Retinopathies

Thursday, 24 July 2014 11:19 AM

Overview
Retinopathies are a group of disease characterised by damage to and decreased function of the retinal layer of
the eye.

Types of retinopathies
- Diabetic retinopathy
- Hypertensive retinopathy
- Retinal occlusions
- Retinopathy of prematurity
- Haemoglobinopathy related retinopathies
- Radiation retinopathies
- Other proliferative retinopathies

Hypertensive retinopathy
Overview
Hypertensive retinopathy represents the opthalmological findings due to systemic hypertension of any cause.
Hypertensive retinopathy not only includes damage to the retina, but also the optic nerve and choroid.

Can be divided into malignant vs chronic changes.

Pathophysiology
Microscopically, early changes from hypertension demonstrate sclerosis and thickening of the arteriolar walls
with luminal narrowing. These findings become more prominent with long -standing systemic hypertension.

In malignant hypertension, the arterioles are similarly thickened, but necrosis and fibrinoid deposition in the
vessel wall occur.

Chronic hypertensive retinopathy rarely results in significant vision loss. Treatment of the underlying systemic
condition can halt the progress of the retinal changes, but arteriolar narrowing and AV nicking are permanent.
Treatment of malignant hypertensive retinopathy, choroidopathy, and optic neuropathy consists of lowering
blood pressure in a controlled fashion to a level that minimizes end -organ damage. The actual level of blood
pressure is less important in gauging the urgency of the situation than is the ongoing end -organ damage.

Symptoms/Signs
- Chronic
○ Usually asymptomatic.
○ Signs include:
 Arteriolar findings: narrowing, tortuosity and increased arteriolar light reflex (
copper or silver colored arteriole light reflex)
 Hallmark is AV nicking: occurs where the arteriole crosses and compresses the vein as the
vessels share a common adventitial sheath. If blood flow is impeded from AV nicking, the vein A. Grade I hypertension and grade I arteriolar sclerosis showing
distal to the point of compression is dilated and tortuous. the earliest generalized narrowing with increase light reflex. B.
Grade I hypertension showing generalized narrowing and grade II
- Malignant
arteriolar sclerosis showing increase in light reflex plus
○ Symptoms: headache, scotoma, diplopia, decreased vision, photopsias
arteriovenous compression. C. Grade I hypertension showing
○ Signs generalized narrowing and grade III arteriolar sclerosis showing
 Chronic hypertensive findings (AV nicking) with: broad light reflex, arteriovenous compression plus copper wire
 Obstruction of retinal arterioles with resulting cotton-wool spots near the optic nerve, lipid arterioles. D. Grade I hypertension plus grade IV arteriolar
exudates in the macula, macular edema, retinal hemorrhages and nerve fiber layer hemorrhages sclerosis showing increase of light reflex, arteriovenous
in the peripapillary region. compression, copper and silver wire arterioles.
 Elschnig spots - Elschnig's spots are black spots surrounded by bright yellow or red halos seen
on the retina during fundoscopy in patients with advanced hypertensive retinopathy.

- Clinical correlation with BP is obviously required.

Mild to moderate hypertensive changes with

exaggerated light reflex


Elschnig spots

- Grades of hypertensive retinopathy

○ Grade 1: Increased light reflex, generalised narrowing
○ Grade 2: Grade 1 plus AV nipping/AV compression
○ Grade 3: Grade 2 plus haemorrhages (flame shapped), or with cotton wool spots, or hard exudates
(soft/hard exudates)
○ Grade 4: Grade 3 plus papilloedema

Investigations
- Fluorescein angiography - not crucial for diagnosis but may be used Bilateral malignant hypertensive retinopathy in a patient with blood pressure of 260/140. Disc
edema, exudate, retinal hemorrhages, venous dilation, narrowing of the arterioles, Elschnig spots
Management are present.
- See hypertension

Diabetic retinopathy
Pathophysiology
The final metabolic pathway that causes diabetic retinopathy is unknown. There are several theories that are not
mutually exclusive. However, diabetic retinopathy is generally considered one of the microvascular
complications of diabetes (due to uncontrolled hyperglycaemia). Refer to a general diabetic complications slide
for more details on the pathogenesis of complications of diabetes.

