Fuchs’ Dystrophy

Fuchs’ dystrophy is an inherited and progressive condition that affects the cornea (dome-shaped outer layer of your eye) and which can cause impaired vision and discomfort in the eye. 

Some people are diagnosed with the disorder in their 30s and 40s but it is most often found in people in their 50s and 60s.

Exact causes are unknown.

Treatments include eye drops, eye ointments and cornea replacement surgery.

What Is Fuchs’ Dystrophy?

Fuchs’ dystrophy is an eye disorder that affects the cornea. Although people are born with the condition, it may remain asymptomatic (undetectable) until middle age or later.

As the condition progresses, the layer of cells responsible for maintaining proper fluid levels in the cornea (called endothelium), deteriorates and eventually dies off, causing tiny bumps (known as guttae) to form at the back of the cornea.

The cells normally pump blood from the cornea to keep it clear. When enough cells die off, fluid builds up in the cornea, resulting in swelling (known as corneal edema). The swelling causes vision to be cloudy, blurry or hazy.

Although doctors usually diagnose the disease when someone is in their 30s or 40s, most people do not develop symptoms until they reach their 50s and 60s.

Some self-care tips and medications may relieve symptoms of Fuchs’ dystrophy. However, if the disorder is advanced and vision is severely affected, surgery may be the only option to restore your vision.

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Stages and Symptoms of Fuchs’ Dystrophy

Fuchs’ dystrophy has two stages — stage 1 and stage 2.

Stage 1

In the early stage (stage 1), people notice few if any symptoms. Excess fluid builds up overnight during prolonged sleep, causing hazy or blurry vision upon waking up.

Haziness can last for hours, but vision usually improves over the course of the day as excess fluid is drawn out of the cornea. 

Stage 2

In the later stage (stage 2), blurry or hazy vision does not get better as the day goes on. Too much fluid builds up during sleep and not enough dries up over the course of the day.

Tiny blisters (guttae) also may form in the cornea. If they grow large enough and break open, the result is eye pain.

Other symptoms include:

• Sandy or gritty feeling in your eyes, occasionally accompanied by sharp eye pain
• Discomfort in bright light
• Halos and/or glares from bright lights 
• Blurred or cloudy vision combined with poor contrasting colors  
• Fluctuating eyesight throughout the day or from one day to the next which is usually worse in the mornings or on humid rainy days. 

Causes of Fuchs’ Dystrophy 

Fuchs’ dystrophy is caused by the destruction of endothelium cells in the cornea. However, doctors and researchers do not know the exact cause of cellular destruction.

People who have Fuchs’ dystrophy usually inherit it, although the genetic basis of the disease is complex. If someone in your family has the condition, you are at a greater risk of developing the disorder.

The condition can occur without a known family history of the disease. 

Risk Factors Associated with Fuchs’ Dystrophy

Factors that may increase your risk of developing Fuchs’ dystrophy include gender, genetics, age and whether you smoke.

• Gender. According to the National Eye Institute, Fuchs’ dystrophy affects more women than men.
• Genetics. If Fuchs’ dystrophy runs in your family, you are more likely to have the genes to develop the malady. 
• Age. The disease typically starts developing in the 30s and 40s, with symptoms typically presenting a decade or two later when people are in their 50s and 60s.
• Smoking also increases the risk of developing Fuchs’ dystrophy.

How Is Fuchs’ Dystrophy Diagnosed?

Ophthalmologist or optometrists diagnose Fuchs’ dystrophy by giving a comprehensive eye exam. They are likely to perform one or more of the following tests to confirm any diagnosis:

• Slit lamp microscopy
• Pachymetry
• Confocal/specular microscopy
• Visual acuity tests

Slit Lamp Microscopy

The doctor will examine the cornea in high magnification to look for subtle changes in the appearance of the cells and irregular bumps (guttae) on the inside surface of the cornea. They will also assess your cornea for swelling and confirm the stage of the condition.

Pachymetry

Your doctor might also perform a measurement of your corneal thickness to detect increased thickness of the cornea that might indicate swelling caused by Fuchs’ dystrophy.

Confocal/Specular Microscopy

This special type of microscope projects light to allow your doctor obtain a special photograph of your cornea (tomography) and detect early signs of corneal swelling. The doctor might also use this instrument to measure the number, density and shape of endothelial cells that line the back of the cornea.

