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Assessment and management of AMD

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hyper-reflective material underlying the RPE is clearly visible on the tomogram. Furthermore, the local elevations of the RPE caused by drusen are also apparent on an OCT image (Figure 3). When a patient is referred to the hospital with suspected wet AMD, fluorescein angiography is carried out as a standard procedure to confirm the presence and type of choroidal neovascularisation. angiography tool in is also an differentiating Fluorescein important between attributable as myopia and

REFERRAl REFINEMENT PART 4 COuRsE CODE: C-16276 O/D

Dr Alison Binns, Bsc (Hons), PhD, MCOptom The previous article in this series outlined the key features of age-related macular degeneration (AMD), a condition that is responsible for more than half of all registrations as sight-impaired or severely sight-impaired in the UK. A review
1

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of treatments showed that medical intervention is currently only available for those with the active, wet form of the disease. Although those with early AMD (also known as age-related maculopathy - ARM), dry AMD (geographic atrophy), and end stage wet AMD are not responsive to current medical treatments, they still present at optometric practices requiring management. This article provides an overview of the optometric assessment of patients with suspected ARM and AMD, and appropriate management of these patients.

neovascularisation conditions such

to AMD and that caused by other birdshot choroidopathy, which may require different treatment strategies.2 VA is often relatively unaffected in early AMD, but there is evidence to suggest deficits in other aspects of visual function when fundus changes are still mild. Reduced sensitivity to flicker3

Clinical Assessment of ARM and AMD

Diagnosis and monitoring of AMD in the clinic has historically been based on the assessment of visual acuity (VA), Amsler chart, and fundus examination of retinal signs. Fundus examination in recent years has expanded to include not only direct and indirect ophthalmoscopy, but also imaging techniques such as (stereo) fundus photography and optical coherence tomography (OCT). Intraretinal or sub-retinal fluid accumulation or sub-retinal pigment epithelium (RPE) neovascular membranes will cause a raised area of the retina, which may not be immediately appreciated without a three dimensional view of the fundus. Binocular indirect ophthalmoscopy (Volk or BIO headset) and stereo fundus photography provide a means of accurately identifying elevations of the retina. OCT, which provides a crosssectional view of the retinal layers, not only allows the clinician to identify

raised or thickened areas of the retina, but also allows some visualisation of the nature of the material that is causing the elevation. For example, Figure 1 shows a serous pigment epithelial detachment (PED), where the fluid under the RPE is seen as black due to its low relative optical reflectivity, whilst Figure 2 shows a fibrovascular membrane, where the

and elevated cone and rod thresholds4 have been reported in individuals with ARM before marked VA loss has occurred. There is substantial evidence that the rod and cone adaptation are also delayed in very early macular disease.5,6 The macular photostress test is one way that cone adaptation may be assessed quickly and easily in the

Figure 1 Fundus photograph (left) and OCT image (right) of serous PED. Black arrow indicates location of OCT scan. Note the bright band of OCT image corresponding to the RPE (marked with a green arrow) shows domeshaped elevation with accumulation of fluid beneath, seen as a dark region due to its low relative optical reflectivity. Images courtesy of Ashley Wood, Cardiff University

clinic. Margrain and Thomson7 found the technique to be most repeatable after exposure of the macula to an ophthalmoscope light for 30 seconds, followed by assessment of the time taken for VA to return to within one line of its pre-bleach level. Their data suggest that a healthy 60-year-old person should have a recovery time of less than about 60 seconds, and that any delay beyond this may be considered abnormal. There is also evidence that patients who are at higher risk of developing choroidal neovascularisation will have more marked delays on the macular photostress test.8 This may provide a useful adjunct to the Amsler chart, which is commonly used to look for central visual distortions in patients at risk of wet AMD (Figure 4).

