Fuchs' dystrophy

Fuchs' dystrophy can cause eye pain and vision problems that worsen over time. Medications can help symptoms, but surgery may offer long-term relief.

In Fuchs' (fewks) dystrophy, fluid builds up in the clear layer (cornea) on the front of your eye, causing your cornea to swell and thicken. This can lead to glare, blurred or cloudy vision, and eye discomfort.

Fuchs' dystrophy usually affects both eyes and can cause your vision to gradually worsen over years. Typically, the disease starts in the 30s and 40s, but many people with Fuchs' dystrophy don't develop symptoms until they reach their 50s or 60s.

Some medications and self-care steps may help relieve your Fuchs' dystrophy signs and symptoms. But when the disorder is advanced and your vision is affecting your ability to function well, the best way to restore vision is with cornea transplant surgery.

As the disease progresses, Fuchs' dystrophy symptoms, which usually affect both eyes, might include:

• Blurred or cloudy vision, sometimes described as a general lack of clarity of vision.
• Fluctuation in vision, with worse symptoms in the morning after awakening and gradually improving during the day. As the disease progresses, blurred vision either can take longer to improve or doesn't improve.
• Glare, which can decrease your vision in dim and bright light.
• Seeing halos around lights.
• Pain or grittiness from tiny blisters on the surface of your cornea.

When to see a doctor

If you have some of these symptoms, and especially if they worsen over time, see an eye care provider, who might then refer you to a corneal specialist. If symptoms develop suddenly, call for an urgent appointment. Other eye conditions that cause the same symptoms as Fuchs' dystrophy also require prompt treatment.

Normally, the cells lining the inside of the cornea (endothelial cells) help maintain a healthy balance of fluid within the cornea and prevent the cornea from swelling. But with Fuchs' dystrophy, the endothelial cells gradually die or do not work well, resulting in fluid buildup (edema) within the cornea. This causes corneal thickening and blurred vision.

Fuchs' dystrophy is usually inherited. The genetic basis of the disease is complex — family members can be affected to varying degrees or not at all.

Factors that increase your risk of developing Fuchs' dystrophy include:

• Sex. Fuchs' dystrophy is more common in women than in men.
• Genetics. Having a family history of Fuchs' dystrophy increases your risk.
• Age. Although there's a rare early-onset type of Fuchs' dystrophy that begins in childhood, typically the disease starts in the 30s and 40s, with symptoms developing thereafter.

Besides testing your vision, your doctor might also have you undergo the following tests to determine whether you have Fuchs' dystrophy:

• Cornea examination and grading. Your doctor will make the diagnosis of Fuchs' dystrophy by examining your eye with an optical microscope (slit lamp) to look for irregular bumps (guttae) on the inside surface of the cornea. He or she will then assess your cornea for swelling and stage your condition.
• Corneal thickness. Your doctor might use a test called corneal pachymetry to measure the thickness of the cornea.
• Corneal tomography. Your doctor might obtain a special photograph of your cornea (tomography) to assess for early signs of swelling in your cornea.
• Corneal cell count. Sometimes your doctor might use a special instrument to record the number, shape and size of the cells that line the back of the cornea. However, this test is not required.

Some nonsurgical treatments and self-care strategies might help relieve the symptoms of Fuchs' dystrophy. If you have severe disease, your doctor might suggest surgery.

Medications and other therapies

• Eye medication. Saline (5% sodium chloride) eyedrops or ointments can help reduce the amount of fluid in your cornea.
• Soft contact lenses. These act as a covering to relieve pain.

Surgery

People who have surgery for advanced Fuchs' dystrophy can have much better vision and remain symptom-free for years afterward. Surgical options include:

• Transplanting the inner layer of the cornea. Known as Descemet membrane endothelial keratoplasty (DMEK), this procedure involves replacing the back layer of the cornea with healthy endothelial cells from a donor. The procedure is usually done with local anesthesia in an outpatient setting.
• Transplanting the cornea. If you have another eye condition or have had previous eye surgery, DMEK may not be an option. Your doctor may recommend a partial-thickness cornea transplant, a procedure known as Descemet-stripping endothelial keratoplasty (DSEK). Rarely, a full-thickness cornea transplant, known as penetrating keratoplasty (PK), may be recommended.

Potential future treatments

A variety of new treatments are being investigated that could change how Fuchs' dystrophy is managed in the future. After the genetic abnormality associated with most cases of Fuchs' dystrophy was discovered, there is a better understanding of how the disease might develop, and this offers the potential for nonsurgical therapies in the future. Various eyedrop treatments are being developed and may enter clinical trials in the future. Novel surgical treatments also are being studied to determine if they may be of benefit.

In addition to following your doctor's instructions for care, you can try these techniques to help reduce glare or soothe your eyes.

• Apply over-the-counter (nonprescription) salt solution (5% sodium chloride) eyedrops or ointment.
• Dry your eyes with a hair dryer. Hold it at arm's length and direct warm — not hot — air across your face, especially in the morning when swelling is worse. This helps remove excess fluid in the cornea, which decreases swelling.

If you suspect that you have Fuchs' dystrophy, make an appointment to see an eye care provider (optometrist or ophthalmologist). In some cases, you might be referred to an ophthalmologist who specializes in corneal disease.

Here's information to help you get ready for your appointment.

What you can do

Make a list of:

• Your symptoms, and when they began
• Key personal information, including family history of eye conditions
• All medications, vitamins or other supplements you take and dose
• Questions to ask your doctor

Take a family member or a friend along, if possible. You might not want to drive yourself home if your pupils have been dilated for the exam, and your companion can help you remember information you get during your appointment.

For Fuchs' dystrophy, questions to ask your doctor include:

• What else could cause my symptoms?
• How will my vision be affected?
• How rapidly will my condition progress?
• What tests do I need?
• What treatments are available, and what do you recommend?
• I have these other health conditions. How can I manage them together?
• Do I need to restrict my activities?
• Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, such as:

• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?
• Do your symptoms change throughout the day?
• Have you noticed changes in your vision?
• Does your vision seem worse in the morning and improve during the day?

Last Updated:

September 16th, 2021