Fuchs’ Dystrophy

Fuchs’ dystrophy (pronounced “fyooks DI-struh-fee”) is a disease that usually affects the corneas of both of your eyes. Your cornea is the clear, tough, protective tissue that covers your iris and pupil. Other names for this corneal dystrophy are Fuchs’ corneal dystrophy and Fuchs’ endothelial dystrophy.

In Fuchs’ dystrophy, your corneal endothelium (the innermost layer of your cornea) gradually stops working as cells die. When it’s healthy, your 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 from excess fluid, which can cause blurred vision or a haze over your eye. Small blisters may appear on the surface of your cornea, causing pain and irritation.

What are the stages of Fuchs’ dystrophy?

The two main stages of Fuchs’ corneal dystrophy are:

• Early stage. In this stage, you have blurred vision in the morning that gradually clears up during the day.
• Late stage. As the disease worsens, swelling becomes more consistent, and your vision may be blurry at all times.

Symptoms of Fuchs’ dystrophy

In the early stage, you might not have any symptoms. 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.

Later, Fuchs’ endothelial dystrophy symptoms may include:

• Poor night vision, like seeing a glare and halos around lights
• Difficulty seeing on rainy days
• Problems seeing objects against a background (poor contrast sensitivity)
• Sensitivity to bright light
• Feeling like you have something like sand or dirt in your eyes
• Eye pain from blisters that form on your cornea and then break open
• Scarring that affects your central cornea first

Fuchs’ dystrophy causes

You can inherit Fuchs’ dystrophy from one of your biological parents during conception (autosomal dominant inheritance). But some cases happen randomly (sporadically) without a history of the condition in your biological line.

Some things can make Fuchs’ corneal dystrophy worse, including:

• Smoking
• Being exposed to ultraviolet (UV) light
• Having diabetes

Risk factors for Fuchs’ endothelial dystrophy

Certain factors can determine whether you have an increased risk for developing Fuchs’ dystrophy. These risk factors include:

• Age. The earliest signs of Fuchs’ dystrophy may be visible in people in their 30s and 40s. But the disease doesn’t normally affect vision until you turn 50 or older.
• Sex. Fuchs’ dystrophy is more common in females.
• History. It’s also more common in people who have a biological family history of Fuchs’ dystrophy.

How doctors diagnose Fuchs’ dystrophy

An eye care specialist will begin by taking a medical history, asking questions about your symptoms and doing a thorough eye exam.

Your provider 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

Tests to diagnose Fuchs’ corneal dystrophy

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

• Microscopy. These types of exams use microscopes to make diagnoses. Examples include a slit lamp and a confocal/specular microscope.
• 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.

Fuchs’ dystrophy treatment

A healthcare provider can treat Fuchs’ endothelial dystrophy, but they can’t cure it. Your provider may choose observation during the early stage. Other Fuchs’ dystrophy treatments may include:

• Ointments and eye drops. For mild symptoms, these medications reduce swelling of your cells by helping to dry up excess fluid. The improvement is usually temporary.
• Handheld hair dryer. Using a handheld dryer to blow warm air at your face can help the fluid dry up. This may be helpful for morning blur.
• Corneal transplant. When Fuchs’ starts to limit your vision, surgery is the definitive treatment. You may get a partial corneal transplant (endothelial keratoplasty) or a full corneal transplant.
• Descemet’s stripping only (DSO). A newer procedure involves stripping away the diseased cells in the center to allow your own, healthier cells from the sides to replace them.

What questions should I ask my healthcare provider about Fuchs’ endothelial 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:

• When should I contact you about symptoms?
• What treatment do you recommend?
• Are there clinical trials I can join?
• Are there low-vision aids that can help me?

How can I prevent Fuchs’ dystrophy?

There’s currently no way to prevent Fuchs’ corneal dystrophy. But you can help yourself by quitting smoking if you do smoke. If you have diabetes, work with your healthcare provider to manage your blood sugar levels.

What can I expect if I have Fuchs’ dystrophy?

Depending on how severe it is, Fuchs’ dystrophy may cause extreme pain, low vision or blindness if you don’t seek treatment.

With treatment, your expected outcome is much better. Your vision may improve to 20/20 within days of surgery (with glasses). That’s why it’s important to work with your provider to find the right treatment for you.