THE RETINA

RETINAL DISEASES

Rarely occur in isolation

Frequently affected by systemic diseases /
RETINOPATHIES
VASCULAR Diseases
INFLAMMATORY Diseases
DEGENERATIVE Conditions
Retinal Detachment
Tumors- Retinoblastoma
Macular disorders
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HYPERTENSIVE RETINOPATHY
Modified Keith, Wagner & Barker (1939)
Classification

Correlates directly with degree of systemic HTN and

inversely with prognosis for survival
GRADE I: Mild to moderate narrowing/ sclerosis of
smaller arterioles (generalized arteriolar attenuation)

GRADE II: Moderate to marked narrowing, broadening

of the arteriolar light reflex & AV crossing changes

GRADE III: Retinal arteriolar narrowing, focal

constriction & AV crossing changes, retinal oedema,
cotton wool patches, flame shaped haemorrhages.

GRADE IV: All above features + Papilloedema

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HYPERTENSIVE
RETINOPATHY

HYPERTENSIVE
RETINOPATHY

HYPERTENSIVE
RETINOPATHY

HYPERTENSIVE
RETINOPATHY

HYPERTENSIVE
RETINOPATHY

Grading of Arterio-sclerotic changes:

Grade I- Broadening of arteriolar light reflex

Grade II- Arterio-venous crossing defects
Grade III- Copper wire appearance
Grade IV- Silver wire appearance

HYPERTENSIVE
RETINOPATHY

Arterio-venous Crossing changes:

Salus sign
Gunns sign
Bonnets sign / Distal banking

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RETINAL ARTERIAL OCCLUSIVE DISEASE

Obstruction of arteriolar circulation

Embolus + Superadded spasm Complete obstruction

Atheroma at bifurcation of Carotid artery
Diseased Mitral/ Aortic valve

Embolus- Common

From the heart

Calcific from valves

Vegetations from valves; esp. in Bacterial endocarditis
Thrombus - MI

From carotid artery

Cholesterol emboli (HOLLENHORST PLAQUES) frequently

asymptomatic
Fibrino-platelet emboli Retinal TIA/ Amaurosis fugax, may be
ass. with cerebral TIA on same side and contralateral signs
Calcific emboli

CENTRAL RETINAL ARTERY OCCLUSION

Other Causes:
Systemic hypertension seen in two thirds of patients
Diabetes mellitus
Cardiac valvular disease seen in one fourth of patients

Atherosclerotic changes
Giant cell arteritis
Hypercoagulable state
Rare causes - Consider in younger patients
Behet disease
Syphilis
Sickle cell disease
Migraine
Hydrostatic arterial occlusion

CENTRAL RETINAL ARTERY OCCLUSION

Occurs at lamina cribrosa

Sudden and complete retinal ischaemia,
tissue rapidly dies and vision is lost
Fundus:

Pale cloudy retina

Arteries thread like
Veins slightly constricted near the disc
Cherry red spot at the macula

CENTRAL RETINAL ARTERY OCCLUSION

CENTRAL RETINAL ARTERY OCCLUSION

Clinical features:
Painless catastrophic loss of vision in seconds
Amaurosis fugax ( transient visual loss ).
VA: CF to PL+ ( 90% )
Afferent pupillary defect
Cherry red spot: opacification of superficial NSR
Macular sparing in 25% due to cilio-retinal artery
Attenuated retinal arterioles
Cattle tracking / Boxcar segmentation can be seen
in both arteries and veins. This is a sign of severe
obstruction.

CENTRAL RETINAL ARTERY OCCLUSION

TREATMENT Rarely helps

To be done within 6 -Relieve spasm and drive embolus into

less important branch
Firm ocular massage in supine position IOP &Blood
flow Dislodges embolus
I/V Acetazolamide or Mannitol
Hyperbaric oxygen
Paracentesis
Peripheral vasodilators (Inhalation of Amylnitrite,
pentoxifylline/ Sublingual Isosorbide)

Invasive treatment: selective catheterization of the ophthalmic

artery with administration of fibrinolytic drugs.

