Fuchs’ Dystrophy

What is Fuchs’ Dystrophy?

Fuchs’ dystrophy is a genetic disease of the cornea. It occurs when the innermost cells of the cornea called the endothelium die off. These cells normally pump fluid from the cornea to keep it clear. As these cells decrease in number, fluid builds up and the cornea gets swollen causing the vision to become cloudy.

Fuchs’ dystrophy has two stages. In the early stage (stage 1), you may notice few, if any, problems. Vision may be hazy in the morning but gets better throughout the day. In the more advanced, stage 2, vision remains blurry all day. Fluid builds up in the cornea during sleep and not enough dries up during the day. Also, tiny blisters may form on the surface of the cornea and may cause irritation or pain. People in their 30s and 40s may have Fuchs’ dystrophy but not know it. Vision problems might not appear until age 50 or later. Women are more likely than men to have Fuchs’ dystrophy.

Fuchs’ Dystrophy Overview

How Is Fuchs’ Dystrophy Diagnosed?

Fuchs’ dystrophy can be diagnosed during a routine eye exam. In people with Fuchs’, the cornea displays small “bumps” on its back surface called guttae. Your doctor can detect these with a specialized microscope called a slit lamp. Usually, Fuchs’ dystrophy can be diagnosed long before patients have any visual symptoms.

As Fuchs’ dystrophy gets more severe your eye doctor may detect swelling in the cornea or small blisters on the surface of the eye. An advanced corneal imaging system, called specular microscopy, can be used to determine the severity of Fuchs’ dystrophy. This microscope takes a picture of the individual cells, called the endothelium, on the back surface of the cornea. With this instrument, your eye doctor can determine the health of these cells and the severity and prognosis of the disease. It may also be useful to measure the thickness of the cornea with an ultrasonic pachymeter. By measuring corneal thickness, it is possible to determine the progression of the dystrophy.

Treatment for Fuchs’ Dystrophy

In the early stages, Fuchs’ dystrophy may not affect your vision and no treatment is needed. If some mild early morning blurry vision occurs, eyedrops can be used to reduce some of the swelling. As the condition advances a corneal transplant may be necessary to restore vision. The surgeons at Northeast Ohio Eye Surgeons are at the forefront of corneal transplant technology. To treat Fuchs’ dystrophy, Dr. Jones and Dr. Lohman use the most advanced techniques of corneal transplantation — DMEK and DSAEK. These procedures provide the best vision and fastest visual recovery possible for patients with Fuchs’ dystrophy. With DMEK and DSAEK, 99% of your cornea is left intact and only the damaged innermost layer of the cornea is replaced. It is sometimes possible to restore 20/20 vision with these advanced surgeries.

More information about Fuchs’ dystrophy can be found at The Corneal Dystrophy Foundation. They also have a support group, called Fuchs’ Friends, where people with Fuchs’ dystrophy can talk with others who have the same condition.