Diseases of The Vitreous
Diseases of The Vitreous
Diseases of The Vitreous
and Retina
Asamere Tsegaw, MD
Lecture for Optometry students
1
CONGENITAL AND
DEVELOPMENTAL DISORDERS
COLOBOMA OF THE OPTIC DISC
It results from the failure in closure of the embryonic fissure.
It occurs in two forms. The minor defect is more common and
manifests as inferior crescent, usually in association with
refractive error.
The fully-developed coloboma typically presents inferonasally as
a very large whitish excavation, which apparently looks as the
optic disc.
The actual optic disc is seen as a linear horizontal pinkish band
confined to a small superior wedge.
Defective vision and a superior visual field defect is usually
associated.
2
Optic disc coloboma
3
Retinochoroidal coloboma involving the
optic disc
4
HYPOPLASIA OF OPTIC DISC
Hypoplasia of the optic nerve may occur as an isolated anomaly
or in association with other anomalies of central nervous system.
The condition is bilateral in 60% of cases.
It is associated with maternal alcohol use, diabetes and intake of
certain drugs in pregnancy.
It forms a significant cause of blindness at birth in developed
countries.
Diagnosis of mild cases present little difficulty.
In typical cases the disc is small and surrounded by a yellowish
and a pigmented ring; referred to as ‘double ring sign’.
5
Optic nerve hypoplasia-double ring sign
6
MYELINATED NERVE FIBERS
Also known as opaque nerve fibers, represent
covering of nerve fibers of the retina by myelin
sheath.
Normally, the myelination of optic nerve proceeds
from brain downwards to the eyeball and stops at
the level of lamina cribrosa.
Occasionally the process of myelination continues
after birth for an invariable distance in the nerve
fiber layer of retina beyond the optic disc on
Ophthalmoscopic examination.
These appear as a whitish patch with feathery
margins, usually present adjoining the disc margin. 7
MYELINATED NERVE FIBERS
The traversing retinal vessels are partially
concealed by the opaque nerve fibers
Such a lesion, characteristically, exhibits
enlargement of blind spot on visual field
charting.
The medullary sheaths disappear in
demyelinating disorders and optic atrophy
(due to any cause) and thus no trace of this
abnormality is left behind.
8
Different optic discs with mylination
9
VITREOUS LIQUEFACTION(SYNCHYSIS)
10
VITREOUS LIQUEFACTION (SYNCHYSIS)
Clinical features.
On slit-lamp biomicroscopy the vitreous
liquefaction (synchysis) is characterized by visible
pockets of liquefaction associated with appearance
of coarse aggregate material which moves freely in
the free vitreous.
Liquefaction is usually associated with collapse
(synersis) and opacities in the vitreous which may
be seen subjectively as black floaters in front of the
eye.
11
Vitreous collapse (synchisis)
12
Posterior Vitreous Detachment (PVD)
It refers to the separation of the cortical vitreous from
the retina anywhere posterior to vitreous base which is
3-4 mm wide area of strong attachment of vitreous to
the ora serrata.
PVD is a common condition and usually occurs in older
patients in their 60s and 70s but patients with high
myopia can develop PVD at early age.
It occurs in eyes with senile liquefaction (synchysis),
developing a hole in the posterior hyaloid membrane.
The synchytic fluid collects between the posterior
hyaloid membrane and the internal limiting
membrane of the retina, and leads to PVD up to the
base along with collapse of the remaining vitreous gel 13
Posterior Vitreous Detachment (PVD)
Symptoms
Sudden onset of one or more floaters described by
patients as veils (strands in their visual field)
Flash lights at the temporal periphery usually at night
time
14
Floaters as seen by the patient with
vitreous Synchisis and Syneresis
15
Posterior Vitreous Detachment (PVD)
Signs
Slit lamp examination of the vitreous reveals a collapsed
vitreous (synersis) behind the lens
The presence of circular piece of condensed vitreous
separated from the optic nerve is seen under slit lamp
examination. It is called Weiss ring.
