Fuchs Dystrophy

Fuchs' dystrophy is a rare disorder that affects the cornea — the transparent front surface of your eye. Although the cause of Fuchs' dystrophy is unknown, it often occurs as an inherited disorder.

Normally, the cells that line the back surface (endothelium) of the cornea prevent excess fluid from accumulating in the cornea. This helps the cornea maintain its transparency, but with Fuchs' dystrophy, those endothelial cells slowly deteriorate. As a result, fluid builds up in the cornea. This may cause swelling, cloudy vision, pain and loss of corneal transparency.   Fuchs' dystrophy causes a variety of vision problems and can eventually lead to blindness.      Symptoms Doctors may see early signs of Fuchs' dystrophy in people who are in their 30s and 40s, but most people don't experience visual symptoms or problems until they're in their 50s and 60s.  Signs and symptoms usually affect both eyes and include:

• Blurred vision on awakening that may gradually clear up as the day goes on
• Painful, tiny blisters (epithelial blisters) on the surface of your cornea — caused by excess fluid within the cornea
• Visual impairment, distorted vision and changes in vision
• Difficulty seeing at night
• Sensitivity to light
• Seeing halos around lights
• A cornea that is cloudy or hazy in appearance

When to see a doctor
If you experience some of these symptoms, and especially if they get worse over time, see your eye doctor (ophthalmologist or optometrist). If symptoms develop suddenly, call an ophthalmologist for an urgent appointment.  Many conditions that cause the same symptoms as Fuchs' dystrophy require prompt treatment.   Causes In some people with Fuchs' dystrophy, the cause is unknown. However, for many people it's inherited as an autosomal dominant familial condition, meaning that about half of an affected person's children also will have the disease. The extent to which relatives experience signs and symptoms may vary. For instance, a parent with a mild case and few symptoms could have a child with a severe case and numerous vision problems.   Risk Factors
Some people are more likely to develop Fuchs' dystrophy. They include:

• Women
• People with a first-degree relative (such as a parent or sibling) who has Fuchs' dystrophy
• People who are in their 50s and older

  Treatments Your doctor may suggest the following methods and procedures to improve your comfort or stop your symptoms and signs of Fuchs' dystrophy from worsening.

• Use eye drops or ointments to reduce the amount of fluid in your cornea.
•  Use a hair dryer and hold it at arm's length.   
• Wear soft contact lenses to improve vision and reduce discomfort.
• Receive a corneal transplant, also known as keratoplasty, which replaces damaged cornea tissue with healthy tissue from a donor. There are many types of corneal procedures — some replace only a few thin layers of the cornea, while others replace the entire cornea. For Fuchs' dystrophy, an increasingly common procedure replaces only the deep layers of the cornea, including the endothelium. This is sometimes referred to as endothelial keratoplasty or posterior lamellar keratoplasty.

*Corneal transplants are common and have high success rates. However, you may have to wait a long time for donor tissue to become available, and some people develop problems following transplants. About 20 percent of the time, the transplant recipient's body attempts to reject the new corneal tissue, according to the National Eye Institute. The rejection may cause increased sensitivity, redness, pain and worsening vision. Many times, rejection can be managed with topical eye medications. After corneal transplant, your eyes may feel uncomfortable. You'll need to use eye drops for several months to help your eyes heal. The majority of people who have a successful transplant for Fuchs' dystrophy continue to be free of symptoms for at least ten years.

• Participate in research or a clinical trial. Clinical trials involve studies of new ways to diagnose or treat a condition. Clinical trials don't always offer a definite diagnosis or cure, but they may allow you to take advantage of the latest knowledge about your condition.