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Vogt-Koyanagi-Harada (VKH)

Disease is defined as a bilateral granulomatous panuveitis with or without extraocular


manifestations affecting young adults.
Originally, VKH disease was classified as two separate entities:

 Vogt-Koyanagi syndrome , characterized by chronic severe anterior uveitis, alopecia,


poliosis, cutaneous as well as perilimbal vitiligo (also known as Sugiura’s sign), and
dysacusia.
 Harada’s disease, characterized by bilateral exudative uveitis accompanied by pleocytosis
of cerebrospinal fluid.
Since there is much overlapping of signs and symptoms between the two entities, in 1932
Babel suggested to call the entity Vogt-Koyanagi-Harada disease.

Signs and Symptoms


Headaches, fever, orbital pain, nausea,
dizziness and light sensitivity are
present.
The symptoms will last around 3-5 days.
Within the first couple of days the
patient will begin to complain about
blurred vision, photophobia, hyperemia
of the conjunctiva, and ocular pain

circumscribed retinal edema, multiple


exudative retinal detachments of
posterior retina, often with optic disc
hyperemia and edema

Bietti’s Crystalline Dystrophy (BCD)


is a rare autosomal recessive ocular disease that involves yellow-white crystalline lipid
deposits in the retina and sometimes cornea, degeneration of the retinal pigment epithelium
(RPE), and sclerosis of the choroidal vessels. Progression of the disease ultimately results in
reduced visual acuity, night blindness, visual field loss, and impaired color vision. Onset of
the disease can occur from early teenage years to third decade of life, but can also occur
beyond the third decade. As the disease progresses, decreases in peripheral acuity, central
acuity or both ultimately results in legal blindness in most patients

Glistening crystalline-like
lesions at posterior pole of
retina in patient with Bietti
crystalline dystrophy. Fundus
also shows atrophic-like changes
of RPE and patchy atrophic
change of choriocapillaris
vessels. Clumping of retinal
pigment is also apparent

Age-Related Macular Degeneration (ARMD)


is an acquired degeneration of the retina that causes significant central visual impairment
through a combination of non-neovascular (drusen and retinal pigment epithelium abnormalities),
and neovascular derangement (choroidal neovascular membrane formation)

Classification

 Early ARMD: Defined by the presence of numerous small (<63 microns, “hard”) or intermediate
(≥63 microns but <125 microns, “soft”) drusen. Note: Small drusen are frequently seen in
those 50 and older, and can represent an epiphenomenon of aging (therefore, intermediate
drusen are more specific for ARMD).
 Intermediate ARMD: Macular disease characterized by either extensive drusen of small or
intermediate size, or any drusen of large size (≥125 microns).
124 micron is the average diameter of retinal vein at the optic disc margin

 Advanced ARMD: Defined by the presence of either geographic atrophy or choroidal


neovascular membrane (along with its sequelae, such as subretinal or sub-RPE hemorrhage or
serous fluid, and subretinal fibrosis).[4] The figure below demonstrates progressive
geographic atrophy over a 29-month period. There is also an area of choroidal
neovascularization along the inferior aspect of the scar with subretinal fibrosis that
progresses over these 29 months.
Signs and Symptoms
 Blurry or fuzzy vision.
 Difficulty recognizing
familiar faces.
 Straight lines appear wavy.
 A dark, empty area or blind
spot appears in the center of
vision.
 Loss of central vision, which
is necessary for driving,
reading, recognizing faces
the formation of drusen, pigmentary changes at and performing close-up work.
the macula, and mild to moderate vision loss.

Epiretinal Membrane (Erm)


is a fibrocellular tissue found on the inner surface of the retina. It is semi-translucent and
proliferates on the surface of the internal limiting membrane.

a cellophane sheen sheet on or above the surface of


the retina with macular pucker, distortion of blood
Cotton Wool Spot (CWS) vessels within vessel arches
in otherwise healthy patients, the observance of a cotton wool spot (CWS) is not considered
normal. A single cotton wool spot in one eye can be the earliest ophthalmoscopic finding
in diabetic or hypertensive retinopathy. In a series of patients who had cotton-wool spots and
no known medical history, diastolic blood pressure equal to or greater than 90 mmHg was
detected in 50% of patients, and an elevated blood sugar was found in 20% of patients

SIGNS AND SYMPTOM

acute signs of vascular


insufficiency to an area of
retina. They have been
described in many
conditions, but only
occasionally cause symptoms
in patients. The most
small, whitish, fluffy
superficial lesions in the
post-equatorial fundus

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.

