Eye diseases: what is maculopathy?

By Cristiano Antonino Last updated Jun 2, 2023

The term maculopathy identifies a whole series of eye diseases that can affect the macula: the macula is a part of the eye, located in the center of the retina, responsible for clear and detailed vision

It is an extremely delicate area which is therefore very vulnerable to pathological and degenerative phenomena.

Maculopathies are varied and affect vision to varying degrees and in different ways.

The macula

It constitutes the central area of the retina, located in the posterior part of the eyeball.

While the retina is responsible for peripheral vision, the macula is the region of the eye with the highest density of photoreceptors and is therefore responsible for:

Central vision: it is the sharpest and most detailed vision that allows you to focus your gaze in the center of the visual field. Thanks to the photoreceptors, the light stimuli intercepted by the eye are transformed into impulses which, through the optic nerve, reach the brain.
Color recognition: in the center of the macula is the fovea, a slight retinal depression where most of the cones capable of distinguishing colors are concentrated.
Point or distinct vision: compared to the retina, the macula is able to distinguish details and very small details more clearly; point vision allows for example to read, recognize a face, thread a needle to sew, etc.
It therefore appears evident that a pathology that affects this anatomical region can involve vision to a different extent and in different forms. The maculopathies can have a bilateral involvement even if sometimes not contemporary between the two eyes.

Types of maculopathy

The pathologies affecting the macula can be many and have different natures: first of all, it is possible to make a distinction between forms of hereditary maculopathy or forms of acquired maculopathy.

While in the first case the degeneration of the macula is due to genetic factors, in the second case there may be predisposing external factors.

Acquired maculopathies

The most frequent acquired maculopathy and the main cause of vision loss in Western countries is age-related macular degeneration.

Other maculopathies can derive mainly from: degenerative processes; inflammatory such as noninfectious posterior uveitis and serous chorioretinopathies; infectious such as uveitis and retinitis; trauma and vascular dysfunction which can lead to retinal ischemia, macular holes, retinal detachment, macular pucker or concussive edema (Berlin edema).

There are also forms of induced maculopathy, which can be the result of post-operative complications, such as macular edema after cataract surgery, or due to particular pharmaceutical therapies such as some antipsychotics.

As already mentioned, maculopathy due to degenerative factors can be senile or linked to pathological myopia.

Macular degeneration due to advanced age is one of the most common causes of acquired maculopathy and is also one of the main factors of blindness after the age of 55.

It is a chronic disease, characterized by a gradual decrease in vision which involves the gradual subversion of the macular anatomy with implications also at the choroidal level.

Age-related macular degeneration can evolve into two forms

Atrophic or dry macular degeneration characterized by a slow evolution, in the initial stages it involves an accumulation of protein and glycemic deposits called drusen, up to the formation of patches of atrophy in the macular area, with a consequent decrease in visual acuity.
Neovascular or wet macular degeneration: This is a more rapidly progressing degenerative disease that involves the formation of abnormal blood vessels in the choroid. The blood and fluids contained in these growths tend to collect under the macula causing exudation between the retinal layers, damaging them with consequent distortion of vision or even blindness.
Myopic maculopathy can instead affect patients with degenerative or pathological myopia, generally with a refractive defect greater than 6 diopters.

This disorder arises because the retina is unable to adapt to the elongation of the eyeball resulting from myopia, and therefore undergoes stretching or injury.

This form of maculopathy can also lead to bleeding with a sudden decrease in visual acuity and image distortion.

A peculiar form of maculopathy is macular pucker which involves the development of a thin translucent membrane on the inner surface of the retina, called epiretinal.

The latter can cause a shrinkage of the central area of the retina, the function of which can therefore be altered.

Instead, the macular hole is a defect of the macula due to a rupture of the retinal tissue and the foveal area.

It can be caused by various pathological conditions including the formation of epiretinal membranes, traumatic events, myopic degeneration, vascular occlusions and hypertensive retinopathy.

Hereditary maculopathy

In some cases, macula defects also occur in children and young patients; often these are hereditary pathologies defined as macular dystrophies.

The main forms of hereditary maculopathy are:

Stargardt disease, is the most common form of juvenile macular dystrophy and involves the decrease of central vision from an early age.
Best’s disease or vitelliform dystrophy, characterized by a large yellow lesion within the fovea.
Retinitis pigmentosa, a disorder of the retina which can lead to loss of peripheral vision and hemeralopathy, i.e. night blindness.

What are the main symptoms

Identifying a maculopathy is not easy at all, especially when it affects only one eye.

An important alarm bell is a noticeable or sudden decrease in vision; to this end it is advisable to undergo a regular eye examination.

The main sign of the possible onset of maculopathies is undoubtedly the loss of central visual function, other symptoms can be:

metamorphopsia or distorted or altered vision of images;
perception of dull or bright colors;
perception of dark spots in the visual field;
scotoma, i.e. the perception of blind, dark or colored spots that can be fixed or intermittent, around which vision is generally good;
poor contrast sensitivity;
reading difficulties;
decreased vision at night;

Diagnosis

Given the degenerative nature of maculopathies, early diagnosis of the disorder plays a role of fundamental importance, as neglecting the disease could lead to very serious consequences and in the worst cases even blindness.

The symptoms of maculopathy can be confirmed by various targeted diagnostic tests:

Amsler test, is one of the simplest and most immediate methods to detect dysfunctions of the macula. During the examination, the patient will have to alternately observe first with one eye and then with the other, a grid of lines with a central point; with normal vision all the lines around the central area will be straight, while if the patient perceives them altered it is possible to suspect a disease of the macula.
Measurement of visual acuity, useful for determining the extent of central low vision;
Analysis of the fundus, thanks to an ophthalmoscope it is possible to observe the retina and all the other structures of the eyeball;
Optical coherence tomography (OCT): to study in detail the retinal layers at the macular level and identify any pathological changes;
fluorescein angiography or retinal fluorescence angiography, in which it is possible to examine the flow of blood within the blood vessels of the retina and choroid;

Treatment and cure of maculopathies

The treatment of pathologies involving the macula is not unique: in fact, there are different therapies and treatments that can be used based on the type of maculopathy, the personal course or the severity of the symptoms.

For some pathologies, in particular atrophic degenerative maculopathy, there are no specific treatments capable of preventing vision loss or significantly slowing down the degenerative process, even if a supplementary diet of some substances including antioxidants has demonstrated its effectiveness in the early age-related macular degeneration.

For the treatment of wet age-related macular degeneration, injections of drugs against angiogenesis are of considerable importance: these drugs can be injected into the eyeball to slow the loss of vision, keep eye lesions under control and avoid formation of new abnormal blood vessels under the retina.

The results obtained with this therapy are very encouraging and currently allow the control or slowing down of the degenerative course of the pathology.

Other forms of maculopathies such as macular pucker lend themselves instead to a therapeutic approach of a surgical type aimed at the removal of the epiretinal membrane which determines the stretching of the macula as previously mentioned.

In some forms, on the other hand, laser treatments are indicated which can be applied to the macula or to other retinal areas to have a resulting improvement effect on the macula.

The choice of treatment is at the discretion of the ophthalmologist and depends on the type of maculopathy, not all of which are suitable for the various pathologies that may involve the macula.

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