SECTION 5 OCULAR VASCULAR ANOMALIES

INTERNAL CAROTID ARTERY DISEASE (ICAD)

Complicatio
Stroke
ns
Px with clots in retina has significant chance of these occurring in
the brain
Px with increased lipid profiles (cholesterol) = higher risk
Visual Sx
May occur due to visual field anomalies and other sx
Clinical
Common > cholesterol is essential for metabolism
significance
Early manifestations = ocular (serious implications)
Disease process
Atherosclero

sis

Atheroma

Thrombus

Embolus

Starts with this

Affects mainly older adults
Hx of lipidemia > younger px affected
Primary lesion in atherosclerosis
Small round area of intimal thickening over which plaques form
Plug or clot in BV forms due to platelet formation
Platelets may break off or atheromas may ulcerate and form
thrombus
Localized
Fragment from atheromatous plaque
Spreads distally until it cannot flow any further (vessels too narrow)
> occlusions
Sites of damage occur at bifurcation areas due to turbulence of
blood flow

Clinical manifestation
Transient ischaemic attack
Presentatio
n
S+S
Pathophys
Mx

Epidemiolog
y
Prognosis

Unilateral

Temporary loss in VA, dim vision or black out

Combination of thrombus, vasspasm and tissue hypotension
Embolus release
If symptomatic, evaluate
If DFE was carried out and other diseases have been ruled ou,
symptoms are due to TIA
Cerebral Vascular Accident
92% 50+

Mx
Complicatio
ns

In younger Px, comparatively poor due to higher likelihood of

collateral development
Older px > where vessels have continued to narrow slowly >
stimulated collateral development
Evaluation of carotid artery disease
Carotid bruits > quiet room + px holds breath
Permanent loss of function
Sustained occlusion of arterial tree in absence of adequate

collaterals

Obstruction of internal carotid artery

Complicatio
n
Location

Test

Pathophys

Major contributor of stroke

50% of symptomatic px have serious stroke if not treated
Most frequent site of stenosis = just distal to bifurcation of internal
carotid artery and external carotid artery
Bifurcation is accessible area to check
Likely to hear turbulence after 50% > Stenosis is not
hemodynamically significant until >50%
70-80% will hear
90% too sluggish and plugged to hear > check pulse
Collateral circulation with external carotid may provide some
compensation

Ocular symptoms
Amaurosis fugax
Def

Aetiology

Characterist
ics
Incidence
DDX

Dimmer/greyer
Transient monocular blindness
Retinal transient ischaemic attack
ICAD
Cardiac problem > Rheumatic fever or brittle valves which break and
become emboli
Caused by tx of strep throat in child
When person is older > fibrotic calcific response
Monocular = ICAD
Binocular = Systsemic/brain
1-10min duration
Total or partial field loss
Shade rolling up or down
30-40% of px with ICAD
A. Cardiovascular/ cerebrovascular
If vertebra-basilar (cerebrovascular) basilar artery supplies much of
visual cortex defects are bilateral
If due to postural hypotension bilateral
Cardiac emboli causing TMB are less likely than emboli with carotid
source bilateral
B. Hyperviscosity syndromes blood dyscrasia
Polycythemia = increased RBCs
Sickle cell anemia = cell sickles and doesnt function properly
SLE
Leukemia = Increased WBC
TX: donate blood
C. Neuro-Ocular
Migraine bilateral
Px usually younger condition lessens due to decreased elastic
vascularity

Presentatio
n
DDx

Def
Clinical

Vasoconstriction followed by vasodilation > vasoconstriction

lessens with age
Papilloedema (obscurations)
Anterior ischemic optic neuropathy/ temporal arteritis (giant cell
arteritis) may be monocular