Optically empty space behind the posterior hyaloid face on
ultrasound
In patients with PVD the presence of blood in the vitreous
or pigment in the vitreous is suggestive of associated retinal
tear.
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PVD with vitreous synchisis and syneresis
17
Gross appearance of PVD in a phakic
enucleated eye
18
“Weiss” ring in a patient with Posterior Vitreous
Detachment (PVD)
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Posterior Vitreous Detachment (PVD)
Signs…
Ancillary testing
Fundus examination should be supplemented with
indirect ophthalmoscopy with scleral depression
for possible associated retina tear with PVD
Ultrasound examination is essential to diagnose
PVD if there is vitreous hemorrhage
20
Posterior Vitreous Detachment (PVD)
Treatment
No treatment is required for PVD
Patients with PVD and vitreous hemorrhage (with out
retinal detachment) should be closely followed up every
3 monthly for spontaneous clearing of the hemorrhage
If there is persistent hemorrhage, pars plana vitrectomy
with endo laser treatment of the retinal tear is required
All patients with PVD should be warned about the signs
of retinal tear and detachment
21
Asteroid Hyalosis
It is a common vitreous abnormality innumerable
refractile calcium salt deposits suspended through out
the vitreous.
Usually observed in older population and affects men
and women equally.
It is usually unilateral but can some times be bilateral
and more common in patients with diabetes mellitus
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Asteroid Hyalosis
Symptoms
It is usually asymptomatic but occasionally patients
may complain of floaters
Signs
Usually visual acuity is normal and on slit lamp
examination a variable number of yellowish white oval
or round refractile deposits with in the vitreous
uniformly deposited are seen.
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Asteroid Hyalosis viewed through Slit lamp
24
Asteroid Hyalosis viewed through fundus
camera
25
Asteroid Hyalosis
Ancillary tests
FA may be helpful to evaluate the retina when asteroid
hyalosis is very dens
Ultrasound is also helpful for retina evaluation
Treatment
Most patients with asteroid hyalosis are asymptomatic
and do not require treatment
Rarely when patients complain of visual alteration or
fundus evaluation can not be done due to dense
asteroid pars plana vitrectomy can be performed
26
Vitreous Hemorrhage (VH)
Vitreous haemorrhage usually occurs from damaged
retinal vessels and may present as pre-retinal (sub-
hyaloid) or an intravitreal haemorrhage.
The intravitreal haemorrhage may involve anterior,
middle, posterior or the whole vitreous body.
27
Vitreous Hemorrhage (VH)
Causes of VH
1. Spontaneous vitreous haemorrhage from retinal breaks
especially those associated with PVD.
2. Trauma to eye, which may be blunt or perforating (with
or without retained intraocular foreign body) in nature.
3. Inflammatory diseases such as erosion of the vessels in
acute chorioretinitis and periphlebitis retinae primary
or secondary to uveitis.
4. Vascular disorders e.g., hypertensive retinopathy, and
central retinal vein occlusion.
28
Vitreous Hemorrhage (VH)
Causes of vitreous haemorrhage:
5. Metabolic diseases such as proliferative diabetic
retinopathy.
6. Blood dyscrasias e.g., retinopathy of anaemia, leukaemias,
polycythemias and sickle-cell retinopathy.
7. Bleeding disorders e.g., purpura, haemophilia and scurvy.
8. Neoplasms. Vitreous haemorrhage may occur from
rupture of vessels due to acute necrosis in tumours like
retinoblastoma.
9. Idiopathic
29
Vitreous Hemorrhage (VH)
Symptoms and signs
Sudden development of floaters occurs when the
vitreous haemorrhage is small.
In massive vitreous haemorrhage, patient develops
sudden painless loss of vision.
Red blood cells in the vitreous can be seen on slit lamp
examination
Blood may be seen in the vitreous cavity
Over time red blood cells may lose their red color and
migrate to the anterior chamber and cause obstruction
of the anterior chamber angle and Glaucoma
30
Vitreous Hemorrhage (VH)
Fate of vitreous haemorrhage
1. Complete absorption may occur without organization
and the vitreous becomes clear within 4-8 weeks.