Visual Acuity Tests

Executed with an eye chart during a comprehensive eye exam, acuity tests are designed to reveal decreased vision from corneal swelling.

Treatment Options for Fuchs’ Dystrophy

Ophthalmologists and optometrists turn to medical treatments and over-the-counter options when they have a patient with Fuchs’ dystrophy.

Home Remedies for Fuchs’ Dystrophy

Medical professionals have no way to encourage the growth of endothelial cells. However, you can take steps to ease the symptoms associated with Fuchs’ dystrophy including: 

• Blow drying your face with a hair dryer set on low and held at an arm’s length can help dry the surface of your cornea. 
• Using over the counter eye drop medication such as saline eyedrops (with 5 percent Sodium chloride).

Medical Treatments for Fuchs’ Dystrophy

The primary medical treatments for Fuchs’ dystrophy are: 

• Prescription eye drops or ointments to reduce pain and swelling 
• Soft contact lenses to relieve pain
• Surgery can remove significant scarring of the cornea through a corneal transplant or endothelial keratoplasty.

Surgeries

During corneal transplant surgery, the center of your cornea is replaced with the healthy cornea from a donor. 

In endothelial keratoplasty (EK), portions of the cornea affected by Fuchs’ dystrophy (Descemet’s membrane and corneal endothelium) are replaced with healthy corneal tissue from a donor.

Types of endothelial keratoplasty commonly performed include Descemet’s membrane endothelial keratoplasty (DMEK) and Descemet stripping automated endothelial keratoplasty (DSEK). 

Complications Associated with Fuchs’ Dystrophy

If you opt for a corneal transplant, it is possible your body may reject the new tissue. Signs that your body is rejecting the donor tissue include:

• Sensitivity to light  
• Hazy or cloudy vision
• Eye pain
• Eye redness

If you notice any of these symptoms or experience other unusual eye problems, contact your doctor right away. Your doctor will prescribe medication to prevent a rejection. 

Outlook/Prognosis for Fuchs’ Dystrophy

Fuchs’ dystrophy is a progressive disorder. As such, it is best to catch the disease in its earliest stages to control any eye discomfort and prevent vision problems.

The challenge is that most people don’t know that they have Fuchs’ dystrophy until its symptoms present themselves.

Although there is no cure for this corneal disease, there are treatment options that can help control its effects on your eye comfort and vision. It is also important to remember that Fuchs’ dystrophy gets worse over time.

Without treatment, a person with Fuchs’ dystrophy might experience severe pain and severely impaired vision. Having regular eye exams can help catch eye conditions like Fuchs’ dystrophy in their earlier stages before they progress.

Frequently Asked Questions Regarding Fuchs’ Dystrophy

Can you go blind from Fuchs’? 

Fuchs’ dystrophy does not cause total blindness even in its advanced stages since the retina and optic nerve are not affected. However, it can severely impair Vision and interfere with your daily activities as it progresses. 

How common is Fuchs’ disease? 

Early onset variant of Fuchs’ dystrophy is rare, although researchers do not know of its exact prevalence. The late-onset form of Fuchs’ dystrophy is common, affecting approximately 4 percent of people over the age of 40 in the United States. The condition also affects women 2 to 4 times more frequently than men.

What is the best treatment for Fuchs’ dystrophy?

Currently, the best way to treat Fuchs’ dystrophy in its earlier stages is to use eye drops or ointments to remove fluids and ease corneal swelling. For advanced Fuchs’ dystrophy, the best treatment is a cornea transplant.

References

1. Cornea. (March 2016). American Academy of Ophthalmology.

2. Endothelium. (March 2016). American Academy of Ophthalmology.

3. What Is Fuchs’ Dystrophy? (September 2021). American Academy of Ophthalmology.

4. Corneal Conditions. (August 2019). National Eye Institute.

5. Corneal Transplantation. (June 2012). Johns Hopkins Medicine.

6. Descemet membrane endothelial keratoplasty (DMEK) for Fuchs endothelial dystrophy: review of the first 50 consecutive cases. (October 2009). Eye.

7. Descemet-stripping automated endothelial keratoplasty: a successful alternative to repeat penetrating keratoplasty. (April 2011). Clinical & Experimental Ophthalmology.

8. Fuchs’ Dystrophy. (June 2017). Cornea Research Foundation of America.