D. Ocular
Impending central retinal artery or vein occlusion (due to plaques
breaking off)
Monocular (ICAD)
Ischaemic Orbital Pain
Pain in orbit
Worsened by sitting or standing up due to brief decrease in vascular
supply
Orbital ceelullitis
Angle closure glaucoma (monocular pain)
Retrobulbar neuritis
Difficult to see > happens behind eye
Later stages > retrogrey optic neuropathy
Neuralgia
Fibroneuralgia > cant see nerve damage
Pain arises from nerve
Aetiology: of nerves
Scleritis
Anterior uveitis (Monocular cause of pain localized in eye)
Decreased vision in bright light and poor retinal function
Especially if patient comes inside, retinal recovery is decreased
Photostress phenomenon/ positive after-images after bright light
exposure
Secondary to compromised choroidal circulation
Results poorer if fluid is in macula due to poor circulation

Ocular Signs of ICAD (Ocular Ischaemic Syndrome asymmetrical, to affected

side)
A. Anterior Segment
1. Asymmetric arcus
- If one eye has heavy deposition of arcus, tat artery is not stenosed
- Lesser deposition of arcus side has stenosed carotid artery (less lipid gets to
eye)
2. Dilation of conjunctival vessels
3. Neovascularization of iris
- Rubeosis iriddis
- Neovasc of angle
- More likely to occur in iris > not in retina > aetiology ICAD
B. Intraocular pressure
1. Decreased intraocular pressure
2. Decreased ocular pulse

3. In affected side due to overall poor circulation

- Pressures may be equal, but one eye may be glaucomatous and is
effectively hidden where IOP is now lowered (low tension glaucoma)

C. Posterior pole
1. Emboli
Significant
Types

Precursor to cerebrovascular accident (CVA)

Cholesterol (Hollenhorst Plaques)
Associated with carotid atheroma
Yellow, refractile
Often at bifurcations
Rarely occlude artery (flat and slippery)
Platelet-fibrin (Fisher Plugs)
Associated with carotid thrombus
Dull white
Mobile
Often at bifurcations
Calcific
Associated with cardiac valve disease
White
Sticky > causes occlusion (BRAO) > abrupt loss of vision
rather than transient loss
Usually in unbranched arterioles
Intracranial emboli vs retinal emboli > causes
homonymous field loss vs monocular vision loss
More related to rheumatic fever
Use echo cardiology to 4x

2. Hypotensive/ hypoperfusion/ slow flow retinopathy/ venous stasis retinopathy

Pathophys
Results from decreased blood flow to eye
Leads to abnormalities in capillary beds > shut down >
stasis
Clinical
Dot and blot haemorrhages in midperiphery
presentation
Narrowed arterioles
Irregular venous tortuosity
Macular oedema
Capillary non-perfusion on fluorescein angiography
Microaneurysm on fluorescein angiography
3.
4.
5.
6.
7.
8.
9.

Narrowed arterioles
Asymmetric retinopathy
Centrla retinal artery occlusion
Central retinal vein occlusion
Anterior ischemic optic neuropathy (Like a stroke to ONH)
Cotton wool spots
Neovascularisation of disc and elsewhere

Relevant Diagnostic procedures never do them simultaneously

1. Palpation of carotid pulse, comparison
2. Auscultation of carotids for bruits
3. Ophthalmodynamometry (ODM) compare relative ophthalmic artery pressure
between eyes
Diagnostic tests
Doppler
ultrasonograp
hy
Carotid
angiography

in a hospital setting
Noninvasive
Studies direction of blood flow through supraorbital artery
(anastomosis between external and internal carotid)
Invasive
Diagnostic standard

Management
A. Expand history + probe for symptoms
1. TMB
2. Weakness of one side of face or tongue
3. Disturbance of mental functions
4. Clumsiness
5. Dysphasia (Disorder of language- partial/complete loss of ability to
communicate resulting from brain injury)
or dysarthria (Disorder of speech usually related to motor function)
6. Weakness of limbs paresthesias
7. Headaches
B. Referral to:
1. Internist/cardiologist
2. Neurologist

RETINAL VEIN OCCLUSION

For vein occlusions and arterial occlusions, look fr aetiologies and systemic
associated conditions

