More Retinal Conditions

 
Retinal diseases are serious conditions. Luckily, we have treated hundreds of patients, giving our Retina Doctors and Specialist Group the capability to take on anything. Here is a list of the most common diseases and conditions, many of which share common symptoms and often-complex treatments. More details on each can be found by clicking on the name. 
 
 

ARTERIAL DISEASES

The carotid arteries are the two main arteries in the neck. They supply blood to the head, including the eyes and the brain. One carotid artery supplies the right side, while the other serves the left. Since the eye and the brain share the same source of blood supply, blockages or conditions of the carotid artery can affect either or both.

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TYPES OF RETINAL ARTERY OCCLUSIONS

A Central Retinal Artery Occlusion (CRAO) occurs when the main artery to the retina becomes totally blocked, causing sudden total vision loss. Later complications may include growth of abnormal vessels and bleeding inside the eye, or a severe, painful form of acute glaucoma (high eye pressure). 

 

In a Branch Retinal Artery Occlusion (BRAO), one of the smaller branches inside the eye is blocked and only a portion of the vision may be lost. This appears as a sudden “blackout” of a segment of the vision. Sometimes, a piece of cholesterol, known as a Hollenhorst plaque, can be seen at the edge of the blockage. Sometimes such a plaque is seen in a retinal artery with no sign of active blockage. Although this causes no symptoms, it serves as a warning sign of possible imminent problems.

Risk Of Stroke 

When the branches of the carotid artery going to the brain are blocked, a Cerebrovascular Accident (CVA or stroke) may result. Depending on the part of the brain involved and the size of the area affected, the effects of a stroke may be mild or devastating. Severe effects can include paralysis of one side of the body and loss of speech. If the part of the brain having to do with vision is involved, a stroke can lead to loss of side vision. If blood supply to the eye or brain is slowly cut off, more subtle symptoms may occur.

 

Chronic poor circulation to the brain can cause dementia-like symptoms and vague neurologic problems. Slow circulation to the eye, known as ocular ischemic syndrome, can cause vague episodes of vision loss, pain and sometimes small hemorrhages within the retina. The eyes can rapidly develop abnormal new vessel growth inside the eye and severe glaucoma after otherwise uneventful cataract surgery.

 

Routine, comprehensive medical eye examinations can help ensure healthy vision and can provide important information concerning carotid artery disease. If problems are detected, your eye doctor will work with your other medical doctors to coordinate your complete medical care.

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CENTRAL SEROUS CHORIORETINOPATHY

At the center of the retina is a tiny spot called the macula that provides most of the sharp central vision for reading and similar tasks. The retina also has several layers. On the outer surface are the cells that actually detect the light. Just outside of the retina is a rich network of blood vessels that normally leak fluid. Between these two main layers is a single layer of cells called the retinal pigment epithelium (RPE). One of its many functions is to keep fluid from leaking out of the vascular area into the area under the retinal surface.

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Central serous chorioretinopathy (CSC) develops when a leak forms in the RPE. This results in a tiny blister of fluid within the macula. Symptoms may include a change in glasses prescription, or images looking smaller, discolored, blurry, dim, or distorted. CSC is sometimes called central serous retinopathy (CSR).

Testing For CSC

CSC can occur at any age, although it is more commonly diagnosed in young adults (under 50). Fluid can be detected in the macula with a dilated eye examination. In many cases, a special photographic test, called a fluorescein angiogram, may be ordered to distinguish CSC from other conditions. This is a photographic test of the retina and does not involve X-rays.

 

After color pictures are taken, a yellow dye called fluorescein is injected into the arm vein and photographed as it passes through the retinal vessels. The photographic appearance of CSC is usually quite distinctive, helping to confirm the diagnosis. The picture can also be used as a “roadmap” to guide laser treatment when this is performed.

 

A test called optical coherence tomography (OCT) may be used to create a 3-D map of your retina showing areas of swelling. This is a painless laser scanning process. Microperimetry is a sophisticated vision test that may be used to create a map of how well the retina is functioning.