Results are often disappointing

Branch retinal artery occlusion. Multiple

emboli are visible in of the affected
arterial branches (arrows).

CENTRAL RETINAL VEIN OCCLUSION

Common & easily diagnosed

Clinical features:

variable visual loss

retinal hemorrhages,
dilated tortuous retinal veins,
cotton-wool spots,
macular edema,
and optic disc edema.

Recent onset central retinal vein occlusion, showing

extensive hemorrhages in the posterior pole giving the
"blood and thunder appearance"

CENTRAL RETINAL VEIN OCCLUSION

Predisposing factors:
Hypertension
Cardiovascular diseases
Hypercoagulability
Hyperopia
High IOP
Broadly, CRVO can be divided into 2 clinical types,
ISCHEMIC AND NONISCHEMIC. All these
classifications take into account the area of retinal
capillary nonperfusion and development of
neovascular complications.

CENTRAL RETINAL VEIN OCCLUSION

Complications:
Macular edema > Cystoid macular edema
Reduced VA
Neovascular glaucoma
IVFA:
Non-ischemic:
Ischemic: , areas of capillary non-perfusion
: 50% develop NVG

CENTRAL RETINAL VEIN OCCLUSION

A number of clinical and ancillary

investigative factors are taken into account
for classifying CRVO, including vision at
presentation, presence or absence of relative
afferent pupillary defect, extent of retinal
hemorrhages, cotton-wool spots, extent of
retinal perfusion by fluorescein angiography
and electro-retino-graphic changes.

CENTRAL RETINAL VEIN OCCLUSION

Nonischemic CRVO is the milder form of the

disease.
It may present with good vision, few retinal
hemorrhages and cotton-wool spots, no
relative afferent pupillary defect, and good
perfusion to the retina. Nonischemic CRVO
may resolve fully with good visual outcome or
may progress to the ischemic type.

Ischemic CRVO (hemorrhagic

CRVO) with marked
hemorrhages and edema

CENTRAL RETINAL VEIN OCCLUSION

Treatment:
PRP to prevent NVG
Macular grid for macular edema

BRANCH RETINAL VEIN

OCCLUSION

Occlusion of the inferior

temporal vein

Peripheral Retinal
Degeneration

Microcystoid degeneration:Tiny vesicles with indistinct

boundaries on a grayishwhite background.
Snowflakes Degeneration:minute glistering yellowwhite dots which are
frequently found scattered
diffusely in the peripheral
fundus.

RETINAL DEGENERATION

BENIGN (Do not cause retinal breaks)

Snow flake: near ora serrata
Paving stone: Focal chorioretinal atrophy
Reticular pigmentary
Equatorial drusens
Peripheral microcystic

Retinal Tears
U-Tears

(arrowhead tears):- Apex of which is

pulled anteriorly by the vitreous, the base
remaining attached to the retina.
Incomplete U- Tears:May be linear, LShaped, I-Shaped.

DETACHMENT OF RETINA

Separation of neuro-sensory retina from RPE

d/t accumulation of SRF in potential space

Depending on mechanism of SRF collection

Retinal Detachments are classified into
Rhegmatogenous
Tractional
Exudative

RHEGMATOGENOUS RD
Secondary to a full thickness defect in the
sensory retina which permits SRF derived
from synchitic (Liquified) vitreous gel to gain
access to this subretinal space.

RHEGMATOGENOUS RD

Commonest cause
Types of breaks

Horse shoe shaped tears: Periphery,> in upper quadrants

Round holes: Less peripheral and also in macula
Giant tears: Involving >1/4 of retina
Dialysis/Disinsertion: Large, sharply defined & choroid
shines through

Predisposing factors

Myopia
Previous intraocular surgery
Family H/O RD
Trauma
Inflammations

RHEGMATOGENOUS RD

Clinical features
Shallow RD- non specific D/V as retina gets some
nourishment from SRF
Transient flashes of light especially seen nasally
Increase in no. of black spots
Curtain/ Veil obscuring field of vision
Gross D/V when macula affected or large bullous
RD

Retinal detachment in the

temporal mid-periphery without
macular involvement

Rhegmatogenous retinal
detachment involving the
macula.