Central Retinal Vein Occlusion

A. Related systemic conditions
1. Hypertension (> 50% affected males; 70% affected females)
2. Diabetes
3. Hyperlipidemia
4. Hyperviscosity syndromes (Polycythemia sickle cell anemia leukemia CT
disease DM SLE)
5. Arteriosclerotic disease
6. Estrogen treatment
7. Chronic obstructive pulmonary disease
8. Idiopathic (20%)
B. Ophthalmoscopic Presentation
Hemorrhagic ischemic central retinal vein
occlusion
1. Blood and thunder fundus dramatic
intraretinal and NFL haemorrhage in
posterior pole
2. Cotton Wool Spots
3. Dilated tortuous veins
4. Exuberant disc oedema
5. Gross macular and retinal oedema
6. Extensive capillary closure on
fluorescein angiography
C. Sequelae
Hemorrhagic ischemic central retinal vein
occlusion
1. 66% incidence of neovascularisation
of iris and angle
2. 33-60% of these develop neovascular
glaucoma (1-4months later)
3. VA = 20/100 CF

Non-Ischemic central retinal vein occlusion

(DDx Low Flow)
1. Intraretinal haemorrhages in posterior
pole (not adjacent to disc
2. Dilated tortuous veins
3. Mild disc oedema
4. Moderate macular and retinal oedema

Non-Ischemic central retinal vein occlusion

(DDx Low Flow)
1. Cystoid macular oedema
2. Preretinal membrane formation
3. 2-3% develop neovascular glaucoma
4. VA = 20/80 20/100 (rare instances
where vision returns to 20/20

D. Management
1. Refer to internist High incidence of underlying systemic disease
2. Monitor for iris neovascularisation
3. Pan-retinal photocoagulation is effective in preventing NVI in ICRVO

Branch Retinal Vein Occlusion

A. Related systemic conditions Same as CRVO
B. Review of disease process
1. Predilection for A/V crossings (Bonnets sign)
2. May occur at rim of deeply cupped disc
3. May occur secondary to vascular inflammation (phlebitis) or vascular stagnation
(diabetes)
C. Ophthalmoscopic presentation
1. Dilated tortuous veins
2. Intraretinal haemorrhages (from site of obstruction out to periphery in section
of retinal normally drained by affected vein)
3. Microaneurysms with subsequent intraretinal oeema
As oedema increases:
4. Cotton wool spots
5. NFL haemorrhages
6. Macular oedema > decreased acuity in BRVO
D. Sequelae
1. Collaterals natures bypass
2. Retinal neovascularisation
E. Management
1. Refer to internist (High incidence of underlying systemic disease)
2. Monitor for neovascularisation
3. Focal laser treatment versus pan-retinal laser (Focal walls off macula and
destroys capillary beds to decrease inflow of arterial blood)

CENTRAL RETINAL ARTERIAL OCCLUSION

Related systemic conditions
1. Internal carotid artery disease (Atherosclerosis ipsilateral, primary systemic
disease implicated in all retinal vaso-occlusive disease)
2. Hypertension
3. Diabetes
4. Hyperlipidemia
5. Hypercholesteremia
6. Hyperviscosity syndromes
7. Cardiovascular disease (more likely cause when TIAs are absent)
Review of disease process
Thrombus of
Most common site of atherosclerotic disease of central retinal
central artery
artery is at the point the artery penetrates the dural sheath
Embolus
Most common site is at point artery enters lamina
Inflammatory
Giant cell arteritis
arteritic disease
SLE (collagen vascular disease)
Polyarteritis nodosa (inflammatory disease of small and
medium sized arteriese
Angiosasm
Small also causes an occlusion
1. Raynauds disease
- Irregular constriction and dilation of blood vessels
- Syndrome is a clinical manifestation
- Abnormal/vascular vasoconstrictor
- Response to cold and stress
- Fingers and toes white/blue/red different sensations
2. Migraine
Clinical presentation
1. Painless, sudden unilateral vision loss (SUVAL)
2. Relative afferent pupillary defect (RAPD)
Ophthalmoscopic presentation
1st few hours
Very white
Narrowing of retinal arterioles
Several hours
Hazy, milky appearance of posterior pole
Due to swelling of ganglion cell layer
Cherry red spot no ganglion cell layer at macula
Boxcarring of veins
2-4 weeks
Fundus grounds resume relatively normal appearance due to recanalisation
Infarcted retina replaced by glial tissue
Arteries remain thin, may develop sheathing
Optic atrophy (retrograde)
Visual prognosis extremely poor (Unless there is cilioretinal
artery)
- Only have 1/2 hours to get any remediation