 

Aftereffects of CSC

The disease usually cures itself, and in most cases will disappear within a few months. Healing occurs as surrounding RPE cells slide over the leak to reseal it. The doctor may recommend that the patient monitor vision at home using a piece of graph paper called an Amsler Grid. Sometimes thinning of the RPE in the leaky area results in mild to moderate vision damage, but this is fairly unusual. Rarely, abnormal new vessels may grow, causing scar tissue and a threat of permanent vision loss. Laser is recommended in many of these cases.

 

Laser treatment may also be used if the condition isn’t resolved after several months. Treatment is usually delayed to try to let the condition heal itself, and because in rare cases laser can worsen the leak. Leakage can recur, although this is fairly unusual.

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CYSTOID MACULAR EDEMA

 

The healthy retina is a very thin tissue, which lines the back of the eye. The retina functions as the film in the back of the camera. The central portion of the retina is called the macula. This area provides all of the sharp central vision for activities such as reading and driving. This area is very delicate and can develop swelling, or “edema,” when it is irritated. This swelling makes the macula look bubbly or “cystic,” hence the name cystoid macular edema, or CME.

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Patients with cystoid macular edema frequently notice their reading vision is blurred. The peripheral or side vision remains unchanged. Frequently, people with cystoid edema notice distortion of objects and dimness. In addition, eyes may appear irritated and red, may water a great deal, and may even be tender to the touch.

Testing For Cystoid Macular Edema

If cystoid macular edema is suspected, a fluorescein angiogram may be performed. During the test, color photographs of the retina are taken, after which a yellow dye is injected into an arm vein. Photographs of the retina following dye injection indicate pooling of dye in the macula in a distinctive pattern, confirming the diagnosis of cystoid macular edema.

 

Other testing includes optical coherence tomography (OCT), which is a noninvasive laser scan that makes a 3-D map of the retina that reveals areas of retinal swelling (edema). Microperimetry is a sophisticated vision examination that may be performed to map the sensitivity of the retina. This test provides a measure of the damage to the retina and it is a way to measure if there is improvement after treatment.

 

Cystoid Macular Edema Treatment

Depending on the severity and the cause, treatment for cystoid macular edema may be done in a number of ways. If the swelling is the result of an observable problem, such as an infection, then treating the cause may help. Edema from diabetes is often treated with laser to seal leaky diabetic vessels. If a problem related to previous surgery is detected, such as a piece of leftover retained cataract, additional surgery may be performed to resolve this issue.

 

Usually, initial treatment for simple postoperative macular edema consists of using anti-inflammatory eye drops, sometimes along with an over-the-counter anti-inflammatory pill, such as Aleve. If this is not adequate, anti-inflammatory steroid injections may be used around the eye, or given in high doses intravenously. In rare cases, the vitreous jelly may be removed from the back of the eye in an attempt to decrease irritation or tugging on the back of the retina. If the edema is felt to be due to irritation from a lens implant, that implant may need to be surgically removed or exchanged for another style of implant.

 

Steroid medications can sometimes cause abnormal elevation of pressure in the eye, requiring additional medications to control this pressure. For this reason, use of these medications must be watched closely. Cystoid macular edema usually responds well to treatment, although response may require several months of continuing treatment.

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EPIRETINAL MEMBRANE

The area in front of the retina is normally filled with a jellylike material called vitreous. At birth, the vitreous is quite firm and has thin fibrous strands running through it and a clear fibrous skin on the back. As the vitreous ages, it becomes more liquid. Eventually, the fibrous skin peels partially or totally away from the retina. This process, called vitreous detachment, increases the number of floaters but does not usually cause damage to the retina.

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Occasionally the vitreous can cause irritation to the macula, which can stimulate the formation of a thin sheet of scar tissue on the macular surface. This looks like cellophane and is sometimes called “cellophane maculopathy.” The scar tissue may thicken or contract over a period of time. This can distort or “pucker” the macula, causing significant visual distortion. This condition is called an epiretinal membrane.

Finding Inflammation 

An imaging test called optical coherence tomography (OCT) is used to confirm the diagnosis. This is a noninvasive laser scan that reveals the wrinkled appearance of this condition.

