What is macular disease

Macular disease is a condition that affects the macula, the central area of the retina responsible for sharp, detailed vision. It impairs reading, driving and recognising faces, while peripheral vision generally remains intact.

The macula is a small circular area, about 5 mm in diameter, at the centre of the retina. It is rich in cones (photoreceptors specialised in fine detail and colour vision) and is the only area of the eye capable of high-resolution vision. Its central part, the fovea, contains the highest density of cones in the entire visual system.

When the macula is damaged — by drusen, atrophy, abnormal new blood vessels or mechanical traction — central vision becomes blurred, distorted or absent, while peripheral vision remains intact. For this reason a person may be able to move around but struggle to recognise faces, read a newspaper or see the details of what they are looking at.

Macular disease is not a single illness but a group of conditions with different causes, courses and treatments. The most common form is age-related macular degeneration (AMD), the leading cause of legal blindness among people over 65 in developed countries.

What it affects: The macula — the central area of the retina, rich in cones for sharp vision.
Main cause: Age (AMD), high myopia, diabetes, genetic predisposition, smoking.
Characteristic: Progressive and largely irreversible: it spares peripheral vision but impairs central vision.
Who is at risk: People over 50 (AMD), people with high myopia, people with diabetes, relatives of people with AMD.

How it progresses

When the macula is damaged, symptoms are not uniform: they vary according to the form, the speed of progression and the areas affected. The most characteristic sign is the distortion of straight lines — the first warning that requires an urgent eye examination.

Progression of visual loss in AMD: from early distortions to a central scotoma — From early distortions to a central scotoma

The Amsler grid test at home

The Amsler grid — simple perpendicular lines with a central dot — allows people to detect early distortions, dark spots or areas where lines are missing. Each eye should be tested separately, once a week for people over 50 or with risk factors.

Macular pucker with wrinkles on the epiretinal membrane and distortion of the macula — Macular pucker: wrinkles on the epiretinal membrane cause visual distortion

The different types

There is not just one form of macular disease: it appears in **different forms** depending on its origin, the speed of progression and the treatment required. Telling them apart is essential to choose the right therapy and plan appropriate follow-up.

Comparison of a healthy macula with different forms of macular disease: dry AMD, wet AMD, myopic and diabetic — The different forms of macular disease compared

01
Dry AMD — age-related macular degeneration (atrophic)
80-90% of cases
It is the most common form of age-related macular degeneration. Its hallmark is the build-up, under the macula, of small yellowish deposits called drusen. Drusen are cellular debris that disrupt the supporting layer of the photoreceptors — the retinal pigment epithelium.
As the disease progresses, areas of atrophy appear, where the retinal cells degenerate and die. When the atrophy reaches the fovea, central vision is severely impaired. Progression is generally slow (years or decades), but the damage is irreversible.
Features
- Build-up of drusen under the macula
- Progression typically slow
- Symptoms initially mild: distortion, reduced ability to read
- Treatment: AREDS2 supplements in cases at risk, and control of risk factors; for advanced atrophy there are not yet any approved medicines in Europe
02
Wet AMD — age-related macular degeneration (exudative)
More aggressive form
Less common than the dry form (10-15% of cases) but much more aggressive. Under the macula, abnormal and fragile blood vessels grow (choroidal neovascularisation, CNV), which bleed and leak fluid, rapidly distorting the macula.
Progression can be very rapid: a person can lose a significant amount of central vision within a few weeks. For this reason prompt diagnosis and immediate treatment with intravitreal anti-VEGF injections are crucial.
Sub-retinal fluid build-up in wet AMD
Warning signs
- Sudden appearance of wavy lines (metamorphopsia)
- Dark or blurred spot at the centre of the visual field
- Rapid loss of visual acuity (days or weeks)
- Changes in the perception of central colours
03
Myopic macular disease
High myopia (over 6 dioptres)
It affects people with pathological myopia (over 6 dioptres and especially over 10). Excessive elongation of the eyeball causes progressive stretching of the retina and the choroid, with consequent atrophy of the macular structures.
It can also appear at a young age. It is often associated with myopic choroidal neovascularisation, which requires anti-VEGF treatment similar to that for wet AMD.
It is the main cause of decline in vision in people with high myopia. For those with myopia over 6 D, regular eye examinations are recommended, including when young.
04
Secondary and genetic forms
Specific causes
This group includes various forms due to identifiable causes: diabetic macular disease (diabetic macular oedema, see the retinopathy section), toxic forms caused by medicines (hydroxychloroquine, tamoxifen, certain anti-cancer drugs), and inherited dystrophies such as Stargardt disease and Best disease.
Eye injuries, vascular occlusions and chronic inflammation (for example chorioretinitis) can also cause secondary macular changes. Each has its own dedicated treatment: identifying the specific cause is the decisive step in planning treatment.
Forms to be aware of
- Diabetic macular disease — macular oedema, a complication of diabetic retinopathy
- Toxic forms — anyone taking hydroxychloroquine should be checked every year
- Stargardt disease — a juvenile form with a genetic basis
- Post-traumatic forms — after bruising or wounds to the eye

Why early diagnosis is crucial

Macular disease is progressive and largely irreversible. Once the macular cells have atrophied or scarred from neovascular damage, they cannot be restored to normal.

However, prompt treatment can slow or halt progression: anti-VEGF medicines in the wet forms can help preserve central vision if started before extensive damage has occurred; controlling risk factors (smoking, diet, blood pressure, diabetes) greatly reduces its progression.

After the age of 50 — or earlier if there are risk factors — eye checks with fundus examination and macular OCT should be carried out regularly, at the interval advised by your ophthalmologist. The Amsler grid test done at home once a week is an excellent way to monitor between visits: at the first sign of distorted lines, an urgent appointment can make all the difference.

What is macular disease