20% have NLP, rest have CF

Ocular Sequelae
A. Few, if any
B. Ocular ischemic syndrome (chronic) not caused by CRAO, but may possible occur
if CRAO is secondary to ICAD with consequent compromised blood supply to eye
1. Aqueous flare
2. Rubeosis iridis or neovascularisation of iris
3. Low flow retinopathy (se ICAD)
4. Narrowed arteries
5. Neovascularisation of disc or elsewhere
Management only if within few hours goal is arterial dilation to dislodge
embolus or get blood around thrombus
1. Rebreathing
2. Breathing 95% oxygen and 5% CO2
3. Ocular massage
4. Anterior chamber paracentesis
5. Surgical intervention (to dislodge embolus)
*Generally there is nothing you can do to restore vision Key concern is to uncover and
treat underlying systemic disease
BRANCH RETINAL ARTERY OCCLUSION
Presentation
Usually secondary to embolus
Embolitic material can be visualized in 75% of cases
Suspect heart disease in younger px and px with no antecedent
TIAs
Clinical
Cimilar to CRAO
appearance
limited to area supplied by occluded vessel
Visual acuity and visual field loss is dependent on site of occlusion
Management
Refer to internist/cardiologist
Suggested
1. Internal carotid artery evaluation
work up
2. Blood pressure
3. Fasting blood glucose
4. Complete blood count
5. Lipid profile
6. Collagen vascular disease
7. Erythrocyte sedimentation rate
8. Coagulation profile
9. Cardiac evaluation

HYPERTENSION AND ARTERIOLAR SCLEROSIS

Terminology
Arterioscler
osis
Atherosclero
sis

Thickening and loss of elasticity of arterial walls

Degenerative process characterized by proliferative fibrovascular
and endothelial thickening of small arteries and arterioles
Degenerative process characterized by formation of intimal (lipid)
deposits in large and medium sized arteries

Hypertension accelerates development of both phenomena

In absence of HTN, they will occur with age (universally found in 40s-50s)

Retinal changes in hypertensive disease

A. Hypertension
Arteriolar

attenuation
Focal

Restriction

Hypertensiv

retinopathy

(signs initially reversible) Big AV changes

Significant elevation of blood pressure that has persisted for
appreciable period
Occurs with severe and abrupt elevations in blood pressure
Diastolic > 110 indicates local vasospasm
Suggest recent and severe increase in BP
More likely to occur in young px who have not yet developed
arteriosclerosis

1.
2.
3.
4.
5.
1.

Cotton wool spots

Flame shaped haemorrhages (seen with chronic HTN)
Microaneurysms
Hard exudates
Disc oedema (hallmark of malignant HTN)
Fibroid necrosis of choroidal vasculature can cause focal non
perfusion
Fluorescein angiography to detect
Overlying RPE appear yellow and leak = Elschnig spots
Healing = hyperpigmentation
Linear streaks of hyperpigmentation = Siegrists streaks
Localized exudative bullous detachment

Choroidal
circulatory
abnormaliti
es

2.
3.
4.
5.
6.

B. Arteriolar sclerosis (permanent changes)

Generalized
Thickening of arteriole wall secondary to long term battering by
sclerosis
increased blood pressure
Deviations
Silver wire or copper wire reflex
Arteriolaven
AV nicking
ous crossing
Thickened arteriole impinges on lumen of vein
changes
Deviation humping tapering banking
Hypertensive vs arteriolar sclerotic changes
Hypertensive vessel changes viewed with fundoscopy
Represent arteriole damage accelerated by elevated arterial pressure

 Reflect longevity of hypertension

Scheies classification
Hypertension
H0
Normal AV ratio >2/3
HI

A/V 1/2

HII

A/V < 1/2 + Focal Constriction

HIII

Above +
Microaneurysms
Haemorrhages
Hard exudates
Cotton wool spots
Above + disc oedema

