganglion cells
secondary to degenerative or inflammatory lesions
of the choroid and/or retina
Its common causes are:
diffuse chorioretinitis, retinitis pigmentosa,
pathological myopia and occlusion of central
retinal artery
3. Postneuritic optic atrophy. It develops as a
sequelae to long-standing papilloedema or
papillitis
62.
4. Glaucomatous optic atrophy. It results from the
effect of long standing raised intraocular pressure
5. Vascular (ischaemic) optic atrophy. It results
from the conditions (other than glaucoma)
producing disc ischaemia
include: giant cell arteritis, severe haemorrhage,
severe anaemia and quinine poisoning
63. Clinical features of optic atrophy
1. Loss of vision, may be of sudden or gradual
onset
2. Pupil is semidilated and direct light reflex is very
sluggish or absent
Swinging flash light test depicts Marcus Gunn
pupil
3. Visual field loss will vary with the distribution of
the fibres that have been damaged
64. 4. Ophthalmoscopic appearance of the disc will
vary with the type of optic atrophy.
In general pallor of the disc and decrease in
the number of small blood vessels (Kastenbaum
index)
Ophthalmoscopic features of different types of
optic atrophy are as described below:
65.
66.
67.
68. Primary optic
atrophy
Conse
cutive
optic
atroph
y
chalky white or white Disc
with bluish hue
appears
yellow
waxy
edges (margins) are edges
sharply outlined
are not
so
sharply
defined
Lamina cribrosa is
clearly seen at the
bottom of the
physiological cup
Major retinal vessels Retinal
and surrounding
vessels
retina are normal
are
attenuat
Post-neuritic
optic atrophy
Glaucomatous
optic atrophy
Optic disc
looks dirty
white in colour
Pale disc
edges are
blurred,.
Ischaemic
optic
atrophy
Edges well defined
physiological cup
is obliterated and
lamina cribrosa is
not visible
vessels are
attenuated and
perivascular
sheathing is often
deep and wide
cupping of the optic
disc and nasal shift
of the blood vessels
Normal
pallor of
the optic
disc
attenuation
of the
vessels
69. Differential diagnosis
1. Non-pathological pallor of optic disc is seen in:
axial myopia, infants, and elderly people sclerotic
changes
physiological
2. Pathological causes of pallor disc (other than
optic atrophy) include:
hypoplasia, congenital pit,and coloboma
70. Treatment
The underlying cause when treated help in
preserving some vision in patients with partial
optic atrophy
once complete atrophy has set in, the vision
cannot be recovered
