Maculopathy: Symptoms, Causes, Types and Treatments

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Maculopathy and its Symptoms

Maculopathy

Maculopathy or macular degeneration is a disease that affects the central part of the retina called macula. Maculopathy is characterized by the progressive loss of central vision, often bilateral, which severely limits visual function. Degenerative maculopathy never leads to complete blindness as lateral vision is usually preserved until the terminal stages of the disease. Maculopathy or macular degeneration does not cause pain. Indeed, initially the visual problem may not be noticed, because it is compensated for with the good eye.

Symptoms

The first symptoms are usually:

  • distortion of images (metamorphopsia), whereby objects appear deformed and/or shrunken.
  • central “non-vision” spot, if the central part of the macula (fovea) is affected, so when, for example, observing a face, you can see the ears but not the expressions, the eyes and the mouth; or you can see the outline of the clock but not the time indicated. This evolution can occur quickly or over months.

For this reason it is advisable to perform the Amsler Test often to monitor your vision. The appearance of these symptoms can be a warning sign and should lead to a visit to the ophthalmologist as soon as possible.

Causes and Types of Maculopathy

There are different types of maculopathy based on the cause that causes them. Each type can then present itself in a dry or wet form.

Here are the most frequent types:

  1. Senile maculopathy or age-related macular degeneration: the most frequent form usually appears after sixty years of age
  2. Myopic Maculopathy: typical of those who suffer from myopia greater than ten diopters
  3. Diabetic Maculopathy: usually appears about 5-10 years after diagnosis of diabetes
  4. Macular Pucker, Macular Hole and Cellophane Maculopathy: more frequent with advancing age but heals with surgery
  5. Maculopathy after Venous Thrombosis: often associated with systemic diseases
  6. Hereditary or Genetic Maculoptia: different from each other but usually all begin at a young age.

Senile Maculopathy or Macular Degeneration

Senile maculopathy or age-related macular degeneration is the most frequent type of maculopathy. There are two forms: dry maculopathy and wet maculopathy.

Dry Maculopathy

The dry form is the most frequent. In 90% of cases it is this type of maculopathy, in which the retina thins because the visual cells stop working and disappear; this form also called atrophic maculopathy is not susceptible to laser treatment. The transport of nutritional factors and the elimination of waste by the retinal pigment epithelium (RPE) are slowed, resulting in yellowish (drusen) or pigmented (dystrophy, focal pigment) intraretinal deposits forming. Usually there is minimal visual impairment and only rarely do areas of atrophy form, which are a kind of stretch marks of the retina, which also involve the central part (fovea).

Exudative or Wet Maculopathy

In the exudative form it is thought that a reduced supply of nutrients to the retina can stimulate substances called vascular growth factors (“vascular endothelial growth factors” or VEGF). These give the signal for the production of abnormal vessels, which exude serum or bleed, then stimulating the formation of a scar. The proliferation of new vessels under the retina is called choroidal neovascularization or CNV. In 10% of cases the new vessels form in the retina and then deepen towards the choroid; in this case they are called retinal angiomatous proliferation or RAP. A variant (3-4% of cases) called polypoidal vasculopathy, tends to produce subretinal bleeding with pigment epithelial elevation (PED) and is often bilateral, but has a good prognosis. A certain percentage of dry maculopathy becomes exudative over time, so it is useful to monitor its evolution. Furthermore, even treatments that manage to effectively close these vessels cannot prevent possible recurrences. In recent years, many innovations have appeared in the diagnosis and treatment of maculopathy, today there is an early diagnosis thanks to the OCT exam, there are treatments to prevent the disease, as well as a genetic test capable of identifying those who are at risk. The therapy today relies on the use of modern drugs and continuously evolving maculopathy laser therapy. Dr. Badalà performs these treatments for the treatment of maculopathy in Milan and Catania.