2. Organization of haemorrhage with formation of a
yellowish-white debris occurs in persistent or
recurrent bleeding.
3. Complications like vitreous liquefaction,
degeneration and ghost cell glaucoma (in aphakia)
may occur due to blockage of A/C angle by RBC that
has lost their hemoglobin
31
Vitreous Hemorrhage (pre-retinal) due to
proliferative DR
32
Vitreous Hemorrhage (pre-retinal) due
proliferative diabetic retinopathy
33
Chronic VH due to proliferative DR
34
Vitreous Hemorrhage (VH)
Ancillary Tests
Ultrasound is very valuable in the initial evaluation and
follow up of eyes with VH where the retina is not visible
Treatment
1. Conservative treatment consists of bed rest, elevation of
patient’s head and bilateral eye patches. This will allow the
blood to settle down.
2. Treatment of the cause. Once the blood settles down,
indirect ophthalmoscopy should be performed to locate and
further manage the causative lesion such as a retinal break,
phlebitis, proliferative retinopathy, etc.
3. Pars plana Vitrectomy should be considered to clear the
vitreous, if the haemorrhage is not absorbed after 3 months. 35
Peripheral Retinal Degenerations
1. Lattice Degeneration
It is a common inherited congenital anomaly of the
peripheral retina
It has autosomal dominant inheritance pattern and its
incidence is 6 to 10% in general population and 15 to 20%
in myopic patients.
Histologically features include discrete areas of thinning
of the inner retinal layer. Newly formed fibro-cellular
layer overlying the vitrous face and sclerotic vessels
bypassing the lesion can be seen
It is characterised by white arborizing lines arranged in a
lattice pattern along with areas of retinal thinning and
abnormal pigmentation
36
Lattice Degeneration
Clinical features
Is usually asymptomatic unless there is retinal tear
The typical lesion appears as small round oval or
linear islands which are white pigmented or
reddish located between the ora serrata and the
equator with its long axis being circumferentially
oriented.
It more frequently involves the temporal than the
nasal, and superior than the inferior halves of the
fundus.
37
Lattice Degeneration
Clinical features
Occasionally white lines traverse it and tiny atrophic
retinal holes can be seen
It usually is associated with strong vitreo-retinal
adhesion at its posterior edge and this usually leads to
retinal flap tear during PVD
38
Different morphologic forms of Lattice
degeneration
39
Lattice degeneration associated with
atrophic hole
40
Large retinal tear on a lattice degeneration
(arrow show the lattice)
41
Lattice Degeneration
Treatment
The risk of retinal tear in patients with lattice and
PVD is 24%
Indications of prophylactic laser in patients with
lattice degeneration
High myopia
Associated symptomatic retinal tear
Aphakia
Other eye retinal detachment
42
Other Peripheral retinal degenerations and developmental Variations
(reading assignmnet)
43
DYSTROPHIES OF RETINA
A wide variety of dystrophies and degenerations of the
retina have been described and variously classified.
RETINITIS PIGMENTOSA
This primary pigmentary retinal dystrophy is a
commmon hereditary disorder predominantly
affecting the rods more than the cones.
Inheritance
Most common mode is autosomal recessive, followed
by autosomal dominant. X-linked recessive is the least
common.
44
RETINITIS PIGMENTOSA
Incidence
It occurs in 1 persons per 4000 of the world
population.
Age. It appears in the childhood and progresses
slowly, often resulting in blindness in advanced middle
age.
Race. No race is known to be exempt or prone to it.
Sex. Males are more commonly affected than females
in a ratio of 3:2.
Laterality. Disease is almost invariably bilateral and
both the eyes are equally affected.
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Retinitis Pigmentosa
Clinical features
Visual symptoms
1. Night blindness. It is the characteristic feature and may
present several years before the visible changes in the retina
appear. It occurs due to degeneration of the rods.
2. Dark adaptation. Light threshold of the peripheral retina is
increased; though the process of dark adaptation itself is
not affected until very late.
3. Tunnel vision occurs in advanced cases due to degeneration
of the peripheral retina and constricted visual field.
