Hypertensive retinopathy

Definition: retinal vascular damage caused by

hypertension
Retinal changes mirrors the systemic
circulation and their severity correlates well
with the development of the systemic
complications of hypertension

Pathophysiology
acute blood pressure elevation causes reversible
vasoconstriction in retinal blood vessels and
hypertensive crisis may cause optic disc oedema
(vasospastic response)
Prolonged and severe hypertension leads to
exudative vascular changes due to endothelial
damage and necrosis
Chronic (years) elevation of blood pressure
results in atherosclerosis through medial
hyperplasia and fibrosis, manifests as arteriole
wall thickening and arteriovenous nicking
(atherosclerotic response)

Types
1) Chronic or atherosclerotic retinopathy due to
chronic elevation of blood pressure
2) Acute or vasospastic retinopathy due to
acute elevation of blood pressure

Grade: Acute (hypertension)

Grade

Features

Generalised arteriolar narrowing

More marked generalised narrowing with irregular points of focal

constriction

Generalised and focal narrowing plus cotton wool spots, retinal

haemorrhages, hard exudates

As grade 3 but with swelling of the optic disc

Grade: Chronic (atherosclerosis)

Grade

Features

Decreased venular visibility at arteriovenous crossing points, slight

broadening of the arteriolar light reflex

Deflection of the venule at arteriovenous crossing points

Copper wire arterioles, marked venular narrowing and deflection

at crossing points

Silver wire arterioles, extreme crossing changes

Clinical features: Symptoms

Diagnosis is through patients history and
fundoscopic examination
Symptoms usually do not develop until late in
the disease and include blurred vision or
visual field defects

Clinical features: Signs

Atherosclerotic neuropathy

Accelerated hypertension

Broadening of the arteriolar light reflex

Focal arteriolar narrowing

Venous nipping at the arteriovenous

crossing points

Flame shaped haemorrhages - located in

nerve fibre layer and result from capillary
damage

Arteriolar macroaneurysms

Cotton wool spots or ischaemic areas swollen axonal endings caused by focal
ischaemia

Silver wiring of arterioles - more severe

vascular wall hyperplasia and thickening

Exudates, often star shaped when at the

macula - well-defined yellow white
intraretinal lipid collections derived from
vascular leakage

Copper wiring - arteriosclerosis with

moderate vascular wall changes

Bilateral optic disc swelling

Arteriolar macroaneurysms

Normal versus grade I/II hypertensive

retinopathy. (Top) Normal ocular fundus.
Note that the ratio between the arteries and
the veins (arteriovenous [AV] ratio) is about
2:3.

(Middle) Grade I hypertensive retinopathy.

Note mild narrowing and sclerosis of retinal
arteries with an overall AV ratio of about 1:2.

(Bottom) Grade II hypertensive retinopathy.

There is AV nicking (arrow) and moderate to
severe narrowing and sclerosis of arterioles
(eg, within the ellipse the AV ratio is less than
1:2).

Black arrow: focal

arteriolar
narrowing
White arrow:
arteriovenous
nicking
Yellow arrow:
haemorrhage
Blue arrow:
microanuerysm
Red arrow: cotton
wool spot

Examples of grade III/IV

hypertensive retinopathy.
Note the features of grade III
retinopathy: exudates
(asterisks), cotton wool spots
(arrows), and nerve fiber
layer hemorrhages (ellipses).

Grade IV hypertensive
retinopathy is defined by the
presence of features of
grade III retinopathy plus
optic nerve head edema (eg,
the middle right panel).

The top figure shows optic disk

swelling, which appears as
blurring and elevation of disk
margins and a characteristic
star-shaped macular lesion
caused by leaking retinal
vessels.

The bottom image shows a

characteristic flame-shaped
hemorrhage and dilated veins

Retinal arteriolar
narrowing due to
thickening and
opacification of arteriolar
walls (copper wiring)
caused by hypertensive
arteriosclerosis. Image
also shows macular
edema

Management
1) BP control - avoid rapid reduction of systemic
blood pressure as it can precipitate vascular
occlusion, pharmacological management
(antihypertensive)
2) If vision loss occurs,
Intravitreal injections of corticosteroids
antivascular endothelial growth factor
(ranibizumab)

Retinopathy of prematurity (ROP)