SIGNS AND SYMPTOM

The symptoms of optic


atrophy relate to a change
in vision, specifically:

 Blurred vision.
 Difficulties with
peripheral (side)
vision.
 Difficulties with
color vision.
 A reduction in
sharpness of vision.
white disc, reduction of small
vessels on the disc, attenuation of
peripapillary vessels and thinning
of RNFL, sometimes with Paton lines

Terson Syndrome
is now recognized as intraocular hemorrhage associated with SAH, intracerebral hemorrhage, or
traumatic brain injury. Hemorrhage may be present in the vitreous, sub-hyaloid, subretinal
space, or beneath the internal limiting membrane

SIGNS AND SYMPTOM

include decrease of visual


acuity following
generalized symptoms such
as headache, loss of
consciousness and
meningealsigns
Terson syndrome can present with dome-
shaped hemorrhages in the macula [27].
A macular “double ring” sign may be seen
with the inner ring caused sub-ILM
hemorrhage and the outer ring caused by
sub-hyaloid hemorrhage

Diabetic Retinopathy
is a diabetes complication that affects eyes. It's
caused by damage to the blood
vessels of the light-sensitive tissue at
the back of the eye (retina).

SIGNS AND SYMPTOM


Dark or empty areas in
your vision,
fluctuating vision, spots
or dark strings floating
in your vision
(floaters)

diabetic retinopathy
include microaneurysms, hard
exudates, macular edema (diabetic
macular edema or DME), and new vessels
(in proliferative DR or PDR)

Retinal Tears
is the retina is the
inner lining of the eye; it is the thin,
light-sensitive tissue that generates
vision. Tears can form in the retina,
creating a risk of retinal
detachment and severe loss of vision.

SIGNS AND SYMPTOM

A patient with
an acute retinal tear may
experience
the sudden onset of black
Apparance of many floaters- tiny
specks that seem to drift through
your field of vision

Ocular Hypertension
is when the pressure of fluid in the eye becomes
high. This alone may not cause
symptoms, but without treatment, it
can damage the optic nerve and lead
to glaucoma.
SIGNS AND SYMPTOM
people with
ocular hypertension usually
have no
symptoms.

arteriolar
constriction, arteriovenous
nicking, vascular wall changes,
flame-shaped hemorrhages, cotton-
wool spots, yellow hard exudates,
and optic disk edema.

SIGNS AND SYMPTOM


Retinitis pigmentosa (RP) loss of depth
perception, photopsia (Spontaneously
is a genetic disorder of the eyes that occurring
causes loss of vision. Symptoms flashes/blinking/swirling/shimmer
include trouble seeing at night and ing lights), Photophobia (aversion to
decreasing peripheral vision (side bright lights)
and upper or lower visual field)

mid stage (Bone spicule-shaped


pigment deposits are present in the
mid periphery along with retinal
atrophy, while the macula is
preserved although with a peripheral
ring of depigmentation. Retinal
vessels are attenuated.)
Stargardt Disease
is the most common inherited single
gene retinal disease. In terms of the
first description of the disease, it
follows an autosomal
recessive inheritance pattern

Fundus flecks are pisiform, round or


dot-like yellow-white lesions typically
SIGNS AND SYMPTOM found in Stargardt patients and should
The most common symptom of Stargardt be seen at a given point in order to
disease is a slow loss of central vision consider the clinical diagnosis.
in both eyes.
.
 Gray, black, or hazy spots in the
center of your vision.
 Sensitivity to light.
 Needing more time for your eyes to
adjust between light and dark
places.
 Color blindnes

Optic Nerve Drusen


is the optic nerve that has physical
connection between
the eye and the brain. Visual
information received by the eye is
transmitted to the brain along the
optic nerve

SIGNS AND SYMPTOM

 Elevated, often small, optic


disc with indistinct and
irregular disc margins
 Drusen seen as round,
white/yellow refractile
bodies on the surface of the having a "lumpy, bumpy"
nerve or buried beneath it appearance. The drusen appear as
 Anomalous vascular branching round, refractile bodies which
pattern (tortousity, may be yellow or white in color
optociliary shunt vessels) and located on the surface of or
 Nasal margin is most common
site of drusen
buried under the nerve.
 Spontaneous venous pulsations
often seen
 Afferent pupillary defect if
there is asymmetric nerve
Neuromyelitis Optica (NMO)
is an inflammatory disease that causes demyelination of the central nervous system, primarily
affecting the optic nerve (optic neuritis) and the spinal cord.

SIGNS AND SYMPTOM

 eye pain.
 loss of vision.
 colours appearing faded
or less vivid.
 weakness in the arms
and legs.
 pain in the arms or
legs – described as
sharp, burning,
shooting or numbing.
 increased sensitivity
to cold and heat.
 tight and painful
muscle spasms in the
arms and legs.
 vomiting Segmented demyelination and inflammation of
the spinal cord and the optic nerves
inducing axonal loss and perivascular
lymphocytic infiltration.
Macular Hole
is when a circular opening forms in your macula. As the hole forms, things in your central
vision will look blurry, wavy or distorted. As the hole grows, a dark or blind spot appears in
your central vision. A macular hole does not affect your peripheral (side) vision
SIGNS AND SYMPTO

Depending on the stage of


the MH, a subfoveal
lipofuscin-color spot or
ring can be noted. In more
advanced cases, a partial
or full-thickness macular
break is observed.

