NORMAL EYE WITH

SUDDEN DECREASED
VISION
Regan Januardy Marliau
I11109020

Optic Neuritis
Optic neuritis (ON) is a demyelinating inflammation of the optic
nerve that typically first occurs in young adulthood. Many cases
ofONare
associated
withmultiple
sclerosis(MS)
or
neuromyelitis optica (NMO), but ON can occur in isolation.
Optic neuritis, or primary inflammation of the optic nerve, is
referred to as papillitis when the optic disc is swollen and
retrobulbar neuritis when the disc appears normal. The most
common form of optic neuritis is acute demyelinating optic
neuritis.
Loss of vision is the cardinal symptom of optic neuritis and is
particularly useful in differentiating papillitis from papilledema,
with which it may be confused on ophthalmoscopic examination.

Etiology
The most common etiology ismultiple sclerosis.
Up to 50% of patients with MS will develop an
episode of optic neuritis, and 20-30% of the time
optic neuritis is the presentingsign of MS
Most cases of ON are associated with MS, even
though ON can occur in isolation. In MSassociated and isolated, monosymptomatic ON,
the cause is presumed to be an autoimmune
reaction that results in a demyelinating
inflammation of the nerve.

History
The patients history may reveal the following signs and
symptoms of optic neuritis:
Preceding viral illness
Rapidly developing impairment of vision in 1 eye or, less
commonly, both eyes: During an acute attack
Dyschromatopsia (change in color perception) in the
affected eye: Occasionally may be more prominent than the
decreased vision[25]
Retro-orbital or ocular pain: In association with the vision
changes and usually exacerbated by eye movement; the
pain may precede vision loss
Uhthoff phenomenon, in which vision loss is exacerbated by
heat or exercise
Pulfrich phenomenon, in which objects moving in a straight
line appear to have a curved trajectory: Presumably caused
by asymmetrical conduction between the optic nerves

Ocular Manifestations

Visual loss is generally subacute, developing over 27 days.

Color vision and contrast sensitivity are correspondingly
impaired.
Visual loss is usually monocular, although occasionally both
eyes are affected simultaneously, particularly in children.
In over 90% of cases, there is pain in the region of the eye,
and about 50% of patients report that the pain is
exacerbated by eye movement.
Almost any field defect is possible, but with manual
perimetry, a central scotoma is most commonly found. It is
usually circular, varying widely in size and density.

The optic disc appears normal in approximately two thirds

of adults with acute demyelinating optic neuritis
(retrobulbar optic neuritis), while disc swelling is present in
about one third of adult cases (papillitis).
Funduscopic features of optic disc swelling include elevation
of the optic nerve head, disk hyperemia, blurring of the disc
margins, and edema of the nerve fiber layer.[31] Although
the clinical features are similar in both forms, optic disc
hemorrhages were uncommon

Papilitis

Optic papillitisis a specific type ofoptic neuritis.

Inflammation of the optic nerve head (papilla)is called
"papillitis" or "intraocular optic neuritis"

Retrobulbar optic neuritis

A monocular loss of vision that has developed over hours to

days and that is often accompanied by pain on movement
of the eye who shows no abnormalities in eye examination
probably caused by retrobulbar optic neuritis
In retrobulbar optic neuritis, the inflammation and
demyelination occur behind the globe of the eye. The optic
disc appears normal with no signs of swelling or pallor.

Diagnosis

The diagnosis of acute demyelinating optic neuritis is based

on an appropriate history (typical versus atypical course)
and clinical signs, and symptom.
Diagnostic tests, including magnetic resonance imaging
(MRI), cerebrospinal fluid (CSF) analysis, and serological
studies, usually are performed for the following reasons:
To determine if the cause is noninflammatory (such as a
compressive lesion), or a nonidiopathic inflammatory or
infectious process in cases that are not typical for acute
demyelinating optic neuritis.
To determine the prognosis or risk for subsequent
development of MS in monosymptomatic cases for which
the history and clinical signs are typical.

Treatment

Steroid therapyeither intravenously, orally, or by

retrobulbar injectionprobably accelerates recovery of
vision but does not influence the ultimate visual outcome
IV steroids do little to affect the ultimate visual acuity in
patients with ON, but they do speed the rate of recovery.
Oral prednisolone alone did not improve rapidity of visual
recovery and increased the risk of recurrent optic neuritis in
either eye.

