All About Fuchs' dystrophy

Fuchs' dystrophy, also known as Fuchs' endothelial dystrophy, is a slowly progressing corneal 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.

The condition was first described by Austrian Ernst Fuchs (1851-1930), for whom it is named.

Etiology

In Fuchs' dystrophy endothelial cells gradually deteriorate without any apparent reason (other than aging). Progressive endothelial cell loss causes relative influx of aqueous humor into the cornea, leading to swelling (corneal stromal edema), with resultant distorted vision. Eventually, the epithelium also becomes edematous, resulting more severe visual impairment. Focal areas or blisters of epithelial edema ("bullae") may be particularly painful.

Endothelial cell loss may be aggravated or accelerated by intraocular trauma or surgery. A common scenario involves excessive corneal edema following cataract surgery.

Signs and symptoms

At first, a person with Fuchs' dystrophy will awaken with blurred vision that will gradually clear 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 will remain constant and reduce vision throughout the day.

Treatment

When treating the disease, doctors will try first to reduce the swelling with drops, ointments, or soft contact lenses. They also may 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.

References

This article was originally based on content from the National Eye Institute web page Facts About The Cornea and Corneal Disease. The National Eye Institute (NEI) is one of the United States federal government's National Institutes of Health.