Metamorphopsia (distortion
of the central vision),
central visual loss, or
central scotoma can be
reported.
A macular hole (MH) is a retinal
break commonly involving the fovea.

Papilledema
is a term that is exclusively used when a disc swelling is secondary to increased intracranial
pressure (ICP). It must be distinguished from optic disc swelling from other causes which is
simply termed "optic disc edema

SIGNS AND SYMPTOM


Fleeting vision changes—
blurred vision, double
vision, flickering, or
complete loss of vision—
typically lasting seconds
are characteristic of
papilledema. Other symptoms
may be caused by the
elevated pressure in the
brain. A pulsating
whooshing noise in the
ears, headache, nausea,
vomiting, or a combination
may occur.

Papilledema is a term that is


exclusively used when a disc
swelling is secondary to increased
intracranial pressure (ICP).
Rhegmatogenous Retinal Detachment
is the most common type of retinal detachment. It can happen if you have a small tear or break
in your retina. When your retina has a tear or break, the gel-like fluid in the center of your
eye (called vitreous) can get behind your retina.

SIGNS AND SYMPTO


Early symptoms of
rhegmatogenous detachment
may include dark or
irregular vitreous floaters
(particularly a sudden
increase), flashes of light
(photopsias), and blurred
vision.

slightly opaque, convex or corrugated


appearance of elevated retina,
sometimes with breaks in view

Cherry Red Spot


is a significant fundoscopic finding in the macula, observed in central retinal artery
occlusion (CRAO) and a variety of lipid storage disorders.

a red-tinted region at the center of the


macula surrounded by retinal opacification.
Cherry-red spots at the macula may be
present in various pathologic conditions, SIGNS AND SYMPTOM
including lysosomal storage disorders,
retinal ischemia, and retinal infarction  Sudden onset painless vision
loss occurring over several
Variants of the "cherry-red spot' sign in
different ethnicitie

Central retinal vein occlusion (CRVO)


is a condition in which the main vein that drains blood from the retina closes off partially or
completely. This can cause blurred vision and other problems with the eye.

SIGNS AND SYMPTOM

Presentation is with
sudden, unilateral blurred
vision. In non-ischemic
CRVO, the blurring is mild
and may be worse on waking
and improves during the
day. In ischemic CRVO,
show retinal hemorrhages, dilated visual impairment is sudden
tortuous retinal veins, cotton-wool and severe
spots, macular edema, and optic
disc edem
Branch Retinal Vein Occlusion
The eye's retina has one main artery and one main vein. When branches of the retinal
vein become blocked, it is called branch retinal vein occlusion (BRVO). When the vein is
blocked, blood and fluid spills out into the retina. The macula can swell from this fluid,
affecting your central vision

SIGNS AND SYMPTOM


Typically, patients present
with sudden painless vision
loss or visual field defect.
Rarely, patients present with
floaters from vitreous
hemorrhage if the initial vein
occlusion was asymptomatic and
retinal neovascularization had
developed.

Branch retinal vein occlusion of superotemporal


branch vein in the right eye. Fundus photograph
showing widespread haemorrhages and axonal
congestion (cotton wool spots; white circles)
upstream of the venous occlusion
FUNDUS
PLATES
CLINICAL OPTOMETRY PRACTICE
2
RAÑON, LYAN ANACLETO Q.

DOPT- 3A
Coloboma
is derived from the Greek koloboma, meaning mutilated, curtailed, or with defect. The term is
used to describe ocular defects of the eyelids, iris, lens, ciliary body, zonules, choroid,
retina or optic nerve. It is typically located in the inferonasal quadrant of the involved
structure and is often associated with microphthalmia. It can affect one eye (unilateral) or
both eyes (bilateral).

SIGNS AND SYMPTOM

Some people with coloboma


have no symptoms. Others may
have problems with their
vision, including:
 Vision loss or blindness
 Low vision
 Sensitivity to light
Some colobomas are visible.
For example, iris coloboma
can make the pupil (the round
opening at the center of the
iris) look more like a
keyhole or teardrop
Coloboma of the iris, ciliary body, choroid,
retina and/or optic nerve derive from failed or
incomplete closure of the embryonic fissure
(also known as choroidal or optic fissure)
during development. Eye colobomas are located
in the inferior nasal quadrant

Epiretina

SIGNS AND SYMPTOM


A sheen or abnormal reflectivity
of the macular surface is
suggestive of an ERM. More
SIGNS AND SYMPTOM advanced ERMs can become opaque.
 Blurry or hazy vision.
 Trouble seeing in low light. Metamorphopsia, blurred vision,
monocular diplopia, and micropsia
 Not being able to see things can be noted with any macular
out of the corner of your eye pathology. The vast majority of
(peripheral vision) patients with ERMs are
 Trouble seeing certain colors asymptomatic.
 They experience a loss of
sharp vision (reduction in
visual acuity) and difficulty
seeing in dim light (night
blindness)

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