Prognosis

Without treatment, vision characteristically begins to

improve 23 weeks after onset and sometimes recovers
within a few days.
A poor visual outcome is also associated with longer lesions
in the optic nerve, especially if there is involvement of the
nerve within the optic canal.
In general, there is close correlation between recovery of
visual acuity, contrast sensitivity, and color vision.

Vitreous Hemorrhage

Vitreous hemorrhage is the extravasation of blood into one

of the several potential spaces formed within and around
the vitreous body.
This condition may result directly from retinal tears or
neovascularization of the retina, or it may be related to
bleeding from pre-existing blood vessels in these
structures.
Hemorrhage into the vitreous cavity
can result in sudden painless loss of
vision. The extent of visual loss will
depend
on
the
degree
of
hemorrhage
A large hemorrhage will cause
total visual loss
A small hemorrhage will
present as floaters and normal
or only slightly reduced visual

Pathophysiology
The vitreous is avascular and inelastic.
Pathological mechanisms of vitreous hemorrhage
include hemorrhage from diseased retina,
traumatic insult, and/or spread of hemorrhage
into the retina and vitreous from any other
intraocular sources.
The most common causes include proliferative
diabetic retinopathy, vitreous detachment with or
without retinal breaks, and trauma. Less common
causes include vascular occlusive disease, retinal
arterial macroaneurysm, hemoglobinopathies,
age-related macular degeneration, intraocular
tumors, and others.

Mechanism of hemorrhage

Abnormal vessels.Abnormal retinal blood vessels are

typically the result of neovascularization due to ischemia in
diseases such as diabetic retinopathy. As the retina
experiences
inadequate
oxygen
supply,
vascular
endothelial growth factor (VEGF) and other chemotactic
factors induce neovascularization. These newly formed
vessels lack endothelial tight junctions, which predispose
them to spontaneous bleeding.

Rupture of normal vessels.Normal vessels can rupture

when sufficient mechanical force overcomes the structural
integrity of the vessel. During a posterior vitreous
detachment, vitreous traction on the retinal vasculature
may compromise a blood vessel. This may happen with or
without a retinal tear or detachment.
Blunt or perforating trauma can injure intact vessels
directly and is the leading cause of vitreous hemorrhage in
people younger than 40.

Blood from an adjacent source.Pathology adjacent to

the vitreous can also cause vitreous hemorrhage.
Hemorrhage from retinal macroaneurysms, tumors and
choroidal neovascularization can all extend through the
internal limiting membrane into the vitreous.
Age-related macula degeneration (AMD)haemorrhage
may occur into the vitreous from the abnormally weak
vessels forming a subretinal neovascular membrane

Sign and symptoms

The symptoms of vitreous hemorrhage are varied but

usually include painless unilateral floaters and/or visual
loss.
Early or mild hemorrhage may be described as floaters,
cobwebs, haze, shadows or a red hue.
More significant hemorrhage limits visual acuity and visual
fields or can cause scotomas. Patients often say vision is
worse in the morning as blood has settled to the back of the
eye, covering the macula.
Patients should be questioned regarding a history of
trauma, ocular surgery, diabetes, sickle cell anemia,
leukemia, carotid artery disease and high myopia.
Complete examination consists of indirect ophthalmoscopy
with
scleral
depression,
gonioscopy
to
evaluate
neovascularization of the angle, IOP and B-scan
ultrasonography if complete view of the posterior pole is
obscured by blood. Dilated examination of the contralateral
eye can help provide clues to the etiology of the vitreous

Treatment

The presence of a retinal detachment may be determined

using ultrasonography if an adequate view of the posterior
segment is not possible.
Treatment is directed at the underlying cause, such as laser
photo-coagulation for proliferative diabetic retinopathy or
for retinal breaks. Occasionally, hemorrhage does not
resolve spontaneously and vitrectomy surgery is necessary
and beneficial.
Vitrectomy is performed urgently when a retinal
detachment or break is identified.