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large soft and confluent drusen and/or focal hyperpigmentation, or wet AMD in the fellow eye) should be monitored particularly closely. Amsler charts may be given to patients to take home so that they can check for distortions in their vision on a daily basis. It is important that the patient understands how to carry out the test (for example, the importance of checking each eye separately), and it is also vital that they understand the need for prompt assessment in their The should vision any should changes apparent. consider become

to wear protective sunglasses when outdoors, especially on bright days. Other nominated modifiable risk factors such as elevated body mass index (BMI), lack of physical activity and excessive alcohol consumption are less consistently significant across studies.20 Nutritional supplements The efficacy of nutritional supplements in preventing or delaying the onset of late AMD has been a matter of some debate. The first large randomised controlled trial of the benefits of supplementation for people with early ARM was the Age-Related Eye Disease Study (AREDS).21 This study reported a 28% risk reduction in progression from intermediate to late AMD over five years in people taking a combination of zinc plus antioxidants (high dosage of vitamins C, E and beta carotene). There was evidence of a beneficial effect in those who had at least one large druse, multiple intermediate sized drusen, parafoveal geographic atrophy in one or both eyes, or unilateral advanced AMD (individuals with only small drusen did not benefit). There is, therefore, a strong case for recommending vitamin supplements conforming to the AREDS formulation for people who fall into this intermediate AMD category.

Optometric management of ARM and AMD

Age-related maculopathy On identifying drusen or pigmentary changes in the retina, a key role of the optometrist is to exclude the possibility of neovascular changes through thorough fundus examination and checking for marked central visual field distortions using the Amsler chart (bearing in mind that drusen may themselves cause small distortions). These patients should be referred for urgent ophthalmological assessment if they have noticed a sudden onset of blurring or distortion of the central vision. Not all patients with drusen will imminently develop severe visual loss (one study observed that nine out of 49 patients with bilateral drusen developed severe sight loss in at least one eye over a period of about five years).
9

optometrist

giving advice to patients with ARM about lifestyle changes which may reduce advanced and their risk
11

of

developing
10

AMD.

Epidemiological studies

longitudinal

have

consistently reported that smoking is the strongest modifiable risk factor for the development of late AMD, which gives a firm basis for recommending that patients with ARM stop smoking. Some population-based studies have indicated that increased light exposure, especially to short wavelength (blue) light, may also be a risk factor for AMD.
12-17

However, patients should be made aware of the risks of developing late AMD and the symptoms of wet AMD. Patients with risk factors for developing choroidal neovascularisation (eg,. lots of

Although this finding has

18,19

not been universal,

it may be wise

to advise individuals at risk of AMD and those with early fundus changes

20/05/11 CET

Figure 2 Fundus photograph (left) and OCT image (right) of choroidal neovascular membrane resulting in PED. Black arrow indicates location of OCT scan. Note disruption of RPE on OCT image, and reflective material beneath (red arrow). Images courtesy of Ashley Wood, Cardiff University

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regular fish and green, leafy vegetables in their diet is a reasonable precaution to take, but it should be noted that the protective effect cannot be fully determined until large, randomised controlled trials have been carried out.

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Wet AMD

Figure 3 Fundus photograph (left) and OCT image (right) of drusen. Black arrow indicates location of OCT scan. Note bright band of OCT image corresponding to RPE (marked with a green arrow) is raised by underlying drusen (red arrows). Images courtesy of Ashley Wood, Cardiff University
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Contraindications and Alzheimers for the AREDS Some advanced AMD.22 There was also an apparent decrease in the risk of early AMD with regular fish consumption. The carotenoids which lutein are the and main zeaxanthin, supplements include smoking, anaemia disease.20 supplements that are currently available commercially lack beta carotene and so are less hazardous to current smokers. However, of such there is less rigorous evidence available regarding the efficacy non-AREDS formulations. Evidence for the beneficial effects of other dietary factors is less robust. Omega-3 long chain polyunsaturated fatty acids are required to maintain healthy photoreceptor outer segments, and may be associated with preventing oxidative, inflammatory and age-related damage to the retina. Oily fish such as salmon and tuna are rich in omega-3 fatty acids, and other sources include nuts, seeds and olive oil. A recent systematic review of nine studies that had the evaluated benefits of fatty

Patients who require urgent referral to the hospital eye service (HES) by the optometrist are those who present with newly developed wet AMD. These patients are at risk of rapid development of visual loss, and are also the patients who the could potentially intervention, therapy Novartis benefit from medical (Lucentis; particularly ranibizumab Pharma AG,

anti-VEGF

Switzerland and Genentech, California). There are guidelines provided by the College of Optometrists for referral of AMD cases, but these were published in 2005, pre-dating the widespread use of therapies based on growth factor inhibitors.24,25 Other referral guidelines have been published since, for example by Novartis, with specific reference to anti-VEGF treatments.26 The key features of these documents are the same; newly developed wet AMD warrants urgent attention by an ophthalmologist. Delayed treatment for wet AMD has been strongly associated with a poorer visual outcome, so time is of the essence.27 Some health authorities now employ a direct referral scheme for these patients, for example hospitals in Wales use an AMD direct referral pad, adapted from the Thames Valley Macular Group Referral Pad, which allows urgent and direct referral straight to the local macular specialist. In a different