Total retinal detachment

RHEGMATOGENOUS RD Management

Retinal breaks to be localized

Prophylaxis

50% cases have > 1 break

Upper temporal quadrant affected the most
Early detection of breaks & treatment by cryotherapy or
photocoagulation and take care of risk factors
Long term follow up by repeated I/O and examination with 3
mirror CL to detect further breaks

Surgical Management depends upon extent,

duration and condition of retina.

Horseshoe-like break with

bridging vessel
surrounded by laser spots

Principles of management
Identification of breaks and traction if present
Sealing of breaks
Release of traction ( Vitreal/ Pre- retinal)
Drainage of SRF
Ensuring chorio-retinal apposition for atleast 2
weeks by

External tamponade (silicon buckle, tyre, sponge

or bands)
Internal tamponade ( Air, gas -sulpur hexafluoride,
perfluorocarbons or liquids as silicone oil)

PARS PLANA VITRECTOMY

Non - Rhegmatogenous RD
Tractional:- Sensory retina is pulled away
from the RPE by contracting vitreoretinal
membrane the source of SRF is unknown.
Exudative:-SRF derived from the
choriocapillaries gains access to the
subretinal spaces.

TRACTIONAL RETINAL
DETACHMENT
Second most common
Etiology:
PDR
PVR
ROP
Trauma

EXUDATIVE RETINAL
DETACHMENT
Fluid collection beneath Retina and choroid
Etiology:
Degenerative macular disease > SRNVM
Inflammation eg uveitis in VKH dz, Posterior scleritis
Infections
Choroidal tumor
RPE disease > leak
Systemic vascular disease- hypertension &
eclampsia

EXUDATIVE RETINAL
DETACHMENT

PIGMENTARY RETINAL
DYSTROPHY

Slow degenerative disease, bilateral

Starts in childhood, leads to blindness in middle/
advanced age
Degeneration mainly of Rods, starts at equator with
anterior & posterior extension
Macula affected very late
Symptoms: Night blindness (most prominent)
Visual field concentric contraction- Tubular vision
Loss of vision central, 50-60 yrs of age,
Associated with Glaucoma, Myopia, Cataract &
Keratoconus

RETINITIS PIGMENTOSAFUNDUS

Small jet black pigment cells (Bone corpuscle like)

around equator, spreading anteriorly & posteriorly
Retinal veins- sheathing with pigment along the
course
Tesselated fundus d/t migration of pigment cells
upwards
Pigments often lie over vessels
Retinal vessels extremely narrow/ attenuated
Degeneration of Ganglion cellsOAPale waxy
yellow disc (Consecutive OA)

RETINITIS PIGMENTOSA

RETINITIS PIGMENTOSA

RETINITIS PIGMENTOSA

Progressive posterior cortical cataract Complete

opacification of the lens
ERG & EOG Subnormal/ Extinguished (Seen
earlier than appearance of signs & symptoms; D/D
Secondary RP where ERG EOG is subnormal after
advanced changes in retina)
Condition is genetically determined

Majority Recessive; Consanguity of parents

Occasionally Dominant hereditary (Through generations &
is less severe form of the disease)
Sex linked (Rare- visual prognosis is very poor)

RETINITIS PIGMENTOSA

Other associations

Laurence Moon Biedl Bartum Syndrome (Obesity

Hypogoandism, Mental retardation & Polydactyly)
Deafness (Ushers Syndrome)
Cardiac conduction defects
Abetalipoproteinemia