Retinal Artery Occlusion

Retinal arterial obstructions are divided anatomically into

central and branch, depending on the precise site of
obstruction.
A central retinal artery obstruction occurs when the
blockage is within the optic nerve substance itself and
therefore the site of obstruction is generally not visible on
ophthalmoscopy.
Central retinal artery occlusion causes painless catastrophic
visual loss occurring over a period of seconds; antecedent
transient visual loss (amaurosis fugax) may be reported.
Visual acuity ranges between counting fingers and light
perception in 90% of eyes at initial examination

A branch retinal artery obstruction occurs when the site of

blockage is distal to the lamina cribrosa of the optic nerve.
Branch retinal artery is usually embolic in origin and results
in visual field loss. Visual acuity is only reduced if there is
foveal involvement.

Central Retinal Artery

Occlusion
Pathogenesis
The majority of central retinal artery obstructions are caused by
thrombus formation at or just proximal to the lamina cribrosa.
Atherosclerosis is implicated as the inciting event in most cases,
although congenital anomalies of the central retinal artery,
systemic coagulopathies, or low-flow states from more proximal
arterial disease may also be present and render certain individuals
more susceptible.
Inflammation in the form of vasculitis (e.g., varicella infection),
optic neuritis, or even orbital disease (e.g., mucormycosis) may
cause central retinal artery obstruction.
Local trauma that results in direct damage to the optic nerve or
blood vessels may lead to central retinal artery obstruction. Arterial
spasm or dissection rarely produces retinal arterial obstruction. In
addition, systemic coagulopathies may be associated with both
central and branch retinal artery obstructions.

Ocular manifestation

The hallmark symptom of acute central retinal artery

obstruction is abrupt, painless loss of vision.
Within the first few minutes to hours after the obstruction,
the fundus may appear relatively normal. Eventually, the
decreased blood flow results in ischemic whitening of the
retina in the territory of the obstructed artery
A cherry-red spot of the macula is typical and arises in this
area because the nerve fiber layer is thin.

Diagnosis

Diagnosis of central retinal artery obstruction is

straightforward when diffuse ischemic retinal whitening is
present in the setting of abrupt, painless visual loss.
Fluorescein angiography may help if the diagnosis is in
doubt.

Treatment

No proved treatment exists for central retinal artery

obstruction, but treatment strategies center around the
following goals:
Increase retinal oxygenation
Increase retinal arterial blood flow
Reverse arterial obstruction
Prevent hypoxic retinal damage

Branch Retinal Artery

Occlusion
Pathogenesis

Branch retinal artery obstruction is a rare event, even less

common than central retinal artery obstruction overall.
Over two thirds of branch retinal artery obstructions are
secondary to emboli to the retinal circulation. In most
cases, the emboli are clearly visible in the arterial tree.
Emboli to the retinal circulation may originate at any point
in the proximal circulation from the heart to the ophthalmic
artery.
Risk factors reflect the vasculopathic mechanisms that
produce disease within the cardiovascular system. These
include predisposing family history, hypertension, elevated
lipid levels, cigarette smoking, and diabetes mellitus.

Three main types of retinal emboli have been identified:

Cholesterol (Hollenhorst plaque)
Platelet-fibrin
Calcific
Cholesterol emboli typically emanate from atheromatous
plaques of the ipsilateral carotid artery system. They are
yellow-orange in color, refractile, and globular or
rectangular in shape. They may be small and can on
occasion be seen intravascularly without blockage of blood
flow.
Platelet-fibrin emboli are long, smooth, white-colored, intraarterial plugs that may be mobile or break up over time.
Usually, they are associated with carotid or cardiac
thromboses.
Calcific emboli are solid, white, nonrefractile plugs
associated with calcification of heart valves or the aorta.

Ocular manifestation

Abrupt, painless loss of vision in the visual field

corresponding to the territory of the obstructed artery is the
typical history of presentation.
Retinal whitening that corresponds to the areas of ischemia
is the most notable finding. The whitening stops at adjacent
retinal veins, as these vessels mark the extent of the
territory of the retinal arteries

Diagnosis

Ancillary testing is not usually necessary to make the

diagnosis. Fluorescein angiography reveals an abrupt
diminution in dye at the site of the obstruction and distally.
Visual field testing can confirm the extent of visual loss and
may pick up contralateral field loss from previous emboli or
other associated conditions.

Treatment

Warfarin prevents the blood from clotting. This medication

is often used in patients with atrial fibrillation to decrease
their risk of stroke.
To prevent stroke, most patients with branch retinal artery
obstruction (BRAO) are placed on some form of antiplatelet
therapy, such as aspirin, clopidogrel , dipyridamole , and
ticlopidine.