constituents of macular pigment, have also been the subject of much interest given and their short protective wavelength antioxidant absorption

characteristics. Large epidemiological studies have found a reduced incidence of intermediate and advanced AMD in those individuals with the highest dietary intake of these dietary factors, suggesting that they may indeed have a protective effect in individuals predisposed to AMD.23 Green, leafy vegetables such as kale and spinach are a particularly good source of these carotenoids. On the basis of the evidence, advising patients to include

omega-3

acid intake (and included a total of 88,974 people) found that a high dietary associated in the intake with of of omega-3 was a 38% reduction risk developing

Figure 4 Image showing the Amsler chart as it may be perceived by a patient with distortions due to wet AMD. Images courtesy of National Eye Institute, National Institute of Health

scheme, Royal runs an Eye

Manchester Hospital optometry-led

fast-access direct referral clinic, in which optometrists and GPs may refer patients directly to a referral refinement optometrist, who can then re-direct either to the retinal specialist clinic for treatment, or to alternative clinics/ discharge.24 It is of great importance that all optometrists familiarise themselves with the local protocols for normal referrals and fast track referrals. Signs and symptoms of wet AMD requiring features urgent are: treatment are summarised in Table 1. The key retinal intra-retinal/sub-retinal or sub-RPE haemorrhage, the presence of exudates (which suggests leakage from the vessels, indicating a need for urgent treatment), intra-retinal or sub-retinal fluid, PED, raised central retina, and/or visible neovascular membrane (often seen as a greenish or greyish lesion). Functional changes

include metamorphopsia, recent onset blurred central vision, VA loss and uniocular hyperopic shift. Clinical trials suggest that baseline VA does not influence the outcome of ranibizumab treatment within the range of 6/12 to 6/48, and NICE guidelines support medical intervention if presenting VA is within the range of 6/12 to 6/96. Advanced AMD When AMD has advanced to a stage where VA is markedly reduced (to less than 6/96), as a result of geographic atrophy or advanced wet AMD including fibrosis or disciform scarring, treatment is unlikely to result in a positive outcome.2 These patients should still be referred to the HES on a non-urgent basis for assessment of the fellow eye, and also to determine whether they may benefit from other services available. A low
2

vision assessment, visual impairment counselling and/or registration as sight impaired or severely sight impaired may be appropriate. If they have only early changes in the fellow eye, provision of an Amsler grid for self-assessment, and advice on lifestyle changes should also be given (eg, stopping smoking, visual can nutritional supplements). who are advice not on For patients with an AMD-related impairment also give amenable to treatment, the optometrist useful household modifications that may help Generally increased lighting levels, with directional lighting when reading, can be particularly beneficial. Advice on improving contrast can also be helpful, for example suggesting that the patient use a thick black felt tip pen when writing. Later articles in this

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Fundus Features Dry ARM Drusen (several small hard drusen are considered a normal ageing change) Focal Hyperpigmentation

Functional status Slight distortion on Amsler grid, corresponding to location of drusen Gradual reduction in VA

Optometric Action Monitor, advise on lifestyle changes (eg, stopping smoking and nutritional supplements) and provide Amsler grid for selfassessment.

Wet AMD (suitable for Treatment)

Haemorrhage (sub-RPE, sub-retinal, intraretinal) Exudates (requires urgent referral as it is a sign of leakage from new vessels) Visible retinal elevation Sub-retinal fluid or pigment epithelial detachment Sub-retinal neovascular membrane may be seen as greenish grey lesion

Presence of markedly distorted, REFER URGENTLY blurred, or absent lines on Amsler grid (via rapid access referral route if Recent onset marked reduction in VA available locally) (6/12 to 6/96) Hyperopic shift in Rx

Advanced AMD

Geographic atrophy Disciform scar Extensive exudates, haemorrhage, fibrosis, macular elevation

Central scotoma on Amsler chart VA reduced to below 6/96

Refer non-urgently to assess fellow eye, and consider LVA assessment and training, visual impairment counselling and registration.

Table 1 Summary of the clinical features of ARM, wet AMD and advanced AMD, and appropriate action for optometrists

20/05/11 CET

with performance of daily activities.