HIV

Arteriosclerosis
AS0 Normal arteriole light reflex
No AV crossing changes
ASI
Slight broadening of arteriolar light
reflex
Minimal AV crossing changes
ASII Increased broadening of arteriolar
light reflex
Tapering
ASIII Copper wire appearance
Tapering and banking

ASIV

Silver wire appearance

Severe AV crossing changes

Blood pressure referral criterion

Immediate
Diastolic >130 or Systolic > 200
Disc oedema
ASAP
Diastolic > 110
Any hypertensive retinopathy
Repeat
Diastolic > 90
Systolic > 140
Staging (from Medscape 2011)
A. Keith-Wagener-Barker classification (1939)
- Px grouped according to ophthalmoscopic findings
- First system to correlate retinal findings with hypertensive disease state
Group 1
Slight narrowing
Sclerosis
Tortuosity of retinal arterioles
Mild asymptomatic hypertension
Group 2
Deffinite narrowing
Focal constriction
Sclerosis
AV nicking
Blood pressure higher and sustained
Few if any symptoms referable to blood pressure
Group 3
Retinopathy (cotton wool patches arteriolosclerosis
haemorrhages)
Blood pressure higher and more sustained
Headaches, vertigo and nervousness
Mild impairment of cardiac, cerebral and renal function
Group 4
Neuroretinal oedema + papilloedema
Siegrist streaks + Elschnig spots

Blood pressure persistently elevated

Headaches, asthenia, loss of weight, dyspnea, and visual
disturbances
Impairment of cardiac, cerebral and renal function
B. Scheie classification (1953)
Staging
Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

Grade 0
Grade 1
Grade 2
Grade 3
Grade 4

Grade
Grade
Grade
Grade
Grade

0
1
2
3
4

Diagnosis of hypertension but no visible retinal abnormalities

Diffuse arteriolar arteriolar narrowing
No focal constriction
More pronounced arteriolar narrowing with focal constriction
Focal and diffuse narrowing
Retinal haemorrhage
Retinal oedema
Hard exudates
Optic disc oedema
Light reflex changes from arteriolosclerotic changes
Normal
Broadening of light reflex with minimal arteriolovenous compression
Light reflex changes
Crossing changes more prominent
Copper wire appearance
More prominent arteriolovenous compression
Silver wire appearance
Severe arteriolovenous crossing
Modified Scheie classification
No changes
Barely detectable arterial narrowing
Obvious arterial narrowing with focal irregularities
Above + retinal haemorrhages and/or exudates
Above + disc swelling

RETINOPATHHY IN OTHER BLOOD DISORDERS

Sickle Cell Retinopathy (due to sickle cell anaemia)
Presentation
Inherited
Four main types
Mild (one parent contributes normal Haemoglobin A) or severe
Ocular manifestations do not necessarily correlate with severity of
systemic disease
Types
SS
Sickle cell disease
0.4% incidence
Most severe systemic problems and not much retinopathy
Ocular complications mild and asymptomatic
SC
Sickle cell haemoglobin C disease
0.2% incidence
Most ocular complications
AS
SC trait carrier haemoglobin in sickle cell
Mildest form
Requires severe hypoxia to produce sickling
S
Thalassemia
thal
Most prevalent in mediterraneans (Yellow-brown nations)
Variety is beta thalassemia major (Cooleys anemia)
Mild anaemia but has severe ocular manifestations
Pathophysiol
1. Abnormal haemoglobin results in abnormal RBCs
ogy
2. RBCs clump > oxygen poorly released (Occurs in conditions of
hypoxia and acidosis)
3. Capillary beds become anoxic
4. Induces sickling > increased blood viscosity
5. Vascular occlusion in small blood vessels
6. Ischemia of retina and subsequent neovascularisation
7. Tractional retinal detachment or rubeosis iridis

