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Fuchs’ Dystrophy

What is Fuchs’ dystrophy?

Fuchs' (pronounced "fooks") dystrophy, also called endothelial dystrophy, is a condition where the endothelial cells that line the inner surface of the cornea slowly start to die off. As more and more cells are lost, fluid begins to build up, causing swelling in the cornea. Most patients with Fuchs dystrophy have a very mild form that never affects vision. When it does, it usually occurs in middle-age or later.  Over time, vision will worsen, along with glare, sensitivity to light and difficulty seeing at night, and is often worse in the morning than later in the day. Pain or severely decreased vision can occur later in the course of the disease.  Fuchs’ dystrophy can be inherited, though it also occurs in people without a known family history of the disease. It is more common in women than in men.

How is Fuchs’ dystrophy diagnosed?

Your ophthalmologist can diagnose the disease through a slit-lamp exam, which uses a high-intensity beam to show the eye structure in great detail. Other tests may include pachymetry, (link to diagnostic page) which measures the thickness of the cornea; specular microscopy, (link to diagnostic page) which examines the thin layer of cells that line the back part of the cornea; and a visual acuity test.

What is the treatment for Fuchs’ dystrophy?

Eye drops or ointments that draw fluid out of the cornea are the first step in relieving the swelling from Fuchs’ dystrophy. Blowing cool or warm (not hot) air from a blow dryer upon wakening can often improve vision earlier in the day in the early stages of the disease. If blisters develop on the cornea, soft contact lenses may temporarily help reduce the pain.

Because the condition worsens over time, a corneal transplant may be necessary to prevent blindness or severely reduced vision.  Currently, endothelial transplants (Descemet’s stripping endothelial keratoplasty [DSEK] or Descemet’s membrane endothelial keratoplasty [DMEK] are the surgical treatments of choice. In eyes with significant corneal scarring, a full thickness corneal transplant may be required to achieve good vision.

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