46
Retinitis Pigmentosa
Clinical features
Fundus changes
1. Retinal pigmentary changes.
These are typically perivascular and resemble bone
corpuscles in shape.
Initially, these changes are found in the equatorial
region only and later spread both anteriorly and
posteriorly.
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Retinitis Pigmentosa- bonny specules at
equatorial area
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Retinitis Pigmentosa- severe bonny
specules involving posterior pole
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Retinitis Pigmentosa- severe form involving
posterior pole
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Retinitis Pigmentosa
Fundus changes
2. Retinal arterioles are attenuated (narrowed) and may
become thread-like in late stages.
3. Optic disc becomes pale and waxy in later stages and
ultimately consecutive optic atrophy occurs
4. Other associated changes which may be seen are,
cystoid macular oedema, macular scar or epiretinal
membrane.
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Retinitis Pigmentosa- Waxy disc pallor and
retinal vascular attenuation
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Retinitis Pigmentosa
Visual field changes
Annular or ring-shaped scotoma is a typical feature
which corresponds to the degenerated equatorial zone
of retina.
As the disease progresses, scotoma increases anteriorly
and posteriorly and ultimately only central vision is
left (tunnel vision).
Eventually even this is also lost and the patient
becomes blind.
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Retinitis Pigmentosa-Summary Points
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Retinitis Pigmentosa
Atypical forms of retinitis pigmentosa
1. Retinitis pigmentosa sine pigmento.
It is characterized by all the clinical features of
typical retinitis pigmentosa, except that there are no
visible pigmentary changes in the fundus.
2. Sectorial retinitis pigmentosa. It is characterized by
involvement of only one sector of the retina.
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Atypical Retinitis Pigmentosa-Sectoral
pigmented para-venous type
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Atypical Retinitis Pigmentosa-pigmented
para-venous type
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Retinitis Pigmentosa
Atypical forms of retinitis pigmentosa
3. Pericentric retinitis pigmentosa. In this condition all
the clinical features are similar to typical retinitis
pigmentosa except that pigmentary changes are
confined to an area, immediately around the macula.
4. Retinitis punctata albescens. It is characterised by the
presence of innumerable discrete white dots scattered
over the fundus without pigmentary changes. Other
features are narrowing of arterioles, night blindness
and constriction of visual fields.
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Some syndromes or diseases of which retinitis
pigmentosa is a part include:
Bassen-Kornzweig syndrome (abetalipoproteinemia)
Refsum disease
Friedreich-like ataxia with retinitis pigmentosa
Usher syndrome: types I, II, and III
Laurence-Moon-Bardet-Biedl syndrome
Kearns-Sayre syndrome
Hereditary cerebroretinal degenerations including
Batten disease
Olivopontocerebellar atrophy
Alström disease
Cockayne syndrome
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RetinitisTreatment
Pigmentosa
It is most unsatisfactory and till date there is no
effective treatment for the disease.
1. Measures to stop progression,
Photosolar glasses to reduce excessive light and
UV exposure of the retina
Antioxidant vitamins like low dose Vitamin A
15,000IU po per day
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Retinitis Pigmentosa
Treatment
2. Low vision aids (LVA) in the form of ‘magnifying glasses’
and ‘night vision device’ may be of some help.
4. Prophylaxis. Genetic counselling for no consanguinous
marriages may help to reduce the incidence of disease.
Further, affected individuals should be advised not to
produce children.
5. Experimental treatments- various vitamins and minerals,
vasodilators, tissue therapy with placental extract,
cortisone, cervical sympathectomy, injections of fetal
retinal cells.