Treatment Unsatisfactory

RETINOBLASTOMA
Epidemiology:
Rare, occurring in 1:20 000 children
Life threatening
2/3 of cases within 3 years of life / mean 12/18
1/3 are bilateral
Inheritance:
Inheritable: Germline mutation - one inherited inactive
allele on Chr 13 q14
Non-inheritable: Somatic mutation - both alleles are
inactivated by a mutation

RETINOBLASTOMA
Clinical presentation:
Leucocoria
Squint
Uveitis
Iris nodules / Heterochromia
Asymptomatic
Orbital inflammation & Proptosis
Secondary Glaucoma
Nystagmus
Visual impairment

RETINOBLASTOMA
Clinical Stages :
1.
Quiescent stage
2.
Glaucomatous stage
3.
Stage of extra-ocular extension
4.
Stage of Metastasis
Growth patterns:
5.
Endophytic
6.
Exophytic
7.
Mixed / Diffusely infiltrating

Retinoblastoma, intraocular
stage (leukocoria).

Retinoblastoma, intraocular
stage

Retinoblastoma, glaucomatous
stage

RETINOBLASTOMA
Pathology:
Microscopy:
Small polygonal cells
Flexner Wintersteiner rosettes
Degenerative changes: Necrosis & calcification
Gross:
Exophytic: growing towards choroid
Endophytic: growing towards vitreous
Spread:
> ON > Brain
> Emissary vein > sclera > Orbit

Flexner-Wintersteiner rosettes
in retinoblastoma

RETINOBLASTOMA
Treatment:
Enucleation
Intravenous chemoreduction
Radiotherapy
External beam teletherapy / Brachytherapy
Photocoagulation
Cryotherapy
Chemotherapy if metastatic
Thermochemotherapy
Photodynamic therapy

Age-related macular
degeneration
Epidemiology:

Leading cause of irreversible visual loss in the

industrialized world
Unknown cause
Risk factors:
Family history, sex (females), race (Caucasians),
smoking, age > 50 years.
Classification:
Non-exudative ( dry ): 10%
Exudative ( wet ): 90% of legal blind patients.

Two types of macular degeneration exist, the "dry"

form and the "wet" form. The dry, or nonexudative,
form involves atrophic and hypertrophic changes in
the retinal pigment epithelium (RPE) underlying the
central retina (macula) as well as deposits (drusen)
on the RPE.
Patients with nonexudative ARMD can progress to
the wet, or exudative, form of ARMD, in which
abnormal
blood
vessels
called
choroidal
neovascular membranes (CNVMs) develop under
the retina, leak fluid and blood, and ultimately cause
a blinding disciform scar in and under the retina.

Early stage of AMD with large

confluent drusen

Dry AMD with geographic

atrophy

Exudative ARMD
Clinical features:
Present with subretinal fluid, pigment epithelial
detachments (PED), subretinal lipid, or flecks
of subretinal hemorrhage in the affected eye,
in addition to RPE changes and drusen.
Reduced VA:
Choroidal Neovascularisation.
Exudative maculopathy.

Wet AMD with choroidal

neovascularization

Disciform scar

TREATMENT
LASER PHOTOCOAGULATION OF THE CNVM
REPRESENTS THE BEST-STUDIED AND
STANDARD TREATMENT FOR THIS DISORDER.
IVFA : CNV or SRNVM is extrafoveal, juxtafoveal or
subfoveal
Extrafoveal: > or = 200um from FAZ/ Argon
Juxtafoveal: < 200um from FAZ / Krypton
Subfoveal: below FAZ / Controversial
Post laser recurrence rate of 50% in 2 years

Other modalities

Photodynamic therapy
Intravascular dyes cause vascular occlusion by
a photochemical reaction.
Transpupillary thermotherapy
Transpupillary thermotherapy (TTT) involves
slowly heating the subfoveal choroidal
neovascular complex with infrared (810 nm) diode
laser light to occlude the CNVM
Antiangiogenic agents AntiVEGF Intravitreal

Macular translocation

Thank You