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What is Fuchs' Corneal Dystrophy?

Who is at risk for Fuchs endothelial corneal dystrophy?

What are the symptoms of Fuchs endothelial corneal dystrophy?

What is the treatment for Fuchs endothelial corneal dystrophy?

Where can I get more information on Fuchs endothelial corneal dystrophy?

 

What is Fuchs endothelial corneal dystrophy?

Fuchs’ corneal dystrophy is a disease of the endothelial cells that line the back of the cornea. The cornea is the clear tissue on the surface of the eye in front of the iris. These endothelial cells are responsible for pumping fluids out of the cornea to keep it clear and allow the light to pass. In Fuchs’ dystrophy, the endothelial cells gradually die off, and the pumping function is impaired. The cornea becomes thickened and cloudy, which eventually causes a decline in vision. When the swelling reaches the outer surface of the cornea, it can also form small blisters and cause pain. Early in the course of the disease, the endothelial cells secrete abnormal material that deposits on the back of the cornea, forming characteristic outgrowths, the “guttae.” These can be seen during an eye exam, and their presence is essential for diagnosing the disease.

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Who is at risk for Fuchs endothelial corneal dystrophy?
Fuchs’ dystrophy is a relatively common disease that affects older people. It is not preventable. For unknown reasons, it is more common and more severe in women. In approximately one-third of cases, this disorder is hereditary. A mutation in a type of collagen gene has been found in some patients who develop the disease at an unusually early age, e.g., in their thirties. Endothelial cell loss in people with Fuchs’ may be aggravated or accelerated by eye trauma or surgery. For this reason, it is important to diagnose the disease early for good management, especially in the presence of other eye diseases like cataract and glaucoma, which may necessitate intraocular surgeries.

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What are the symptoms of Fuchs endothelial corneal dystrophy?
Fuchs’ progresses slowly and affects both eyes. Typically, it can be detected clinically by age 40 or 50, but usually does not produce visual symptoms until a decade later, as the severity of the corneal swelling progresses. At first, the only symptoms are a painless decrease in vision and an increase in glare. These symptoms are usually worse in the morning and gradually improve during the day. As the disease advances, the blurred vision needs more time to clear, and episodes of pain from ruptured blisters can occur. Eventually, vision becomes severely and persistently limited.

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What is the treatment for Fuchs endothelial corneal dystrophy?

Currently the only effective cure for Fuchs’ is a corneal transplant, which involves eye surgery. Non-surgical management of the disease is limited, and should be directed by an ophthalmologist. In the early stages, special eye drops may help reduce corneal swelling and cloudiness. A hairdryer, at low setting, held at arm’s length in the direction of the eyes, can be helpful in alleviating the symptoms. And a special soft contact lens can be used to decrease the irritation and pain. Corneal transplantation (keratoplasty) is the only effective treatment available to restore vision and eliminate the pain in later stages of the disease. The timing for surgery usually depends on how the disease’s symptoms affect the patient’s quality of life and daily activities. Different types of procedures are available, depending on the case. A full-thickness corneal transplant replaces the whole cornea with a disease-free cornea from an eye bank donor. A newer technique, also performed frequently, consists of removing only the inner part of the cornea containing the diseased cells, and replacing it with healthy tissue from a donor cornea.

Where can I get more information about Fuchs endothelial corneal dystrophy?

The National Eye Institute (NEI) has a website for corneal diseases that includes a section on dystrophies:

One of several support groups

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