Fuchs' dystrophy reduces the number of specific cells (called endothelial cells) that make up the inner layer of the cornea. Endothelial cells are key to processing water that makes up the corneal cell structure. When the endothelial cells diminish, the cells stop processing water properly and fluid starts to build up. The corneal tissue gradually thickens, causing the cornea to become swollen and cloudy, losing its crystal-clear transparency.
Because Fuchs' dystrophy is a progressive disease, over time, changes to the corneal cells may interfere with vision. The condition can result in corneal scar tissue, which may have to be removed surgically, and can even lead to blindness.
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 and die off. As a result, fluid builds up in the cornea. This may cause swelling, cloudy vision, pain and loss of visual clarity.
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 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 (astigmatism)
- A cornea that is cloudy or hazy in appearance
The method of treatment greatly depends on the progression of Fuchs' Dystrophy. Your doctor may instruct you to use eyedrops or ointments to reduce the amount of fluid in your cornea or wear soft contact lenses to improve vision and reduce discomfort. If the disease is severe, a corneal transplant, also known as keratoplasty, may be indicated where damaged cornea tissue is replaced 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. 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 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 at least ten years.