None of these has been shown to have proven
therapeutic benefit. 61
Other Dystrophies of the retina (reading
assignment)
Gyrate atrophy
Choroidermia
Stationary forms of night blindness
Leber congenital amaurosis
62
Macular Hole
Idiopathic Macular Holes are full thickness retinal
defects that occur at the macula in less than 1% of the
general population
Occur in 50-80 years of age
More common in females and in 10% of cases it tends
to be bilateral
Pathogenesis of idiopathic MH is due to perifoveal
vitreous detachment resulting in dynamic and static
traction and full-thickness foveolar dehiscence
63
Macular Hole
Other Causes of Macular hole include
Trauma
Myopia
Chronic cystoid macular edema
Tangential traction of Epiretinal membrane
64
Macular Hole
Symptoms
Viable Vision loss depending on the size of the hole,
its location relative to the foveola and any associated
sensory retinal detachment
Associated symptoms include central Scotoma and
metamorphosia
Signs
Varies based on the stage of macular hole at
presentation
65
Normal Foveal Anatomy with OCT
66
Normal Foveal Anatomy with OCT
67
Macular Hole
Stage 1 macular holes also have been referred to as
premacular holes, macular cysts, or involutional
macular thinning.
In a stage 1 macular hole, no true neural retinal defect
is present, the photoreceptor layer is believed to be
intact, and no vitreofoveal separation has occurred.
Stage 1 holes spontaneously resolve in about 50% of
eyes with no visual sequelae
68
Macular Hole- Stages OCT
Stage 1A
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Macular Hole- Stages OCT
Stage-1B
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Macular holes
Stage 2 holes are full-thickness neural retinal defect of
size less than 400μm,
The visual acuity typically is diminished and a pseudo-
operculum, which represents condensed vitreous, may
overlie the hole.
It is believed that once a stage 2 hole occurs, it nearly
always progresses to stage 3, with little hope for
spontaneous visual improvement.
The visual acuity with a stage 2 hole varies between
20/50 (6/15) and 20/400 (6/120).
71
Macular Hole- Stages OCT
Stage-2
72
Macular holes
At stage 3, the hole is developed fully and has the
classic appearance of an idiopathic macular hole.
This consists of a round full-thickness neural retinal
defect of size more than 400μm and vitreo-macular
separation but vitreous still attached at the optic disc
(no complete PVD).
The visual acuity typically is 20/200−20/800
(6/60−6/240);
73
Macular Hole- Stages OCT
Stage-3
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Macular holes
A stage 4 macular hole has all the features of a stage 3
hole, but with complete posterior vitreous
detachment.
75
Macular Hole- Stages OCT
Stage-4
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Natural history of Macular Holes
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Natural history of Macular Holes
78
Macular Hole- Stages
79
Macular Hole- Stages
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Lamellar Macular Hole
A lamellar macular hole represents an aborted macular
hole.
Clinically, a round central inner retinal defect is found,
with no thickening, cystic change, or subretinal fluid. An
overlying operculum is common.
Vitreofoveal separation occurs with loss of the inner
retinal layers; however, the outer, photoreceptor layer is
intact. The vision usually is good (20/20−20/30 (6/6−6/9))
and many patients are asymptomatic.
Fluorescein angiography typically shows no abnormal
fluorescence
81
Lamellar Macular Hole
82
Macular pseudoholes
83
Macular pseudohole with Epiretinal membrane-U-shaped foveal pit with
vertical edges, due to the contraction of
the ERM (arrows)
84
Diagnosis
Usually a clinical diagnosis with slit-lamp
The slit-beam test (Watzke-Allen sign) usually is
reliable to test subjectively for a full-thickness retinal
defect.
In this test, a thin, vertically oriented slit beam is
focused on the macula and the patient is asked to
describe the line of light.
In a full-thickness defect, a break or thinning of the
beam is seen centrally.2
85
Differential diagnosis of MH
86
Treatment of Macular Holes
A lamellar macular hole represents an abortive
macular hole and is stable with good vision.
Stage 1 holes are initially observed for three reasons:
Stage 1 macular holes have a 50% rate of spontaneous
improvement.
Surgical intervention does not prevent macular hole
formation universally, and intraoperative macular hole
can occur.
87
Treatment of Macular Holes
Once a stage 2 hole occurs, without surgery visual loss is
nearly always permanent.
Stages 2 and above macular holes treatment is surgery
Components of macular hole surgery include
Pars plana Vitrectomy
ILM peeling and
Gas temponad and
Strict face down head positioning for 7 days
88
Ocular Disease-II Retina Lectures will
continue
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