Fuch’s Dystrophy

Fuchs’ dystrophy (fooks DIS-truh-fee) is an uncommon, slowly progressive disorder that affects the cornea — the transparent front surface of your eye. Fuchs’ dystrophy is a type of corneal dystrophy, a group of conditions that may cause a hazy deposit to build up over the cornea.

Normally, the cells that line the back surface (endothelium) of the cornea prevent excess fluid from accumulating. This helps the cornea maintain its transparency. But with Fuchs’ dystrophy, those endothelial cells slowly deteriorate, lose function and die. As a result, fluid builds up in the cornea. This may cause swelling, cloudy vision, pain and loss of corneal transparency.

Although the cause of Fuchs’ dystrophy is unknown, it may be inherited. Treatments, including surgery, are available for Fuchs’ dystrophy.

Doctors may see early signs of Fuchs’ dystrophy in people who are in their 30s and 40s. But most people don’t experience symptoms or problems until they’re in their 50s and 60s. Signs and symptoms usually affect both eyes and may include:

  • Blurred vision on awakening that may gradually clear up as the day goes on
  • Other types of visual impairment, including distorted vision, sensitivity to light, difficulty seeing at night and seeing halos around lights
  • Generalized eye discomfort
  • Painful, tiny blisters (epithelial blisters) on the surface of your cornea — caused by excess fluid within the cornea
  • A cornea that is cloudy or hazy in appearance
  • Blindness — may occur late in the disorder

 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

  • Use eye medication. Eyedrops or ointments can reduce the amount of fluid in your cornea.
  • Dry your eyes. Use a hair dryer and hold it at arm’s length. Direct warm — not hot — air across your face two or three times a day to evaporate excess fluid in the cornea and dry out blisters.
  • Wear soft contact lenses. Soft contact lenses can improve vision and reduce discomfort.
  • Receive a corneal transplant. This surgical procedure, also known as keratoplasty, replaces damaged cornea tissue with healthy tissue from a donor. There are many types of corneal procedures. Some procedures 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 endothelial keratoplasty.Corneal transplants are common and effective. However, you may have to wait a long time for donor tissue to become available. And some people develop problems following transplants, such as rejection of the new corneal tissue. 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 eyedrops 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 years after the transplant.

 

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