What is Fuchs' Endothelial Dystrophy?

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 it usually does not cause vision problems until the patient's 50s or 60s, though early signs of the disease may appear as early as the 30s. The disease is genetic, although it may be worsened by trauma to the eye or surgery.

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

Symptoms of Fuchs' endothelial dystrophy are often worst in the morning and decline throughout the day, since fluids causing the swellings evaporate more readily when the eyes are open. As the disease progresses, vision stays blurry throughout the day. In later stages, the accumulations of fluid in the cornea can also cause painful blisters.

The first line of treatment for Fuchs' endothelial dystrophy comprises methods of drying out the cornea. These include topical saline, therapeutic soft contact lenses, and the use of a hair dryer on the eyes. The last method requires holding the dryer, set with the fan on low and the temperature cool, at the side of the face in order to be gentle on the eyes. These methods of treatment for Fuchs' endothelial dystrophy only alleviate the symptoms and are not cures for the disease.

The only current cure for Fuchs' dystrophy is corneal transplant surgery, also called keratoplasty. There are many different forms of this surgery, which has been significantly improved in recent years. Traditionally, penetrating keratoplasty, in which the entire cornea is replaced, has been most common. Other surgeries, including lamellar keratoplasty and Descemet's stripping endothelial keratoplasty (DSEK), in which only a portion of the cornea is replaced, have become more common. One of the newest methods, Descemet Membrane Endothelial Keratoplasty (DMEK), is less invasive than other options, as it involves the transplant of only Descemet's membrane. Any patient with Fuchs' endothelial dystrophy should speak to a specialist about what type of surgery is best.