Fuchs’ Endothelial Dystrophy

What is Fuchs’ dystrophy?

Also known as Fuchs’ corneal endothelial dystrophy (FCED) and Fuchs’ endothelial dystrophy (FED), this is a slowly progressing corneal disease that typically affects both eyes, leading to a gradual decline in vision due to corneal swelling (edema) and clouding. Although early signs of Fuchs’ dystrophy are sometimes seen in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s. It is slightly more common in women than in men.

Fuchs’ dystrophy may be discovered as an incidental finding at a routine visit by your ophthalmologist during assessment for cataract surgery. As a result of irregularities on the inner surface of the cornea, affected individuals may notice a reduction in the quality of vision, as well as glare or halos, particularly when driving at night.

Is Fuch's dystrophy hereditary?

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. The genetic basis of the disease is complex — family members can be affected to varying degrees or not at all.

However, the condition may also occur in people without a known family history of the disease.

What causes Fuchs' endothelial dystrophy?

Fuchs’ dystrophy can have a genetic cause, but it can also occur without a previous family history of the disease. In many cases, the reason is unknown.

Fuchs’ dystrophy is a degenerative disease of the endothelium, characterized by the death of endothelial cells. The endothelium is the outer layer of the cornea.  The endothelium cells usually pump fluid from the cornea at a specific level that maintains clarity and proper hydration.  As the cells die off, the endothelium becomes less efficient at pumping water out. This causes the layers of the cornea to get waterlogged, swollen, and puffy, finally resulting in pain and severe visual impairment.  Light can no longer pass through the cornea correctly, resulting in cloudy or blurred vision.

Corneal swelling:

  1. Changes the normal corneal curvature
  2. Causes cloudiness of the tissue
  3. Creates tiny blisters on the surface called bullae (BULL-eye). This condition is known as bullous keratopathy.

What are the risk factors for Fuchs' dystrophy?

Factors that increase your risk of developing Fuchs’ dystrophy include:

  • Sex
    Fuchs’ dystrophy is slightly more common in women than in men.
  • Genetics
    Having a family history of Fuchs’ dystrophy increases your risk. If either of your parents has the disease, you have a 50% chance of developing the condition.
  • Age
    Although there’s a rare early-onset type of Fuch’s dystrophy that begins in childhood, typically the disease starts in the 20s and 30s, with symptoms developing in the 50s and 60s.

Smoking and having diabetes might also put you at higher risk of the disease.

What are the symptoms of Fuchs' dystrophy?

The first symptom is blurry vision that clears during the day.

Suppose the endothelial cells’ pumping action isn’t working correctly, and excess fluids have accumulated while you were sleeping, with your eyes closed. In that case, the cornea becomes slightly waterlogged, swollen, and thicker in the morning.  The excess fluid evaporates normally once you’re up and about.

However, as the condition worsens, the swelling doesn’t improve after you wake up and begins to reduce your vision throughout the day.

Fuchs’ dystrophy symptoms include:

  • Foggy or blurred vision occurs in the morning after awakening and gradually improves during the day. As the disease progresses, blurred vision either takes longer to improve or doesn’t improve.
  • Glare and sensitivity to light, which can decrease your vision in both dim and bright light
  • Seeing colored halos around lights
  • Difficulty seeing at night
  • Eye pain or grittiness, feeling that something is in your eye (foreign body sensation) from tiny blisters on the surface of your cornea

What are the stages of Fuchs' dystrophy?

People in their 30s and 40s may have Fuchs’ dystrophy but not know it. Vision problems might not appear until age 50 or later. Women are more likely than men to have Fuchs’ dystrophy.

Fuchs’ dystrophy has two stages.

  • Stage 1
    You may notice a few, or even none, problems. Vision is usually hazy in the morning but gets better throughout the day. This is because your eyes typically stay moist when they are closed during sleep. But when you are awake, the fluid dries normally.
  • Stage 2
    Vision remains blurry all day. Too much fluid builds up during sleep and not enough dries up during the day. Tiny blisters may form in the cornea. The blisters get bigger and eventually break open, causing eye pain. See bullous keratopathy.

What are the treatments of Fuchs' dystrophy?

Some medications and self-care steps may help relieve your Fuchs’ dystrophy signs and symptoms. But when the disorder is advanced and you’ve lost vision, the only way to restore vision is with cornea transplant surgery.

Fuchs Endothelial Dystrophy
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