A vascular response of the retina occuring
predominantly in low-birthweight premature
infants exposed to oxygen therapy in the early
weeks of life
This leads to traction detachment of the retina
and potentially to bilateral blindness
Patients will have high incidence of myopia
and strabismus

Pathophysiology
Initial failure of normal retinal vascularisation
Followed by a phase of aggressive new vessel
formation extending forward into the vitreous
and causing traction detachment

Risk factors

Gestation less than 32 weeks

Birthweight below 1.5 kg
Exposure to supplemental oxygen
Apnoea
Sepsis
Duration of ventilation
Blood transfusion
The presence of intraventricular haemorrhage
Retinal light exposure

Clinical features
Active ROP may progress
through 5 stages of increasing
severity
Spontaneous regression in
earlier stages occur without
intervention, but sequelae may
still cause visual loss
Signs:
1. New vessels
2. Development of retinal
haemorrhage
3. Increased tortuosity and dilation
of retinal vessels (poor
prognosis)
4. (severe) bleeding into vitreous
and retinal detachment, results
in blindness

The retina is divided into three zones, all centered on the optic nerve, to express
the location of the disease.
Zone I is defined as a diameter twice the distance between the fovea and the
center of the optic nerve. Clinically, this is approximately the area of the retina
seen through a 28D lens when the view is centered on the optic nerve.
Zone II is a circle that extends from the nasal ora serrata toward the temporal ora
serrata.
Zone III is a crescent encompassing the temporal area of the retina to the
temporal ora serrata that is not included in zone II.
The extent of the disease is defined in clock hours. Three oclock indicates the
nasal aspect of the right eye and the temporal aspect of the left eye.

In Stage 1 retinopathy
of prematurity, the
normal avascularized
retina appears to be
grayish and opaque in
color. A definite, flat,
demarcation line
between the
vascularized and
avascularized
characterizes this stage.

Plus disease in ROP.

Note that the
dilation and
tortuosity is in at
least two quadrants
around the optic
nerve.

Screening
Recommended for babies born at or less than
31 weeks gestational age and low birthweight
< 1.5 kg
Dilated fundal examination is carried out
regularly from 6 or 7 weeks after birth until
nasal retina is fully vascularised
A more premature infants are screened more
intensively

Treatment
Treatment is indicated for threshold disease
(stage 3), by which the benefits of the
treatment outweigh the risks
Avascular area is ablated using either
cryotherapy or laser to induce regression of
abnormally growing new blood vessels

Sickle cell retinopathy

An ophthalmologic manifestations of sickle
cell disease, causing vascular occlusion in the
retina, conjunctiva, iris, and choroid
Impaction of deformed RBCs in retinal
vasculature, leading to occlusion and
ischaemia causes sickle cell retinopathy

Pathophysiology
Sickle cell disease is due to point mutation in
the haemoglobin molecule in the RBCs in a
rigid and abnormally shaped RBCs, often
appeared as sickle-shaped.
Sickle-shaped RBCs will behave abnormally,
making them less flexible and unable to pass
freely through small blood vessels
Hypoxia exacerbates this tendency

Types
The most common and least severe:
haemoglobin S combined with haemoglobin A
(sickle trait)
Severe form: sickle cell haemoglobin C
diseases (SC disease) and sickle cell
haemoglobin with thalassaemia (SThal)
Confined retinopathy: homozygous sickle cell
disease (SS)

Clinical features
Systemic manifestations include anaemia and
sickle crises
Eye manifestations are divided into 2 types:
proliferative and non-proliferative

Eye manifestation
Proliferative retinopathy

Non-proliferative retinopathy

New vessels resemble a fan (sea-fan

neovascularisation)

Black sunburst scar and salmonpatch retinal haemorrhages

Vitreous haemorrhage

Venous tortuosity

Tractional retinal detachment

Retinal artery or vein occlusion

Retinal tear

Salmon patch
and black
sunburst
retinal
haemorrhages

Multiple
dilated
vascular
segments
sea-fan
neovascularisation

Management
Screening for retinopathy at regular interval
for patients with sickle cell disease
Laser photocoagulation of neovascularisation
develops

Abnormal retinal blood vessels

Abnormalities in retinal vessels may be seen in
rare ocular disease associated with the
development of massive exudate
May also be an indication of systemic disorder
as in the retinal and optic disc angioma
associated with the familial von Hippel-Lindau
Syndrome
Patients and relatives require repeated MRI
screening