Retinopathy

Phase 1: Non-proliferative usually asymptomatic

1. Venous tortuosity
2. Black sunbursts (Combination of melanin and hemosiderin)
3. Refractile deposits
- Glistening deposits in peripheral retina
- From collection of hemosiderin in small schisis (pockets)
- Resulting from reabsorption of intraretinal blood
4. Salmon-patch haemorrhages (Round or oval retinal haemorrhages
resulting from eripheral arteriolar occlusions)
5. Rarely angoid streaks
Symptoms: (4) CRAO Macular arteriole occlusion Retinal vein occlusion
choroidal vascular occlusion
Phase 2: Proliferative classified into 5 stages
1. Peripheral arteriolar occlusions
2. Peripheral arteriovenous anastomosis
3. Peripheral neovascularisation to retinal surface
- Anastamosis new vein occurring to bring blood
- Occurs peripherally to retina, where retina wouldve been
avascular and non-perfused
- Sea fan shaped neovascular tufts which adhere to vitreous
4. Vitreous haemorrhage
5. Vitreous traction and retinal detachment

Rubeosis iridis can also occur (iris neovasc)

Management (ocular): Photocoagulation/vitrectomy

Management (Systsemic):
Blood transfusions (Can cause iron overload, therefore done with
adjunctive Fe chelator)
Splenectomy an bone marrow transplant

Anaemias
Definition

Systemic
clinical
symptoms
Ocular
findings
(retinopathy)
Pathogenesis

Cause
Clinical
features

Ocular signs

Cause
Clinical Sx

Ocular signs

Reduction in total circulating RBC mass

With consequent lowering of oxygen carrying capacity of blood
Sufficient to provke compensatory increases in cardiac output and
erythropoietin production
(7) Fatigue irritability dyspnea palpitations dizziness
headache shortness of breath
Haemorrhages (Nerve fibre layer may have white centre)
CWS
Venous tortuosity
Blood loss
Deficient RBC production (Deficiency of Fe, B12, folate and other)
(or) Accelerated RBC destruction
Iron deficiency anaemia
Decreased Fe availability or increased Fe loss
Angular stromatitis (oral mucosa)
Atrophy of tongue
Glossitis
Dysphagia
Pica
Flattened or spoon shaped nails
Conjunctival and retinal flame shaped haemorrhages
Conjunctival pallor
Retinal venousdistension
Oedema
Exudates
Pernicious Anaemia
Due to deficiency of B12 (Synthesis, dietary deficiency or binding)
Anaemia and peripheral neuropathy or numbness
Depression
Poor memory and dementia
Conjunctival pallor
Retinal or choroidal haemorrhages
Optic neuropathy (with centrocaeccal scotoma)
Rarely ophthalmoplegia

Leukaemia
Def
Presentatio
n

Retinopath
y

1.
2.
3.
4.
5.
6.
7.

Group of neoplastic disorders characterized by abnormal proliferation

of WBC
Retinopathy more often seen from secondary changes, like those
seen in other blood dyscrasias
Less common primary acute leukaemic infiltrate also manifests in
ocular involvement
Flame shaped haemorrhages
Roth spots
CWS
Peripheral neovascularisation
More rarely: leopard spot fundus leukaemic pigment epitheliology
May find optic neuropathy
Pseudo-hypopion
- Pus in anterior chamber
- On fundus looks like haemorrhage but white
- ONH swelling in one eye (usually inflammation)

Hyperviscosity states
Characteriz
1. Venous dilation, segmentation and tortuosity
ed by
2. Superficial and deep retinal haemorrhages
3. CWS
4. Retinal vein occlusions
5. Disc oedema
Affects
Diverse group of disorders (with increased blood viscosity)
More commonly polycythaemia or abnormal proteins (Waldenstrom
macroglobulinaemia and myeloma)

