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.
Also seen in Fuchs’ dystrophy, are dewdrop-shaped outgrowths called guttata in the layer just underneath the endothelial cell layer (called Descemet’s membrane).
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 somewhat more common in women and usually affects people in their 50s or 60s, although occasionally it appears earlier in one’s adulthood.
Fuchs’ dystrophy can be inherited, which means it can be passed down from parents to children. If either of your parents has the disease, you have a 50% chance of developing the condition.
However, the condition may also occur in persons without a known family history of the disease.
Fuchs’ dystrophy is more common in women than in men. Vision problems usually do not appear before age 50, although doctors may be able to see signs of the disease in affected persons by their 30s or 40s.
Fuchs’ dystrophy affects the thin layer of cells that line the back part of the cornea. These cells help pump excess fluid out of the cornea. As more and more cells are lost, fluid begins to build up in the cornea, causing swelling and a cloudy cornea.
At first, fluid may build up only during sleep, when the eye is closed. As the disease gets worse, small blisters may form. The blisters get bigger and may eventually break, causing eye pain.
Fuchs’ dystrophy can also cause the shape of the cornea to change, leading to more vision problems.
Fuchs’ Dystrophy Causes
Fuchs’ dystrophy, when the endothelial cells in the cornea diminish and the cells stop processing water properly, is largely hereditary. Those who inherited the condition from a parent have a 50 percent chance of passing it on to their children.
- Eye pain
- Eye sensitivity to light and glare
- Foggy or blurred vision, at first only in the mornings
- Seeing colored halos around lights
- Worsening vision throughout the day
Exams and Tests
A doctor can diagnose Fuchs’ dystrophy during a slit-lamp examination.
Other tests that may be done include:
- Pachymetry — measures the thickness of the cornea
- Specular microscope examination — allows the doctor to look at the thin layer of cells that line the back part of the cornea
- Visual acuity test
Eye drops or ointments that draw fluid out of the cornea are used to relieve symptoms of Fuchs’ dystrophy.
If painful sores develop on the cornea, soft contact lenses or surgery to create flaps over the sores may help reduce pain.
The only cure for Fuchs’ dystrophy is a corneal transplant.
Until recently, the most common type of corneal transplant was penetrating keratoplasty. During this procedure, a small round piece of the cornea is removed, leaving an opening in the front of the eye.
A matching piece of cornea from a human donor is then sewn into the opening in the front of the eye.
A newer technique called endothelial keratoplasty (DSEK, DSAEK, or DMEK) has become the preferred option for patients with Fuchs’ dystrophy. In this procedure, only the inner layers of the cornea are replaced, instead of all the layers.
This leads to a faster recovery and fewer complications. Stitches are usually not needed.
Treatment of Fuchs’ dystrophy will vary depending on the stage at which it was diagnosed. The frequency of follow-up visits to your doctor will also vary.
Early stage Fuchs’ dystrophy may involve annual visits, but more advanced cases or certain treatments may call for visits every few months or even more frequently. Your provider will tell you what your condition requires.
It is not possible to stop the change in the corneal tissue, so treatment of Fuchs’ dystrophy will focus on addressing your symptoms and associated pain.
Because part of the underlying problem, especially in the early stages, is the collection of fluid, your doctor may recommend treatments to help the extra water evaporate, such as exposure to warm, dry air (vents in a car, blow dryer, etc.).
Your doctor may also prescribe ointments or drops or bandage contact lenses. In very advanced cases, your doctor may recommend a corneal transplant or other surgical procedure.
Fuchs’ dystrophy gets worse over time. Without a corneal transplant, a patient with severe Fuchs’ dystrophy may become blind or have severe pain and very reduced vision.
Mild cases of Fuchs’ dystrophy often worsen after cataract surgery. A cataract surgeon will evaluate this risk and may modify the technique or the timing of your cataract surgery.
When to Contact a Medical Professional
Call your health care provider if you have:
- Eye pain
- Eye sensitivity to light
- The feeling that something is in your eye when there is nothing there
- Vision problems such as seeing halos or cloudy vision
- Worsening vision
There is no known prevention. Avoiding cataract surgery or taking special precautions during cataract surgery may delay the need for a corneal transplant.