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Fuchs dystrophy

Definition

Fuchs' (pronounced "fooks") dystrophy is an eye disease in which cells lining the inner surface of the cornea slowly start to die off. The disease usually affects both eyes.

Alternative Names

Fuchs' dystrophy; Fuchs' endothelial dystrophy, Fuchs' corneal dystrophy

Causes

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.

Symptoms

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

Treatment

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.

Outlook (Prognosis)

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

Prevention

There is no known prevention. Avoiding cataract surgery or taking special precautions during cataract surgery may delay the need for a corneal transplant.

References

Cockerham GC, Kenyon KR. The corneal dystrophies. In: Tasman W, Jaeger EA, eds. Duane's Ophthalmology. 2013 ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:chap 16.

Shamie N, Phillips P, Terry MA. Descemet stripping automated endothelial keratoplasty. In: Tasman W, Jaeger EA, eds. Duane's Ophthalmology. 2013 ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2013:chap 29.

Vanmeter WS, Lee WB, Katz DG. Corneal edema. In: Tasman W, Jaeger EA, eds. Duane's Ophthalmology. 2013 ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2013:chap 16A.

Review Date:9/2/2014
Reviewed By:Franklin W. Lusby, MD, ophthalmologist, Lusby Vision Institute, La Jolla, CA. Also reviewed by David Zieve, MD, MHA, Isla Oglvie, PhD, and the A.D.A.M. Editorial team.

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Outcome Data

No data available for this condition/procedure.

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