Retinopathy of prematurity
Definition
Proliferative retinopathy affecting pre-term infants
Eye
16 Weeks: Retinal vasogenesis starts at optic disc
developme
36 weeks: complete in nasal retina (tissue has no BV until 4 months)
nt
At term or 1/12 after: Temporal retina complete
*Infants born at 31 weeks or less are at risk for ROP
Pathogenes
Temporal retina only develops later
is
With incubation full of O2, retina doesnt develop because there is
enough O2
When taken out of incubation, proliferation of vessels occur due to
need for O2
Give anti-VGF
Risk factors
Oxygen oxposure
Prematurity
Acidosis during early neonatal period
Divisions
1. Acute Severity defined by location, extent and stages (I-V)
2. Cicatricial
Stages of
Stage 1
Demarcation line
acute ROP
Thin grey line between vascularized and vascular immature
peripheral retina
Abnormal branching vessels may lead up to it
Stage 2
Demarcation line develops into elevated ridge
BVs nter ridge
Small elevated tufts seen on posterior side
Stage 3
Ridge develops pink colour with fibrovascular tufts which proj
grow into vitreous
Dilation and tortuosity of vessels posterior to equator
Retinal and vitreous haemorrhage common
Stage 4
Sub-total retinal detachment
Stage 5
Total retinal detachment
Tx
Ablation of immature retina with cryotherapy or vitreoretinal surgery

Retinal telangiectasia
Def
Rare retinal abnormality
S+S
Leakage
Deposition of lipid exudates
Dilated tortuous vessels (capillaries)
Aetiology
Presents in Coats Disease as primary condition
Lebers miliary aneurism (May be considered as Coats Disease
affecting older px with less severity)
Idiopathic juxtafoveolar retinal telangiectasia (capillary
malformation occurs at edge of foveal avascular zone)
May be secondary to other vascular diseases:
1. Retinal vein occlusion
2. Diabetic retinopathy
3. Eales disease
4. ROP
Coats Disease (Presents later)
Def
Most severe form of retinal telangiectasia
Presentation
Usually unilateral
More common in boys (first decade of life)
Associated
Strabismus
with
Leukocoria
Loss of vision
Ophthalmosc
Earlier
opy
1. Large areas of exudates
2. Tortuous and dilated vessels
Later
1. Massive exudation
2. Retinal detachment
3. Secondary cataracts
4. Rubeosis iridis
5. Uveitis
6. Secondary glaucoma
7. Phthisis bulbi
Tx
(If early) Photocoagulation or cryotherapy
Purtscher Retinopathy
Aetiology
Microvascular damage with occlusion and ischaemia
Association
Severe head trauma
s
Chest compression injury
Embolism
Systemic ddisease
Retinopath
Multiple superficial white retinal patches (like CWS), often with
y
superficial peripapillary haemorrhages
Often rresolves within few weeks leaves patchy retinal function
Prognosis guarded due to possible macular or ON damage

Tersons syndrome
Aetiology
Sudden Valsalva manoeuvre
Stranging
Subarachnoid haemorrhage
Clinical
Acute rise in venous pressure which may result in pre-retinal
presentatio
haemorrhages
n
If severe may produce intragel vitreous haemorrhage
Retinal Artery
Def
Location
Test
Epidemiolo
gy
Glaucoma
Def
Presentatio
n

DDx tests

New Tx

Macroaneurysms
Localized dilation of retinal arteriole
Usually occurs in first three orders of arterial tree
Hyperfluoresce
Elderly px with systemic HPT

Ability of eye to tolerate its own IOP

One of the most common diseases in the world
Increasing frequency due to increased longevity
Afflicts at least 2% of adults in the world
Most common glaucoma = primary open angle glaucoma
Goldmann tonometry (IOP)
Humphreys visual field test (32 threshold)
Look at changes in ONH (cupping)
- Indicates RFL damage
- Other disease can show pallor but if theres no cupping, then
its not glaucoma
Causes perfusion of vessels
Analogues

Risk factors
1 Age
2
3

Race
Family Hx

Myopia
primary
open angle
Systemic
disease

One of the highest risk factors

Elderly more than young
Blacks more than whites
Case Hx important
Maternal family Hx increases risk 7 fold
Paternal family Hx increases risk 3 fold
Myopic px at higher risk due to weakening of laminar
area in ONH due to stretching of globe

Px with following diseases have higher incidence of

glaucoma due to inability of ON to withstand high
normal intraocular pressure due to reduced blood
flow to region:
1. Diabetes
2. Vascular occlusive disease
3. Haemodynamic crisis

4. Low BP
5. High BP if it results in arteriosclerosis
6. Thyroid eye disease

IOP

ONH
appearance

C/D

NFL dropout

Elevated IOP = strong risk factor

Normal IOP is sometimes associated with glaucoma
Generally, higher the IOP, higher the risk
Symmetry = less likely to be glaucoma

