10/21/2015

AgerelatedMacularDegenerationOphthalmology|Fastbleep

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Revision Notes
/ Biology Notes
/ Ophthalmology

AgerelatedMacularDegeneration
Written by: Namratha Mathai from Glasgow University,

AGERELATEDMACULARDEGENERATION

Agerelated macular degeneration (ARMD) is a term used to describe a characteristic pattern of

macular change and central visual loss, thought to be associated with the natural ageing
process.
Commonest cause of irreversible visual loss in the developed world
Burden of disease with ARMD likely to increase with an ageing population

PATHOGENESIS

Normal Structure and Function

The relevant layers at the back of the eye are:
Photoreceptor layer: outermost part of the neuroretina, composed of rods (light
perception) and cones (colour perception)
Retinal pigment epithelium (RPE)
Bruch's membrane
Choroid: highly vascular layer providing nutrition to the outer layers of the retina,
including the photoreceptor layer

The RPE is a single layer of cells, with microvilli that project in and around the outersegments
of the photoreceptors i.e. it is in close association with the neuroretina, but is only loosely
attached. The RPE is essential for normal retinal function by carrying out the following tasks:
Phagocytosis of redundant external segments of photoreceptors.
Recycling of vitamin A and therefore helping regeneration of rhodopsin and cone opsin
Absorption of light scattered by the sclera to allow better image formation (RPE cells
contain melanin)
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Facilitating nutrient and waste transfer between the retina and choroid (RPE cells are
held together by tight junctions and therefore form part of the bloodretinal barrier)

Bruch's membrane refers to the joint basement membrane of the RPE and the choroid. This
contributes to the diffusion barrier between choroidal blood vessels and the retina.

Pathological changes

The progression from normal macula, to dry ARMD through to wet AMD is variable only 10%
of patients will develop wet changes in their lifetime. The risk factors involved in determining
progression are as yet uncertain, however the following have been suggested:
Increasing age
Family history
Smoking: single greatest modifiable risk factor in progression
Drusen type: soft drusen (larger >63um, illdefined borders) is more strongly associated
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with progression from ARM to ARMD than hard drusen (small, welldefined). Increasing
number and confluence of drusen also increases risk.
Oxidative stress: some evidence that vitamin/mineral supplementation slows
progression
Cardiovascular risk factors: recent studies have linked poorly controlled hypertension,
sedentary lifestyle, obesity, etc with increased risk of progression to wet ARMD.

CLINICALPRESENTATION
Classic patient: Elderly, caucasian, positive family history
Smoking history (link to progression)
Changes tend to be bilateral but asymmetrical

Symptoms:
1. Decreased vision: blurred central vision or central scotoma (more advanced). Usually
gradual onset, but may notice more rapid deterioration with wet ARMD (during episodes of
haemorrhage/macular oedema).
2. Metamorphopsia/visual distortion: patients may describe certain parts of their visual
field appearing bigger (macropsia) or smaller (micropsia)
Functionally, as ARMD affects central vision, patients complain of difficulty reading, watching
TV, differentiating colours and recognising faces.

Signs:
1. Reduced visual acuity
2. Reduced visual fields: central
scotoma + loss of colour
saturation/contrast perception
3. Amsler grid: this is a useful tool to
quantify and qualify the degree of visual
impairment caused. It refers to a grid of
vertical and horizontal black lines on a
white background, which appear
distorted/blurred/darkened in areas
corresponding to the patient's scotoma.
4. Ophthalmoscopy:
absent foveal reflex
drusen (discrete yellow lesions)
hypo/hyperpigmented areas (atrophic
change)
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wet changes: haemorrhages,

neovascularisation

INVESTIGATION
A good history and examination is usually sufficient to diagnose ARMD. However, the following
imaging techniques help (1) confirm diagnosis (2) identify early wet changes and (3) act as a
baseline to monitor disease progression and/or response to treatment.

MANAGEMENT
Aims:
(1) preventing progression of disease
(2) minimising functional impairment/improving visionrelated quality of life

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Dry ARMD:
Most active interventions for ARMD are only indicated in patients with wet changes. The
mainstay of treatment of dry ARMD is watchful waiting and symptom management (discussed
later).
Observation: 624 month followup with community optometrists to monitor
changes/progression to wet ARMD. Patients may be given a copy of the Amsler grid for
selfmonitoring and asked to report any new visual symptoms developing in between
visits.
Vitamin supplementation (lutein): may be recommended in patients with advanced
dry ARMD who are at high risk of progression to wet ARMD. The evidence base for
effectiveness is currently under debate. Consequently, these have to be purchased
individually by the patient, as they cannot currently be prescribed on the NHS.
Smoking cessation: lowers rate of progression in both dry and wet ARMD.

Wet ARMD
A number of new therapies have been developed to limit neovascular changes. It must be noted
that these do not affect areas of established scarring and fibrosis, so patients may still be left
with residual visual loss.

Visual rehabilitation / Symptom management

The measures described above are aimed at preventing progression of disease. However, a
number of patients will have some degree of irreversible visual loss, that may cause significant
functional impairment. Visual loss may be due to progressive geographic atrophy in dry ARMD,
or complications of wet ARMD including subretinal haemorrhage, retinal detachment and scar
formation. Needs are specific to each patient and must be identified and managed on an
individual basis. Some available options include:
Low vision aids, magnifiers
Training in eccentric focusing (using peripheral vision to carry out tasks like
reading/watching TV that normally involve central vision)
Driving: as peripheral vision is spared, most patients with ARMD can continue to drive.
However, legally they are required to undergo a DVLAorganised visual field assessment
to ensure that the central visual field loss is not severe enough to cause difficulty.
Blind registration: a few patients with advanced disease will require to be registered
blind, particularly those with longstanding progressive changes.

References
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