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Approved for: Optometrists

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series will look at the management of patients with low vision at greater depth.

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the retina). Patients with end stage AMD (geographic atrophy/disciform scarring) should be referred non-urgently to check the status of the fellow eye, and for low vision aid provision and training, or registration as sight impaired or severely sight impaired. As new treatments become available, referral guidelines are likely to be reviewed in the future. Five key points early to remember: should Patients with AMD

for areas,

ophthalmological pathways it is and

assessment. vary between for all

Referral

important

summary
Patients with active wet (neovascular) AMD require urgent medical treatment to prevent rapid visual loss occurring. It is

optometrists to know their local system. When AMD has advanced to endstage disciform scarring or geographic atrophy, refer non-urgently for low vision assessment and evaluation of the fellow eye. Advise on the importance of improving lighting and contrast.

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important that all optometrists are aware of the urgent referral pathway for these patients in their area. Those individuals with early AMD may benefit from guidance about modifiable risk factors, particularly with respect to smoking and the potential positive effect of dietary supplements. Even if the presence of wet AMD has been excluded, advice on monitoring for symptoms of choroidal neovascularisation and the provision of Amsler charts is advisable, especially for those patients with risk factors for development of wet AMD (such as choroidal neovascularisation in the fellow eye, or lots of large soft and confluent drusen or focal hyperpigmentation of

be thoroughly examined to exclude possibility of wet AMD and given an Amsler chart smoking, dietary to self-monitor. AREDS only), supplements Stopping formulation (to days taking patients be

About the author

Dr and Alison a Binns is at an the optometrist School of lecturer Optometry and Vision Sciences, Cardiff University. Her main research interests include the early detection and monitoring of age-related macular degeneration and electrophysiology of the visual system.

20/05/11 CET

non-smoking should

and the use of sunglasses on bright recommended. If a patient has any signs of wet AMD (Table 1), has noticed a sudden onset of blurring or distortion of the central vision, or shows marked distortion on the Amsler grid, refer urgently

References
See http://www.optometry.co.uk clinical/index. Click on the article title and then download "references".

Module questions

PlEAsE NOTE There is only one correct answer. All CET is now FREE. Enter online. Please complete online by midnight on June 17 2011 - You will be unable to submit exams after this date answers to the module will be published on www.optometry.co.uk. CET points for these exams will be uploaded to Vantage on June 27 2011.

Course code: C-16276 O/D

1) Which of the following is NOT an advantage of OCT when assessing AMD? a) It allows visualisation of changes to different retinal layers b) It helps differentiation between different types of PED c) It allows differential diagnosis of classic and occult choroidal neovascularisation d) It helps to identify areas of retinal thickening due to oedema 2) The macular photostress test provides a rapid assessment of: a) Speed of cone adaptation b) Speed of rod adaptation c) Cone absolute threshold d) Rod absolute threshold 3) If a patient presents with bilateral soft drusen in the macular area and VA of 6/6 in both eyes, what is the MOsT appropriate course of action? a) Refer for urgent ophthalmological assessment b) Advise on lifestyle changes and monitor c) Refer for non-urgent ophthalmological assessment d) Refer for low vision assessment 4) If a patient presents with recent loss of central vision in their right eye, accompanied by haemorrhage and oedema in the macular region, and the best VA is 6/36, what is the MOsT appropriate course of action? a) Refer for urgent ophthalmological assessment b) Advise on lifestyle changes and monitor c) Refer for non-urgent ophthalmological assessment d) Refer for low vision assessment 5) Which of the following statements about dietary supplements for AMD is TRuE? a) Low dose vitamin C and E taken daily reduces risk of progression from early to late AMD b) High dose vitamin C and E and beta carotene plus zinc taken daily reduces risk of developing early AMD within five years, in healthy individuals c) High dose vitamin C and E and beta carotene plus zinc taken daily reduces risk of progression from intermediate to late AMD within five years d) High dose vitamin C and E and beta carotene plus zinc taken daily reduces risk of further visual loss in those patients with bilateral wet AMD 6) Which of the following statements about the referral of patients with wet AMD is FAlsE? a) Urgent referral is only necessary if VA is poorer than 6/18 b) Urgent referral is not necessary if VA is poorer than 6/96 c) Sudden onset report of central scotoma should be referred urgently d) Sudden onset report of blurred central vision should be referred urgently even in the absence of choroidal neovascularisation