Retinoschisis

What is retinoschisis?

Retinoschisis is a condition that happens when your retina divides into two or more layers. Schisis means a split or a cleft.

Retinoschisis affects the light-sensing layer of your retina and the layer of cells that transmits signals to your brain through the optic nerve. This division of the layers can affect how well you see. Splits can occur in the center of the retina but are more likely at the periphery (outer edges).

Types of retinoschisis

There are two types of this somewhat rare disorder: acquired or congenital.

Acquired retinoschisis (also called degenerative retinoschisis)

Degenerative retinoschisis usually occurs in people in their 50s, 60s and 70s. For that reason, another name for it is senile retinoschisis. However, it can sometimes happen to younger people as well.

Degenerative retinoschisis can be flat (typical) or bullous (reticular), which means blistered. (Bullae are blisters). These two types are difficult to tell apart, but the bullous type is more likely to develop into retinal holes and retinal detachment.

Myopic macular schisis has other names—myopic traction maculopathy or myopic foveoschisis. It refers to a retinal layer split located at the fovea. The macula is the central part of your retina. The fovea is the central point of the macula. It happens in people who have severe myopia (nearsightedness).

Congenital retinoschisis

A congenital condition is something you’re born with. The name for this form of the condition is juvenile X-linked retinoschisis. It usually affects both eyes.

Congenital retinoschisis mostly affects people assigned male at birth (AMAB). Their mothers carry the gene but often don’t develop the condition.

What is the difference between retinal detachment and retinoschisis?

Retinal detachment means that the retina has pulled away from the tissues that support it. Retinoschisis means that the retina itself has split. The difference may be difficult to see even for an ophthalmologist, but testing will help your provider be sure about the diagnosis.

It’s possible to have both retinal detachment and retinoschisis at the same time, especially if you have the congenital form.

How common is retinoschisis?

Researchers estimate that juvenile X-linked retinoschisis (congenital retinoschisis) happens in about 1 in 5,000 to 1 in 25,000 people.

Degenerative retinoschisis affects an estimated 4% of people over age 40, although another estimate puts the figure at 1% to 4% of people over age 50.

What are the signs and symptoms of retinoschisis?

You may have no symptoms of the disease. If you do, symptoms that may happen with juvenile X-linked retinoschisis include:

• Eyes that turn toward your nose (crossed eyes).
• Eyes that move uncontrollably from one side to the other (nystagmus).
• Loss of central (foveal) vision or side (peripheral) depending on where the split occurs.
• Having farsightedness.

If you’ve developed acquired retinoschisis, you might find that you can’t see clearly on either side (loss of peripheral vision). You may not have any symptoms at all.

If you have retinoschisis and it becomes severe, or you also have retinal detachment, you may notice:

• Floaters and flashers.
• Distorted images.
• Loss of central (foveal) vision or side (peripheral) depending on where the split occurs.

What causes retinoschisis?

A genetic mutation, or a change in your genes, causes congenital retinoschisis in many cases. The X-linked factor is related to the genes you get from your mother.

Scientists haven’t found the cause of acquired retinoschisis. The risk does increase with age, especially after age 40.

How is retinoschisis diagnosed?

This condition is most often diagnosed by an ophthalmologist, a doctor who specializes in diseases of the eyes. After your comprehensive eye exam, these are the most likely tests you will receive to make the diagnosis:

• Ophthalmoscopy (also called fundoscopy): In this test, the provider uses an instrument — an ophthalmoscope — to look at your eyes, especially your retinas.
• Optical coherence tomography (OCT): OCT is a non-invasive testing method that uses reflected light to take images of the back of your eye.
• B-scan ultrasonography: This type of ocular ultrasound (ultrasound of the eye) is non-invasive and uses sound waves that move back and forth to take pictures of the back of your eye.
• Electroretinography (ERG): An electroretinogram tells your provider how your eyes respond to flashing lights.
• Angiography: This type of test provides images of the blood vessels in your retina. The test uses dyes like fluorescein or indocyanine green.
• Genetic testing: Your provider may suggest these tests if they suspect you have juvenile X-linked retinoschisis.

How is retinoschisis treated?

You can’t cure retinoschisis, but you can manage it. You may need glasses to help with your vision or other low vision aids.

If you have acquired retinoschisis, you might not need treatment. Your provider may consider this condition to be benign, which means that the condition may not be serious enough to need treatment. You may need glasses for refractive errors like being nearsighted or farsighted.

If you have juvenile X-linked retinoschisis, you also may need glasses for refractive errors.

If you develop bleeding in your eyes from broken blood vessels, your provider may suggest cryoablation (cold therapy) or laser therapy to stop the bleeding.

If you develop a retinal detachment, you’ll need surgery to re-attach the retina.

Researchers continue to work on potential treatments for juvenile X-linked retinoschisis, including gene therapy, stem-cell therapy and a drug called dorzolamide.

How can I prevent retinoschisis?

You can’t prevent retinoschisis.

There’s no known cause of acquired retinoschisis. If juvenile X-linked retinoschisis runs in your family, you may want to ask your provider about speaking to a genetic counselor.

What can I expect if I have retinoschisis?

There is no cure for retinoschisis. It may result in considerable vision loss, even to the point of being legally blind. However, some people don’t need any type of treatment.

How do I take care of myself if I have retinoschisis?

One of the main things you can do if you have retinoschisis is to have regular eye exams.

You may find it helpful to use low vision aids and to become educated on mobility, orientation and using adaptive technology. This is true if you have retinoschisis and if you have a child with retinoschisis.

You should always let your healthcare provider know about any change in vision.

A note from Cleveland Clinic

Losing any amount of vision can be a scary thing. It’s important to know that you don’t have to manage everything on your own. Keep your regular appointments with your eye care professional. Ask your healthcare team for information on support groups and other resources. Take advantage of the services they offer. The range of services is very broad and can include education, fellowship and technological help.