As with all diabetic complications, it begins with vascular/ECM damage and cellular damage. In diabetic
retinopathy:
- Vascular damage due to AGEs, etc, lead to basement membrane thickening and vascular wall dysfunction,
causing increased vascular permeability and decreased blood flow
- Increased oxidative stress leads to intramural pericyte death, an important histological feature of diabetic
retinopathy.
Diabetic retinopathy can be classed into three stages, and only some diabetics with retinopathy progress:
- Early non-proliferative DM
- Advanced non-proliferative DM
- Proliferative DM
Non-proliferative DR symptoms/signs
- Symptoms
○ Often asymptomatic unless they have macular oedema, in which case they’ll experience chronic,
decreased central vision.
 As the disease progresses, patient may have scotomas, or visual field defects due to retinal
infarction.
- Signs
○ Microaneurysms are the first ophthalmoscopically detectable change in diabetic retinopathy and are
considered the hallmark of non-proliferative diabetic retinopathy. They are seen as small red dots in
the middle retinal layers, typically in the macula.
○ Dot or blot haemorrhages which are the result of ruptured microaneurysms.
○ Soft and hard exudates
 Soft exudates or cotton wool spots result from ischemia, not exudation. Fluorescein
angiography shows lack of capillary perfusion in the area corresponding to a cotton -wool spot.
○ Diabetic macular oedema - DME represents the leading cause of legal blindness in diabetics. The
intercellular fluid comes from leaking microaneurysms or from diffuse capillary incompetence.
○ In more advanced:
 Soft exudates are more associated with advanced NPDR
 Venous beading and vascular loops - Venous beading (see Fig. 6-21-4) is an important sign
of sluggish retinal circulation. Venous loops are nearly always adjacent to large areas of
capillary nonperfusion.
Lecture notes
1. Diabetic macular edema (DME)
o The prevalence of diabetes is increasing because of high fructose particularly!
o Mostly Type 2 DM
o DME is the leading cause of vision loss
o Gradual, painless loss of vision in a patient with long-standing (often poorly controlled) diabetes.
(Though remember sometimes that patients are undiagnosed).
o Signs in the fundus:
 Microaneurysms (“dot” – the hallmark of diabetic retinopathy)
 Retinal haemorrhages – if they’re in the nerve fibre layer, they look like “splinter
haemorrhages”. If they’re deeper in the retina, they’re “blot haemorrhages”
 Soft exudate “cotton wool spot”. Hard exudate – lipid that has leaked out of retinal vessels
(appear as yellow stuff precipitating in the macula).
 If you have a slit lamp, you can see swelling of the macula.
 In the normal fundus, retinal vessels are water-tight. Endothelial damage occurs in
diabetes and there is leakage out into the tissues leading to fluid build up and
swelling. The swelling alone causes damage to vision.
o (The “osmotic” effect of sugar is more significant with regards to effects on the
lens)
o Treatments:
 Control of diabetes
 Laser – treat leaking aneurysms
 Anti-VEGF  monthly injections at personal expense. But it is very effective and gives back
big quality of life. Often need more intense treatment during the first year
o Screening – the recommendation for patients with T2DM is:
 First screen at diagnosis of diabetes (90% is T2DM and you don’t know how long they’ve had

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○ Diabetic macular oedema - DME represents the leading cause of legal blindness in diabetics. The
intercellular fluid comes from leaking microaneurysms or from diffuse capillary incompetence.
○ In more advanced:
 Soft exudates are more associated with advanced NPDR
 Venous beading and vascular loops - Venous beading (see Fig. 6-21-4) is an important sign
of sluggish retinal circulation. Venous loops are nearly always adjacent to large areas of
capillary nonperfusion.
 More haemorrhages - can get splinter, flame, or more dot/blots.
o Treatments:
 Control of diabetes
 Laser – treat leaking aneurysms
 Anti-VEGF  monthly injections at personal expense. But it is very effective and gives back
big quality of life. Often need more intense treatment during the first year
o Screening – the recommendation for patients with T2DM is:
 First screen at diagnosis of diabetes (90% is T2DM and you don’t know how long they’ve had
it)
 Then afterwards 2 yearly (in Australia). Once you have retinopathy, it’s more frequent.

A: Small dot hemorrhages, microaneurysms, hard (lipid) exudates, circinate retinopathy, an intraretinal
microvascular abnormality, and macular edema. B: Fluoroscein angiography of early NPDR.

Proliferative DR
- Symptoms
○ MAY be asymptomatic but more likely to have blurred vision or visual field defects.
○ More likely to present with symptoms of vitreous haemorrhage or retinal detachment
 Haemorrhage: sudden onset of floaters followed by diffuse vision loss; monocular; usually
painless; strong association with retinal neovascularisation; associated with diabetes and sickle
cell disease; may occur after trauma
□ May be small haemorrhages initially, so patient may have a hx of: it will leave just a few
specks of blood, or spots, floating in a person's visual field, though the spots often go
away after a few hours.
 Detachment: flashes and floaters; curtain or veil over vision; sudden onset of painless vision
loss; central vision may be involved; progression of field loss can be over hours to days
- Signs
○ Neovascularisation. If it is close to the disc, then it is called neovascularisation of the disc. If it is
further away, its called neovascularisation elsewhere. Unlike normal retinal vessels, proliferative
vessels leak fluoroscein into the vitreous.

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 vessels leak fluoroscein into the vitreous.

Investigations
- Fluoroscein angiography - is a technique for examining the circulation of the retina and choroid using a
fluorescent dye and a specialized camera.. Would see hyperfluorescence due to leakage in proliferative
DR. Would see hypofluorescence in areas of ischaemia due to vessel pathology.
- B-scan ultrasound: used for vitreous haemorrhage
- Optical coherence tomography (OCT): It is a photographic test performed in the office. Has become
extremely useful in quantifying the extent of diabetic macular edema.
- Diabetic screening (blood glucose, HbA1c)

Management
- See diabetes
- For proliferative disease:
○ Laser photocoagulation
○ Vitrectomy and laser - if there is long standing recurrent vitreous haemorrhage, may be an option
○ Anti-VEGF

Resources
Opthalmology https://www-clinicalkey-com-au.ezp.lib.unimelb.edu.au/#!/ContentPlayerCtrl/doPlayContent/3 -
s2.0-B9781455739844001585/{"scope":"all","query":"retinopathy"}

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