> 1.0 Strong risk

Normal ratios:
- Black = 0.6
- White = 0.4
- Most others = 0.5 0.55
Sectorial
Cup breaks through existing rim
notching
tissue to extent that rim in focal area
is eaten away/ notched
Change tends to occur in vertical
direction
Almost absolute sign of glaucoma
Dric/dranc
Splinter haemorrhage (NFL)
e Haem
Small flame shaped haemorrhage at
disc margin
Probably due to crushing of small
capillaries against edge of ONH
Very strong risk factor for glaucoma
Most often occurs in lower pressure
(normotensive glaucoma)
Beanpottin
Severe undermining
g
ONH and underlying tissue towards
rim is eaten away
Occurs in advanced glaucoma
C/D > 0.9
Peripapillar
Very common change
y atrophy
Area of eroded away tissue
surrounding ONH
Frequently seen in other conditions
(histoplasmosis)
Probably reason in glaucoma =
compromised blood supply to ONH
due to elevated IOP or other
aetiologies of glaucoma
High risk esp. with bigger areas of
atrophy
Temporal
Occurs when temporal rim of ONH is
unfolding
approached by cup
Occurs fairly early in disease
Difficult to see easier with OCT
Appears as if rake was applied to retina, separating
nerve fibres such that there are rake defects (darker
area) and large areas of missing NFL

Frequently directly associated with ONH notch

Occurs congenitally with minimal dysfunction and no
progression to glaucoma

Visual field
defects

1
0

Large
diurnal IOP
variation

1
1

Medications

1
2

Elevation of
post-dilated
IOP

1. First change :
- Often superior depression 25-35 deg from
fixation
- May be paracentral scotomas within 15 deg of
fixation
- May be nasal step, particularly if there is
temporal unfolding
2. Later, superior defects and paracentral scotomas
may coalesce into arcuate defects
3. As more of ONH is eroded, temporal wedge defect
may develop
4. Nasal steps may join arcuate defects to form
altitudinal defects suggestive of relatively advanced
glaucoma (looks like comet)
5. Eventually, only remaining sight may be temporal
island of vision or no light perception
Greater than 4-6mm
Test in morning and late afternoon difference
Fairly strong risk factor
Primarily corticosteroids
Can increase IOP, typically in px who are steroid
responders
Measure pre and post dilation
Important risk factor
Elevation > 3mmHg in non-occludable angle
suggests strong risk of present or future glaucoma

Primary Open Angle Glaucoma (COAG or POAG)

Def
Glaucoma which occurs by itself in presence of no anatomical or
other visible obstructions to anterior chamber angle
Inability of given eye to tolerate its IOP
Critical IOP determines glaucoma different for each individual
Before diagnosing glaucoma, must complete work up for disease
COAG
IOP > 20mmHg
suspicion
C/D > 0.1 above normal
Splinter haemorrhage (Dric/Drance)
Notching
NFL dropout
Workup
Complete exam measure refraction, best corrected vision and
pupillary reflex
Gonioscopy TM in all quadrants > G = open angle
- Look for other gonioscopic anomalies associated with
secondary glaucomas
IOP before and after dilation
Complete dilated fundus exam + complete evaluation of ONH
Threshold visual fields (30-2)
Diagnosis
IOP > 30 no matter what
IOP > 20 + C/D > 0.8

IOP > 20 + Splinter haemorrhage or notch

IOP > 20 + C/D > 0.7 + vascular occlusive disease
IOP > 20 + C/D > 0.7 + diabetes mellitus
Normal IOP + C/D > 0.7 + visual field findings suggestive of G

Follow ups
for unsure
Dx

Diurnal curve initially

IOP + dilated ONH + retinal evaluation every 6 months
Threshold visual fields every 6 months (Automated VF = standard
in glaucoma assessment)
- Humphreys (frequency doubling perimetry) > threshold
strategy ideally 30-2
- In the interest of time, 24-2 is acceptable
- Can give plenty of info in short time and more px friendly