Myopic Maculopathy

Myopic Maculopathy is typical of high myopes (above 10 Diopters of Myopia) and becomes more frequent with advancing age. It is a consequence of the fact that in high myopes the eye is longer than normal and the macula is stretched and forced to occupy a greater space. There is a dry or atrophic myopic maculopathy characterized by an atrophy of the retina and the underlying layer called choroid. Then there is a wet maculopathy characterized by the formation of new blood vessels as in senile maculopathy. Compared to the latter, wet myopic maculopathy responds better to therapies.

Diabetic Maculopathy

In people suffering from Diabetes Mellitus , when blood sugar control is not optimal, diabetic maculopathy can occur. It usually takes a few years between the onset of diabetes and the first manifestations of Diabetic Maculopathy. Diabetes in the eye causes changes in the wall of the retinal blood vessels, resulting in irreversible damage. The retinal vessels dilate, break, and thus create numerous small hemorrhages and exudates and then newly formed blood vessels that leak liquid causing edema of the macula. The signs are typical of Exudative Maculopathy. Symptoms can range from minimal visual disturbances to total impairment of visual function. In the initial phase, there may be a slight reduction or blurring of vision and the presence of spots in the visual field. Over time, the proliferative form can develop, which is particularly serious and can lead to detachment of the retina and consequent blindness . All diabetics should have an annual ophthalmoscopic examination, which allows for early diagnosis and treatment of lesions. The therapy uses multiple tools, the most important of which is careful control of blood sugar levels.

Macular Pucker, Macular Hole and Cellophane Maculopathy

Macular Pucker is a form of age-related maculopathy characterized by the growth on the inner side of the retina of a thin membrane (called epiretinal membrane) which can cause distorted image vision over time. The diagnosis is made through a careful examination of the retina and a test called OCT. Usually it is not necessary to intervene to treat the initial form of the disease called cellophane maculopathy, but if the disease progresses it is called macular pucker. In these cases the epiretinal membrane thickens and deforms the retina. If the deformation of the retina is very accentuated, the macula can even be torn and this is called a macular hole. The treatment of Macular Pucker and macular hole is the removal of the epiretinal membrane through vitrectomy surgery.

Maculopathy after Venous Thrombosis

Thromboses of the retinal vessels can concern the central retinal vein (CRVO) or a branch of the central vein (BRVO) and in both cases they can cause an Exudative Maculopathy similar to diabetic maculopathy. Numerous hemorrhages, exudates, edema of the macula and formation of abnormal blood vessels are observed . It is essential to discover the cause of the thrombosis to avoid the repetition of similar events in the other eye or in other areas of the body such as the heart, brain, etc. The necessary investigations must be carried out immediately to understand the cause of the thrombosis. Therapy is started to make the blood more fluid and correct the factors that caused the thrombosis (for example diabetes, hypertension, etc.). If appropriate therapies are used, vision recovery can usually be slow and progressive and in young subjects the recovery can even be total.

Hereditary or Genetic Maculopathies

There are different types, the most frequent are: Stargardt’s maculopathy, Best’s maculopathy and the maculopathy associated with Retinitis Pigmentosa. They have in common the onset at a younger age than other maculopathies. Being linked to specific genetic alterations, they often occur in multiple members of the same family. There is great hope for their cure with new gene therapies.

Diagnosis

The diagnosis of maculopathy is based on a thorough eye examination with examination of the fundus of the eye. To ascertain the cause and type of maculopathy, some instrumental tests are useful and are usually carried out at the same time as the visit to specialized centers for maculopathy. These tests are:

  1. Optical Coherence Tomography (OCT)
  2. Fluorescein angiography
  3. Indocyanine green angiography.

Today, by far the most important test is the OCT, also in the new version called AngioOCT, which allows for an in-depth evaluation of the blood circulation in the retina. In the past this could only be done with invasive tests such as Fluorangiography and Indocyanine Green Angiography in which a dye is injected into a vein in the arm. The Amsler Test is very useful for the diagnosis of maculopathy.

