Fuchs’ Dystrophy

What is Fuchs’ dystrophy?

Fuchs’ dystrophy (pronounced “fyooksdi-struh-fee”) is a disease that usually affects the corneas of both eyes. The cornea is clear, tough, protective tissue that covers the white part of your eye. Other names for this corneal disease are Fuchs’ corneal dystrophy and Fuchs’ endothelial corneal dystrophy.

In Fuchs’ dystrophy, your corneal endothelium (the innermost layer of the cornea) gradually stops working as cells die. When it’s healthy, the endothelium acts like a pump to remove fluid from your cornea so it stays clear.

As your endothelium cells stop working, your cornea may swell, which can cause blurred vision or a haze over your eye. Small blisters on the surface of the cornea may appear, causing pain and irritation.

Some people with Fuchs’ dystrophy have blurred vision in the morning that gradually clears up during the day. As the disease worsens, swelling becomes more consistent, and vision may be blurry at all times.

Who does Fuchs’ dystrophy affect?

The earliest signs of Fuchs’ dystrophy may be visible in people in their 30s and 40s. However, the disease doesn't normally affect vision until you turn 50 or older.

There is a rare form that begins in some people during their first 10 years of life.

Fuchs’ dystrophy is more common in women than men. It’s also more common in people who have a family history of Fuchs’ dystrophy.

What are the signs and symptoms of Fuchs’ dystrophy?

There are different ways of rating how bad Fuchs’ dystrophy gets. This is called staging. In the early stages, you might not have any symptoms. In fact, it’s unusual to have symptoms before the age of 50.

If you do have an early symptom, it might be that your vision is blurred in the morning, but gets better as the day goes on. This is because your eyes are closed during the night and keep moisture in. When your eyes are open, they become drier. Many eye problems cause dizziness or headaches.

Later signs and symptoms include:

• Blurred vision that starts in the morning and continues throughout the day.
• Feeling like you have something like sand or dirt in your eyes.
• Sensitivity to bright light (photophobia).
• Eye pain from blisters that form on your cornea and then break open.
• Scarring that affects the central cornea first.
• Poor night vision, which may include glare and halos.
• Losing the ability to see precise differences in patterns or shading (contrast sensitivity).
• Losing corneal sensitivity, or the ability of the cornea to respond to touch.

Your eye care specialist will be able to see things in your eye exam that will point to a diagnosis of Fuchs’ dystrophy. These include:

• Thickening of certain membranes.
• Changes in the way your endothelium looks.
• Bumps called guttae on the inside of your cornea.

What causes Fuchs’ dystrophy?

You can inherit Fuchs’ dystrophy from one of your biological parents during conception (autosomal dominant), but some cases happen randomly (sporadically) without a history of this condition in your biological line. There are things that can make Fuchs’ dystrophy worse, including:

• Smoking.
• Being exposed to ultraviolet light.
• Having diabetes.

How is Fuchs’ dystrophy diagnosed?

Your healthcare provider will begin by taking a medical history, asking you questions about your symptoms and doing a thorough eye exam.

Your provider may use the following tests to diagnose Fuchs’ dystrophy:

• Microscopy: These types of exams use microscopes to make diagnoses. The slit lamp equipment used in your eye exam combines a microscope with a light that goes from a small slit to a complete circle. The confocal/specular microscope uses light projection to allow your provider enough of a view to even count your endothelial cells.
• Pachymetry: This painless test measures how thick your cornea is.
• Optical coherence tomography: This is a noninvasive imaging method that uses reflected light to create pictures of the back of your eye.

How is Fuchs’ dystrophy treated?

Your provider can treat Fuchs’ dystrophy, but they can’t cure it. Your provider may choose observation during the early stages. Later treatment may include:

• When Fuchs’ starts to limit vision, surgery is the definitive treatment. A partial-thickness internal corneal transplant (endothelial keratoplasty) is the standard surgery. Your surgeon replaces the damaged endothelial cells with healthy endothelial cells from a deceased donor.
• Stripping away some of the cells in your Descemet layer is a newer procedure that doesn’t require donor tissue. Your surgeon takes away the diseased cells in the center to allow your own, healthier cells from the sides to replace them. This procedure may not be effective for some patients.
• Using ointments and eye drops for people with mild symptoms. The medications reduce swelling of your cells by helping to dry up excess fluid. The improvement is usually temporary.
• Using a handheld hair dryer to blow warm air at your face to help the fluid dry up. This may be helpful for morning blur.
• Using glasses with photochromic lenses (lenses that darken in response to light) to help with light sensitivity.

How can I prevent Fuchs’ dystrophy?

There’s currently no way to prevent Fuchs’ dystrophy. However, you can help yourself by quitting smoking if you do smoke. If you have diabetes, it’s in your best interest to keep your blood sugar levels stable and to quit smoking.

What can I expect if I have Fuchs’ dystrophy?

Fuchs’ dystrophy can cause extreme pain, low vision and blindness without treatment.

What questions should I ask my healthcare provider about Fuchs’ dystrophy?

It’s important to develop a good relationship with your eye care specialist. Keep your scheduled appointments, and contact them if you have any new or worsening symptoms.

You may want to ask your provider these or other questions:

• When should I contact you about symptoms?
• What treatment do you recommend?
• Are there clinical trials I can join?
• Should I see a genetic counselor?
• Are there low vision aids that can help me?
• Am I eligible for disability?

Is there a relationship between cataracts and Fuchs’ dystrophy?

If you have Fuchs’ dystrophy, you may have cataracts. Your provider may recommend you have cataract surgery only if your Fuchs’ dystrophy is only mild or moderate. However, cataract surgery may make Fuchs’ dystrophy worse.

You may need a corneal transplant if you don’t recover quickly after cataract surgery or if your Fuchs’ dystrophy gets worse after the cataract procedure.

If you have an advanced case of Fuchs’ dystrophy, your provider may suggest you have cataract surgery and a corneal transplant at the same time.

Should I have laser vision correction surgery if I have Fuchs’ dystrophy?

Most people with Fuchs’ dystrophy likely will be advised not to choose to have laser vision correction surgery. Fuchs’ dystrophy can make laser correction less accurate.

Can I wear contact lenses if I have Fuchs’ dystrophy?

You may be able to wear contact lenses if you have Fuchs’ dystrophy, but you must be fitted correctly. Your eye care provider will recommend the best types of lenses for you, such as soft contact lenses or rigid gas permeable lenses.

Is Fuchs’ dystrophy related to glaucoma?

Researchers are continuing to examine the relationship between glaucoma and Fuchs’ dystrophy. You may be more likely to have glaucoma or optical hypertension if you have a severe case of Fuchs’ dystrophy. Signs and symptoms of Fuchs’ dystrophy may overlap those of glaucoma. Also, treatments for one disease may make the other worse. For these reasons, researchers need to continue studying this topic.

A note from Cleveland Clinic

Experiencing symptoms of any eye condition can be scary, and that’s true of Fuchs’ dystrophy. If you do have symptoms like pain or early morning blurred vision, see your healthcare provider as soon as possible. Remember that early detection and treatment often offer the best outcomes and that researchers are continuing to develop new ways to manage and treat Fuchs’ dystrophy and other conditions.