 

Sometimes a special computerized picture test called a fluorescein angiography is performed to rule out other problems under the scar tissue, and to find inflammation sometimes associated with the epiretinal membrane. This involves injection of a yellow dye into a small arm vein, which is then photographed as it flows through the circulation in the back of the eye. 

 

Vitrectomy and You

If the epiretinal membrane is severe enough to prevent the patient from seeing and doing what they would like to, then the scar tissue can be surgically removed. This involves an operation called a vitrectomy. Tiny needle-sized openings are made in the white part of the eye. Tiny instruments, the size of hypodermic needles, are then used to gently peel the scar tissue away from the macular surface. The vitreous gel is removed to gain access to the macula. It is replaced with a clear fluid that the eye naturally replenishes continuously.

 

Vitrectomy surgery is usually done as an outpatient. The experience for the patient is similar to having cataract surgery. There may be some scratchy feeling on the day after surgery, but typically there is no severe pain. This surgery is usually done with a local anesthetic injection behind the eye. Complications, such as bleeding, infection, and retinal detachment, are very unusual.

 

Visual recovery may take weeks to months, but the eye will feel relatively comfortable within days of the surgery. You can usually resume normal activities within one or two weeks after surgery.

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WHAT IS OCULAR HISTOPLASMOSIS?

Histoplasmosis is a disease caused by a fungal organism, which is commonly found in the soil mostly in the Midwestern United States. There are two basic types of histoplasmosis: systemic and ocular. Systemic histoplasmosis produces an influenza-like illness with fever and weakness that usually lasts about two weeks. After recovery, the infection can leave small, usually harmless, scars throughout the body.

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The eye can be involved and the scars can be detected during an examination of the retina. Visual problems never develop during the initial, acute infection. Ocular histoplasmosis is sometimes referred to as “presumed” ocular histoplasmosis because there is not a proven direct link between the ocular lesions and the fungus.

 

Developing Ocular Histoplasmosis

Most people with active ocular histoplasmosis have blurred or distorted vision in one or both eyes. Distorted vision is a very important symptom and should be reported promptly to your doctor. This may appear as straight lines looking wavy (doorways, telephone poles, flagpoles, etc.). Many people with decreased vision in one eye may not realize they have a problem because the remaining good eye compensates so well. It is very important to check each eye separately. Ocular histoplasmosis may affect both eyes, although the second eye may not become involved for many years.

 

Ocular histoplasmosis is diagnosed with an eye examination involving dilation of the pupils. A special photograph test called fluorescein angiography is often helpful. In this test, dye is injected through an arm vein and pictures are taken of the eye as the dye circulates through the retina. These pictures are helpful in diagnosis and also serve as a road map for possible laser treatment.

 

Other testing includes optical coherence tomography (OCT), which is a noninvasive laser scan that makes a 3-D map of the retina that reveals areas of retinal swelling (edema). Microperimetry is a sophisticated vision examination that may be performed to map the sensitivity of the retina. This test provides a measure of the damage to the retina and is a way to measure if there is improvement after treatment.

 

Ocular Histoplasmosis Treatment

Intravitreal drug injection may be recommended in order to reduce swelling of the retina in the macula (macular edema). This is a relatively painless technique that is used to inject a drug into the vitreous cavity of the eye. The anti-VEGF drug Avastin has been shown to be an effective treatment for histoplasmosis. Anti-VEGF medications work by inhibiting the growth of new blood vessels. Injections are typically given monthly for a series of three months. More than one series of injections may be needed for effective treatment.

 

There are no eye drops or antibiotics known to be effective in ocular histoplasmosis. When abnormal vessels grow outside of the centermost part of the macula, laser can be used to stop the progression of abnormal blood vessels.

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LATTICE DEGENERATION

The retina is the light-sensitive layer of cells lining the posterior surface of the eye. Lattice degeneration is an area of retinal thinning. This is an area that is weak and can tear. When tears or holes occur within the lattice, retinal detachments may result. Retinal detachment is an urgent, vision-threatening matter for which you should seek medical assistance immediately.

