Fuchs dystrophy

Overview

Fuchs dystrophy is a condition in which fluid builds up in the clear tissue at the front of the eye, called the cornea. This causes your cornea to swell and thicken, leading to glare, blurred or cloudy vision, and eye discomfort.

Fuchs (fewks) dystrophy usually affects both eyes. It may cause your vision to get worse over time. The disease often starts in the 30s and 40s, but many people with Fuchs dystrophy don't develop symptoms until they reach their 50s or 60s.

Some medicines and self-care steps may help relieve symptoms of Fuchs dystrophy. When advanced disease causes more-serious vision problems, cornea transplant surgery is the best way to restore vision.

Symptoms

As Fuchs dystrophy gets worse, symptoms often affect both eyes. Symptoms may include:

• Blurred or cloudy vision, sometimes described as a lack of clear vision.
• Changes in vision throughout the day. Symptoms are worse in the morning when you wake up and slowly get better during the day. As the disease gets worse, blurred vision may take longer to get better or does not get better at all.
• 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 get worse over time, see an eye care professional. The eye care professional may 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 treatment right away.

Causes

The cells lining the inside of the cornea are called endothelial cells. Those cells help maintain a healthy balance of fluid within the cornea and keep the cornea from swelling. In Fuchs dystrophy, the endothelial cells slowly die or do not work well, causing fluid buildup within the cornea. The fluid buildup, called edema, causes thickening of the cornea and blurred vision.

Fuchs dystrophy tends to run in families. The genetic basis of the disease is complex. Family members can be affected to different degrees or not at all.

Risk factors

Some factors make it more likely that you'll develop Fuchs dystrophy They include:

• Sex. Fuchs dystrophy is more common in women than in men.
• Genetics. Having a family history of Fuchs dystrophy increases your risk.
• Age. There is a rare early-onset type of Fuchs dystrophy that starts in childhood. Most cases start in the 30s and 40s, but many people with Fuchs dystrophy don't develop symptoms until their 50s or 60s.

Diagnosis

An eye care professional will test your vision. You also may have tests to help diagnose Fuchs dystrophy. Those tests may include:

• Cornea examination and grading. A member of your eye care team will use a special eye microscope called a slit lamp to look for drop-shaped bumps called guttae on the back surface of the cornea. This eye care professional will then check your cornea for swelling and stage your Fuchs dystrophy.
• Corneal thickness. An eye care professional may use a test called corneal pachymetry to measure the thickness of the cornea.
• Corneal tomography. Taking a special picture of your cornea helps an eye care professional look for swelling in your cornea. This test is called corneal tomography.
• Corneal cell count. Sometimes an eye care professional uses a special instrument to record the number, shape and size of the cells that line the back of the cornea. This test is not required.

Treatment

Some nonsurgical treatments may help relieve symptoms of Fuchs dystrophy. If you have advanced disease, an eye care professional may suggest surgery.

Medicines and other therapies

• Eye medicine. Saline (5% sodium chloride) eye drops 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. Surgical options include:

• Transplanting the inner layer of the cornea. This is called Descemet membrane endothelial keratoplasty, also known as DMEK. In this procedure, the back layer of the cornea is replaced with healthy endothelial cells from a donor. It is usually done with local anesthesia in an outpatient setting.
• Transplanting the cornea. If you have another eye condition or already had eye surgery, DMEK may not be an option. An eye care professional may recommend a partial-thickness cornea transplant. This is called Descemet-stripping endothelial keratoplasty, also known as DSEK. In rare cases, a full-thickness cornea transplant may be done. This type of transplant is called penetrating keratoplasty, also known as PK.

Potential future treatments

A variety of new treatments are being investigated that could change how Fuchs dystrophy is managed in the future. Since the discovery of the genetic mutation associated with most cases of Fuchs dystrophy, there is a better understanding of how the disease might develop. This offers the potential for nonsurgical therapies in the future. Various eye drop treatments are being developed and may enter clinical trials in the future. Novel surgical treatments also are being studied to find if they might be helpful.

Lifestyle and home remedies

Follow instructions from your eye care team to take care of your eyes. You also can try other things to help reduce glare and soothe your eyes.

• Use nonprescription salt solution (5% sodium chloride) eye drops 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 extra fluid in the cornea, which reduces swelling.

Preparing for an appointment

You may start by seeing an eye care professional called an optometrist or ophthalmologist. Or you may be referred immediately to an ophthalmologist who specializes in corneal disease.

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

What you can do

When you make the appointment, ask if there's anything you need to do in advance. Make a list of:

• Your symptoms, including any that seem unrelated to the reason for your appointment.
• Key personal information, including major stresses, recent life changes and family history of eye conditions.
• All medicines, vitamins or other supplements you take, including the doses.
• Questions to ask your doctor.

Take a family member or a friend along, if possible, to help you remember the information you're given. In addition, you might not want to drive yourself home if your pupils have been dilated for the exam.

For Fuchs dystrophy, questions to ask include:

• What's likely causing my symptoms?
• Other than the most likely cause, what are other possible causes for my symptoms?
• How will my vision be affected?
• What tests do I need?
• What's the best course of action?
• What are the alternatives to the primary approach you're suggesting?
• I have these other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• Should I see a specialist?
• 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

You'll likely be asked a few questions, such as:

• When did your symptoms begin?
• 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?