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What is Fuchs' endothelial corneal dystrophy?

Fuchs' endothelial dystrophy is a painless, inherited disease of the cornea -- specifically of the endothelium. It tends to appear in every generation and is more common in women. The endothelium is the inner layer of the cornea, and its function is important to corneal health and clarity. The endothelium actively transports or draws fluid out of the cornea, keeping it thin and clear. If the endothelium does not work, the cornea gradually swells and becomes cloudy and degrades vision. Fuchs' dystrophy is a gradual progressive dysfunction of the endothelium, giving its victim declining vision. Fortunately, it is treatable, although treatment often requires surgery. Today's techniques, though, provide a high degree of success and much faster recovery than in the past. Consult your eye doctor for appropriate diagnosis if you think you or a family member might have Fuchs' dystrophy.
Fuchs’ dystrophy is a progressive disease affecting the part of the eye called the cornea. The cornea is like the crystal covering a clock face. It is a clear, round dome covering the iris, the colored ring in the center of the eye, and the pupil, the black circle in the middle of the iris. By helping to focus light as it enters the eye, the cornea plays an important role in vision.

Fuchs’ dystrophy reduces the number of specific cells (called endothelial cells) that make up the inner layer of the cornea. This reduction of cells causes the cornea to become unusually thick or puffy.

Also seen in Fuchs’ dystrophy are dewdrop-shaped outgrowths called guttata in the layer just underneath the endothelial cell layer called Descemet’s membrane.

These cell changes may cause the cornea to become swollen and cloudy, losing its crystal-clear transparency. Because Fuchs’ dystrophy is a progressive disease, over time, changes to the corneal cells may interfere with vision.

Fuchs’ dystrophy usually occurs after age 40. Studies show that it is an inherited condition.
Fuchs' dystrophy is a slowly progressing disease that usually affects both eyes and is slightly more common in women than in men. Although doctors can often see early signs of Fuchs' dystrophy in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.

Fuchs' dystrophy occurs when endothelial cells gradually deteriorate for no apparent reason. As more endothelial cells are lost over the years, the endothelium becomes less efficient at pumping water out of the stroma. This causes the cornea to swell and distort vision. Eventually, the epithelium also takes on water, resulting in pain and severe visual impairment.

Epithelial swelling damages vision by changing the cornea's normal curvature and causing a sight-impairing haze to appear in the tissue. Epithelial swelling also produces tiny blisters on the corneal surface. When these blisters burst, they are extremely painful.

At first, a person with Fuchs' dystrophy awakens with blurred vision that gradually clears during the day. This occurs because the cornea is normally thicker in the morning; it retains fluids during sleep that evaporate in the tear film while we are awake. As the disease worsens, this swelling remains constant and reduces vision throughout the day.

When treating the disease, doctors try to first reduce the swelling with drops, ointments, or soft contact lenses. They may also instruct a person to use a hair dryer, held at arm's length or directed across the face, to dry out the epithelial blisters. This can be done two or three times a day.

When the disease interferes with daily activities, a person may need to consider having a corneal transplant to restore sight. The short-term success rate of corneal transplantation is quite good for people with Fuchs' dystrophy. However, some studies suggest that long-term survival of the new cornea can be a problem.

This answer is based on source information from National Eye Institute.

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Important: This content reflects information from various individuals and organizations and may offer alternative or opposing points of view. It should not be used for medical advice, diagnosis or treatment. As always, you should consult with your healthcare provider about your specific health needs.