 

Fortunately, most people with lattice degeneration do not develop a retinal detachment. Preventive treatment of lattice degeneration has not been shown to prevent retinal detachment, but lattice degeneration should be monitored. If you have a history of lattice degeneration, you should be aware of the symptoms of retinal tears and detachment. 

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RARE RETINAL DISEASES

Coats’ Disease

Coats’ disease is a chronic, progressive disorder that affects the retina, the light-sensitive nerve layer at the back of the eye. Coats’ disease is an abnormal growth spurt of the small blood vessels (capillaries) that nourish the retina. The fragile abnormal vessels break and leak the clear serum part of the blood into the retina, causing the retina to swell.

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 Coats’ disease usually affects children (especially boys) in the first ten years of life, but it can also affect young adults. The condition affects central vision, typically in only one eye. Severity can range from mild vision loss to total retinal detachment and blindness. No cause has yet been identified for Coats’ disease.

 

The leaking blood vessels can be treated with laser surgery or cryotherapy (freezing). If the retina is detached, a vitrectomy to replace the vitreous (the clear gel-like substance inside the eye) with a gas bubble may be necessary to restore vision.

 

Macular Dystrophy

 

Macular dystrophy is a hereditary condition in which the macula degenerates. The macula is the part of your retina responsible for acute central vision: the vision one uses to read, watch television, and recognize faces.

 

Symptoms of macular dystrophy can range from minimal vision loss and disturbance of color vision to profound loss of reading and night vision. The most common types of macular dystrophies, which tend to appear early in life, are Best’s disease, Stargardt’s macular dystrophy, and bull’s eye maculopathy.

 

Considerable research is directed toward finding the hereditary cause of many types of macular dystrophies. With further research it may be possible to develop medical treatments to prevent or slow the progression of macular dystrophy.

 

Low-vision devices can help affected individuals continue with many of the activities of daily life.

 

Stargardt’s Disease

 

Stargardt’s is an inherited disease that affects the retina, the layer of light-sensitive cells lining the back of the eye. It usually becomes apparent between the ages of 8 and 14. Boys and girls are equally susceptible and more than one child in a family may have it.

 

Stargardt’s disease begins with slightly blurry vision that gradually gets worse. By the late 20s, vision is typically about 20/200, the level labeled legally blind. Remaining vision is good enough for most people to live fairly normal lives, though they won’t drive, or read without using magnification devices.

 

A buildup of lipofuscin (fatty substance) in retinal cells is thought to cause Stargardt’s disease. The buildup typically happens in the central retina, or macula, where it resembles beaten bronze. Or, it can occur in the side retina where it causes small white flecks. This form is called fundus flavimaculatus. Angiography, a special photograph of the retina, may aid in the diagnosis. Although no specific medical or surgical treatment is available, glasses and magnification help affected people adapt to the disease.

 

Retinoschisis

 

Retinoschisis is a genetic eye disease that splits the retina, the light-sensitive layer of cells lining the back of the eye. It occurs in two forms, one affecting young children, the other older adults. Both forms usually affect both eyes, though one eye may be worse than the other.

 

Because the disease is inherited on the X chromosome, childhood retinoschisis occurs in boys more than girls. It is usually detected because of poor vision.

 

If the split retina involves the peripheral or side retina, peripheral vision is lost. One is also at risk for a retinal detachment. But more commonly, retinoschisis affects the macula, the area of the retina responsible for central vision. In this location, one loses central vision.

 

Peripheral retinoschisis, more common in adults, is usually caused by aging and does not affect vision, but it can cause a retinal detachment. If detected early, a retinal detachment can be treated with surgery or laser therapy.

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RETINITIS PIGMENTOSA

Retinitis pigmentosa (RP) describes a group of related diseases that tend to run in families and cause a slow but progressive loss of vision. RP affects the rods and cones of the retina, the light-sensitive layer in the eye, and results in a decline in vision in both eyes. RP usually affects both eyes equally with severity ranging from no visual problems in some families to blindness at birth in others. 

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All in the Family

 

RP gets its name from the fact that one of the symptoms is a clumping of the retinal pigment that can be seen during an eye exam. The earliest symptom of retinitis pigmentosa, usually noticed in childhood, is night blindness or difficulty with night vision. People with normal vision adjust to the dark quickly, but people with night blindness adjust very slowly or not at all.

