Bull’s Eye Maculopathy

What is bull’s eye maculopathy?

Bull’s eye maculopathy is a rare condition that causes degeneration (deterioration) of your macula. The macula is part of your retina, which is the layer of tissue at the back of your eye. The degeneration appears as discolored rings on your macula, like a target or bull’s eye pattern. Bull’s eye maculopathy can cause permanent vision loss.

You can inherit bull’s eye maculopathy (your biological parents passed it down to you). You can also develop it as a complication of other eye diseases or long-term use of certain medications. When people inherit bull’s eye maculopathy, healthcare providers might call it benign concentric annular macular dystrophy (BCAMD).

While any type of vision loss is scary, your healthcare provider can get to the root of the issue. They’ll provide you with treatments and support to help you cope.

What are the effects of bull’s eye maculopathy?

Bull’s eye maculopathy damages the cells that help you see. So, it can lead to varying degrees of central vision loss. Some people have trouble seeing detail or color. It might be hard to see at night. Central vision loss can make it hard to read, drive and see things in front of you. The disease can affect one or both eyes.

Most people with bull’s eye maculopathy don’t have an issue with their peripheral vision (what you see out of the outer corners of your eyes). But it’s usually progressive, meaning it can get worse over time.

Bull’s eye maculopathy risk factors

A risk factor is something that increases your risk of developing a condition. Risk factors for bull’s eye maculopathy include:

• A family history of bull’s eye maculopathy.
• Macular degeneration.
• Genetic conditions like Batten disease or Stargardt disease.
• Certain medications, like disease-modifying antirheumatic drugs, for autoimmune diseases (diseases that cause your immune system to attack healthy cells).

How common is bull’s eye maculopathy?

Bull’s eye maculopathy is rare. It affects fewer than 1,000 people in the U.S.

What causes bull’s eye maculopathy?

Bull’s eye maculopathy has several possible causes, including:

• Genetics: The inherited form of bull’s eye maculopathy, BCAMD, is an autosomal dominant condition. A child with a biological parent who has a mutated gene (abnormal changes to DNA) has a 50% chance of inheriting the condition.
• Other diseases: Other eye diseases, like cone-rod dystrophy or retinitis pigmentosa, can lead to bull’s eye maculopathy. Bull’s eye maculopathy may also develop in people with genetic diseases like Batten or Stargardt disease.
• Toxicity: Certain substances, like medications, can damage cells in your macula. For example, long-term use of chloroquine or hydroxychloroquine for rheumatoid arthritis or lupus can lead to bull’s eye maculopathy. People who need frequent blood transfusions can get hemochromatosis (iron overload), which can also damage your macula.

How does hydroxychloroquine cause bull’s eye maculopathy?

Experts aren’t sure exactly how hydroxychloroquine causes bull’s eye maculopathy. Research suggests that the drug binds to cells in your retina and may affect how they work.

What are the symptoms of bull’s eye maculopathy?

Symptoms of bull’s eye maculopathy may include:

• Blurry or cloudy vision.
• Dark spots in your field of vision.
• Difficulty seeing color or complete color blindness.
• Eye floaters or flashes.
• Low vision.

What does bull’s eye maculopathy look like?

You can’t see bull’s eye maculopathy with the naked eye, so you won’t notice it if you look at your eyes in a mirror. An eye care specialist uses a tool called an ophthalmoscope to see your macula and retina.

The bull’s eye pattern usually appears as a round, dark area surrounded by lighter rings. The round, dark area in the center represents the normal retina. The rings are abnormal. The rings might be pale shades of orange, pink or purple. The different colors are the result of macular damage or degeneration.

How is bull’s eye maculopathy diagnosed?

Your healthcare provider may do a range of eye exams to diagnose bull’s eye maculopathy, including:

• Visual acuity test: Your provider checks your vision by asking you to read different-sized letters on a standardized chart (Snellen chart) or video monitor. They examine your vision when you read with both eyes and with one eye covered.
• Visual field test: Visual field testing checks your central and peripheral vision. It determines if you have any blind spots. You may look at a grid of lines, or your provider may use lights or hand movements to see how well you can see in front of you and to the sides.
• Color vision test: You look at an image of colored dots with differently colored numbers in the center (color plate test). You may have color blindness if you can’t see the numbers. Your provider may also examine how well you see the brightness of lights and colors using a tool called an anomaloscope.
• Ophthalmoscopy: Your provider uses eye drops to dilate your pupils (make them bigger). Then, they use an ophthalmoscope to examine your cornea, lens, retina, macula, optic nerve and surrounding blood vessels. Another name for this test is fundoscopy.
• Optical coherence tomography (OCT): OCT is a noninvasive test that uses light waves to create highly detailed, 3D images of your retina’s layers, including your macula. This test shows macular damage and degeneration earlier and more clearly than other tests.
• Fundus autofluorescence imaging (FAF): FAF is a new imaging technique that uses blue light to examine pigmented cells in your retina. Certain cells absorb or reflect the light differently than surrounding cells, which can help your provider identify retinal damage or degeneration.

Is there a cure for bull’s eye maculopathy?

There isn’t a cure for bull’s eye maculopathy.

How is bull’s eye maculopathy treated?

There aren’t treatments to reverse vision loss from bull’s eye maculopathy. If the condition results from drug or iron toxicity, stopping the drug or managing iron levels may prevent disease progression.

Most treatments focus on helping people cope with vision loss and color blindness. Your provider may recommend:

• Assistive technology, like video magnifiers, projectors or screen readers.
• Glasses or contact lenses.
• Low vision rehabilitation or occupational therapy, which teaches you techniques for coping with vision loss and performing daily activities.
• Vision aids, like handheld magnifiers, telescopes or light-filtering lenses.

How can I prevent bull’s eye maculopathy?

There’s no way to prevent bull’s eye maculopathy.

What’s the outlook for someone with bull’s eye maculopathy?

Vision loss from bull’s eye maculopathy is usually permanent and may be progressive. You might be able to stop toxicity-related macular damage from progressing further by avoiding the toxin.

What questions should I ask my healthcare provider about bull’s eye maculopathy?

There are a few questions you may want to ask your healthcare provider if you have bull’s eye maculopathy:

• Am I taking any medications that could make the condition worse?
• Are there any nutritional supplements that could help my eyesight?
• How can I cope with vision loss?
• How do I know when it’s no longer safe to drive?
• Is my vision loss permanent?
• What are the chances my vision will get worse?

A note from Cleveland Clinic

Bull’s eye maculopathy is rare. If you’re noticing vision loss, it’s unlikely that this disease is the culprit. But you should never let problems with your eyesight go unchecked. Even though vision loss can be scary and stressful, there are ways to cope. Your healthcare provider can connect you to the vision aids, support services and treatments you need to manage the condition. If you’re having trouble seeing or noticing any changes in your vision, talk to your healthcare provider right away. Depending on the cause of vision loss, early treatment may be able to slow, stop or even reverse eyesight problems.