Retinal Vein Occlusion

Venous obstructive disease of the retina is a common

retinal vascular disorder, second only to diabetic
retinopathy in incidence. It typically affects patients 50
years of age or older.
Retinal vein obstructions are classified according to whether
the central retinal vein (Central retinal vein obstruction
(CRVO)) or one of its branches is obstructed (branch retinal
vein obstruction (BRVO)).

Central Retinal Vein

Occlusion

CRVO is also a retinal vascular disease but involves

occlusion of the main central retinal vein. Vascular,
hematologic, and cardiac disease may predispose
individuals to develop CRVO which leads to leakage of blood
and fluid into the retina. In many cases the resultant poor
circulation (ischemia) can lead to abnormal blood vessel
formation in the iris (rubeosis) with painful increases in eye
pressure (neovascular glaucoma).
CRVO can be divided further into ischemic and
nonischemic-varieties. This distinction among types of
CRVO, although somewhat arbitrary, is important because
up to two thirds of patients who have the ischemic variety
develop iris neovascularization and neovascular glaucoma.

Ocular Manifestation

Diagnosis
The diagnosis of an ischemic CRVO is based on the characteristic
fundus findings.
Fluorescein angiography is the most useful ancillary test for the
evaluation of the two most serious, debilitating and, unfortunately,
common
complications
of
CRVO-anterior
segment
neovascularization and macular edema.

Treatment
No known treatment reverses the pathology seen in CRVO. Aspirin;
systemic anticoagulation with coumarin, heparin, and alteplase;
local anticoagulation with intravitreal alteplase; corticosteroids;
anti-inflammatory
agents;
isovolemic
hemodilution;
plasmapheresis; and optic nerve sheath decompression all have
been advocated but without definitive proof of efficacy
Laser photocoagulation is the known treatment of choice in the
treatment of various complications associated with retinal vascular
diseases (eg, diabetic retinopathy, branch retinal vein occlusion).

Branch Retinal Vein

Occlusion

Branch retinal vein obstruction (BRVO) is a common retinal

vascular disorder of the elderly. Visual loss from a branch
retinal vein occlusion usually is caused by macular edema,
macular ischemia, or vitreous hemorrhage. In some
patients, laser treatment can help stabilize or even improve
vision.
It is postulated that a rigid, arteriosclerotic artery
compresses the retinal vein, which results in turbulent
blood flow and endothelial damage, followed by thrombosis
and obstruction of the vein. Most BRVOs occur
superotemporally, probably because this is where the
highest concentration of arteriovenous crossings lies.

Ocular manifestations
Patients with branch retinal vein occlusion usually
complain of sudden onset of blurred vision or a visual field
defect.
Retinal hemorrhages confined to the distribution of a
retinal vein are characteristic for BRVO. As a result of the
distribution, the hemorrhages usually assume a triangular
configuration with the apex at the site of blockage.
Mild obstructions are associated with a relatively small
amount of hemorrhage.
Complete obstructions result in extensive intraretinal
hemorrhages, cotton-wool spot formation, and widespread
capillary nonperfusion.
If the macular region is involved, macular edema,
ischemia, or hemorrhage occurs, which causes decreased
visual acuity.

Branch retinal vein obstruction. Fundus view of extensive retinal hemorrhages in

segmental distribution of a superotemporal retinal vein. Dilated, tortuous veins,
cotton-wool spots, and macular edema also can be seen

Diagnosis
The diagnosis of an acute BRVO is made by finding retinal
hemorrhages in the distribution of an obstructed retinal vein.
Fluorescein angiography is a helpful adjunct for both establishment
of the diagnosis and guidance for the treatment of BRVO.

Treatment
Medical treatment of branch retinal vein occlusion (BRVO) is not
effective. In the past, anticoagulants, fibrinolytic agents, clofibrate
capsules (Atromid-S), and carbogen inhalation have been used but
without success.
Because visual acuity and macular edema may improve
spontaneously, patients were not treated with laser for at least 3
months after the development of the vein obstruction, to allow for
spontaneous improvement. Also, treatment was delayed if the
intraretinal hemorrhage was too dense to allow either
photocoagulation or adequate evaluation with fluorescein
angiography.