Amsler Test, Test to Detect Maculopathy

This is a very simple test that we can easily do at home at any time. The aim of the test is to identify Metamorphopsia as early as possible, which is the most typical and early symptom of degenerative maculopathy or macular degeneration. By metamorphopsia we mean the deformation, waviness, distortion of everything that is straight (lines, door jambs, steps). It is usually felt better while reading: the patient perceives the lines of the newspaper as distorted, wavy or broken. Unfortunately, this symptom is not always noticed early because the patient often does not notice the disorder until the healthy eye is covered. The Amsler test is very useful when you suspect a maculopathy or want to monitor the evolution of a degenerative disease over time. Download the Amsler Test. Print it and following the instructions in the file, run it every week. Who should perform the Amsler Test? Generally speaking, all people who are at risk of developing macular degeneration and therefore:

  • people over the age of 50
  • high myopes
  • people with one eye already affected by the disease
  • people with risk factors such as the presence of drusen (in soft particulars) present on the retina. Drusen are yellow accumulations that are deposited under the retina and are the expression of difficulty in disposing of waste substances.

The Dr. Badalà has worked and conducted important research on the treatment of maculopathy in the most important maculopathy centers in the world such as the Wills Eye Hospital in Philadelphia and the Jules Stein Eye Institute in Los Angeles. He is now involved in clinical trials on new treatments for the disease in Milan and Catania.

How to cure it

The best treatment for maculopathy depends on its form (Dry or Wet) and its cause. Many new features are available today.

Treatment of Exudative or Wet Maculopathy

When faced with an Exudative Maculopathy, action must be taken as soon as possible. Exudative or wet maculopathy is the result of new vessels growing into the macula. Fluid leaks from these newly formed and malformed vessels and accumulates inside the macula or under the macula causing macular edema and image distortion. It is important that the liquid typical of the exudative form dries up as soon as possible. The longer the macula remains edematous, the lower its visual ability will be. There are several therapies with proven effectiveness:

    1. Intravitreal injections
    2. Laser photocoagulation
    3. Micropulsed Yellow Laser
    4. Photodynamic Therapy
    5. EyeMax the New Therapy
    6. Vitrectomy.

Treatment of Dry Maculopathy

Dry senile maculopathy is characterized by progressive and slow atrophy of the macula. The cause is generally chronic oxidation and inflammation of the macula mediated by an inflammatory factor called complement. Today there are various therapies for the Dry form with the dual objective of slowing down the progression of the disease and improving vision. Useful to stop maculopathy are:

  1. Food supplements based on antioxidant vitamin supplements: vit C, vit E, vit B, selenium, zinc, lutein, zeaxanthin
  2. Diet rich in antioxidants, carotenoids and polyunsaturated fatty acids, such as colorful fruits and vegetables (oranges, kiwis, strawberries, blueberries, berries, carrots, pumpkin, spinach), fish and olive oil
  3. 2RT laser
  4. Drugs that inhibit the inflammatory cascade promoted by Complement (much of the research today focuses on this class of drugs, for example: Lampalizumab , Zimura and Elucizumab, all monoclonal antibodies against Complement factor 5)
  5. Low dose doxycycline – clinical trial underway with Oracea in the USA
  6. Gene Therapy
  7. Finally, sunglasses that cut wavelengths below 500 nm, i.e. part of the visible blue and all ultraviolet radiation (A and B).

Useful for improving the vision of those suffering from dry maculopathy are:

  1. Intraocular lenses for Maculopathy (EyeMax – Scharioth Macular Lens) which are implanted inside the eye either during or after cataract surgery .
  2. Optical aids such as video magnifiers, prismatic glasses which, by greatly enlarging the image and diverting the light rays into the area of the still healthy retina, allow you to improve your vision.
  3. Special glasses for maculopathy with colored filters that improve contrast sensitivity.
  4. Numerous applications for mobile devices such as cell phones and tablets can provide valuable help, including the Envision App available for both Apple and Android.

For further information we invite you to visit the page dedicated to the treatment of maculopathy where you can find the details of the techniques summarized here and also the therapies that are still experimental. You will also find on this site numerous testimonials from patients operated on at our eye clinics. Testimonies that give a glimpse of hope where all hope had been discouraged. We hope that this can comfort and encourage those who suffer from this disease.