 

A loss of side vision, or peripheral vision, is also common as RP progresses. Unfortunately, the combination of night blindness and the loss of peripheral vision can be severe and lead to legal blindness in many people. While there is a pattern of inheritance for RP, 40 percent of RP patients have no known previous family history. Learning more about RP in your family can help you and your ophthalmologist predict how RP will affect you.

 

Retinitis Pigmentosa Treatment and Research

 

Considerable research is being done to find the hereditary cause of RP. As hereditary defects are discovered it may be possible to develop treatments to prevent progression of the disease. While developments are on the horizon, particularly in the area of genetic research, there is currently no cure for retinitis pigmentosa.

 

Nutritional supplements may have an effect on RP. It has been reported that Vitamin A can slow the progression of RP. Large doses of Vitamin A are harmful to the body and supplements of Vitamin E alone may make RP worse. Vitamin E is not harmful if taken with Vitamin A or in the presence of a normal diet. Your ophthalmologist can advise you about the risks and benefits of Vitamin A and how much you can safely take.

 

Despite visual impairment, people with RP can maintain active and rewarding lives through the wide variety of rehabilitative services that are available today. Until there is a cure, periodic examinations by your ophthalmologist will keep you informed of legitimate scientific discoveries as they develop.

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VENOUS OCCLUSIONS

The retina is nourished by tiny blood vessels. The arteries carry the blood into the back of the eye, and veins drain the blood out and back to the heart. Sometimes, the main vein that drains blood from the eye can become blocked. This is called a central retinal vein occlusion (CRVO), and can result in mild to severe vision loss.

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If this process occurs in one of the small venous branches, it is called a branch retinal vein occlusion (BRVO). Similar loss of vision can occur, but it is usually more localized and less severe. In the picture at right, the central vision is decreased because the blockage is very near the macula, which provides reading vision. If the macula is not affected, there may be little or no vision loss at all.

 

Occurrences and Causes

When a vein occlusion occurs, the vision may be damaged in three main ways. Blood may back up and break out of blood vessels, resulting in hemorrhages that block the vision. Fluid in the bloodstream may leak out of vessels, resulting in vision loss from swelling, also called “edema.” Finally, the capillaries (the tiniest vessels) may be damaged, decreasing oxygen supply to the retina enough to result in vision loss. The macula is the central portion of the retina responsible for sharp central vision. If the macula is involved in the occlusion, vision loss may result.

 

Vein occlusions may have many causes. In elderly patients, they are more common in patients with other diseases of small blood vessels, such as diabetes and high blood pressure. Patients of any age may have blood disorders that result in abnormally thick blood, causing vein occlusions. If you have not had a recent medical exam, you should have one to check for these possible associated conditions. Glaucoma, a condition involving increased eye pressure, may cause vein occlusions by increasing resistance to blood flow within the eye. We will check for this as part of your evaluation.

 

Venous Occlusion Treatment

 

The treatment of retinal venous occlusion depends on the size and location of the blocked vessel. Some small vein occlusions are self-limited and resolve on their own without the need for treatment. The hemorrhage from vein occlusions clears with time and does not require treatment. The swelling that often results from vein occlusion may persist for long periods and can cause a permanent loss of vision. Here a few types of treatment:

 

1.     Laser: In some cases, laser treatment can be used to seal leaky vessels and assist in the reabsorption of this swelling. One complication of vein occlusion is abnormal new vessel formation in the eye that can result in vitreous hemorrhage with sudden vision loss or a severe, painful form of glaucoma. These complications can be treated with other laser techniques.

 

2.     Intravitreal drug injection: A relatively painless technique is used to inject a drug into the vitreous cavity of the eye. The Vision Research Center and Sabates Eye Centers played a significant role in the evaluation of these drugs during the FDA trials. Drugs that work to reduce retinal swelling include Lucentis, Avastin, and Ozurdex. Lucentis and Avastin are injected monthly for at least three months.

 

3.     Surgery: Doing surgery to physically relieve the site of blockage has successfully treated some severe vein occlusions.

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