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What’s Fuchs’ dystrophy?

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Fuchs endothelial dystrophy is a genetic disease that affects the cornea and can cause vision problems in patients in their 50s or 60s. Symptoms include blurry vision and painful blisters. Treatment methods only relieve symptoms, and the only cure is corneal transplantation.

Fuchs endothelial dystrophy is a degenerative disease of the cornea, the front part of the eye that covers the iris and pupil. The disease is named after the Austrian ophthalmologist who first described it in 1910, Ernst Fuchs. Fuchs endothelial dystrophy is diagnosed more often in women than in men and does not usually cause vision problems until the patient’s 50s or 60s, although the first signs of the disease can appear as early as the 30s. The disease is genetic, although it can be aggravated by trauma to the eye or surgery.

Fuchs endothelial dystrophy is caused by degeneration of the corneal endothelium, the innermost membrane of the cornea. The cells of this membrane are responsible for pumping out accumulations of fluid to keep the cornea clean. Fuch’s dystrophy is characterized by a thickening of a collagen layer of the cornea, Descemet’s membrane, eventually leading to coronal edema or swelling and loss of vision.

Symptoms of Fuchs endothelial dystrophy are often worst in the morning and decrease throughout the day, because the fluids that cause swelling evaporate more easily when the eyes are open. As the disease progresses, vision becomes blurry throughout the day. In later stages, accumulations of fluid in the cornea can also cause painful blisters.

The first line of treatment for Fuchs endothelial dystrophy includes methods to dry the cornea. These include topical saline, therapeutic soft contact lenses, and using a hair dryer on the eyes. The last method involves holding the hairdryer, set on low fan and cool, to the side of your face so it’s gentle on your eyes. These treatment methods for Fuchs endothelial dystrophy only relieve symptoms and are not cures for the disease.

The only current cure for Fuchs’ dystrophy is corneal transplantation, also called keratoplasty. There are many different forms of this surgery, and it has been greatly improved over the past few years. Traditionally, penetrating keratoplasty, in which the entire cornea is replaced, has been the most common. Other surgeries, including lamellar keratoplasty and Descemet’s stripping endothelial keratoplasty (DSEK), in which only part of the cornea is replaced, have become more common. One of the newer methods, Descemet’s membrane endothelial keratoplasty (DMEK), is less invasive than other options, as it involves transplanting only the Descemet’s membrane. Any patient with Fuchs endothelial dystrophy should talk to a specialist about what type of surgery is best.

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