What You Need to Know About Fuchs' Dystrophy

Medically Reviewed By Leela Raju, MD
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Fuchs’ dystrophy is an eye condition wherein the endothelial cells in the cornea malfunction. These cells are responsible for keeping the cornea clear by removing fluid. As such, this condition causes fluid buildup and swelling. People with Fuchs’ dystrophy experience hazy or blurry vision that progressively worsens. While there is no cure for the condition, treatments are available to help manage vision difficulties and pain.

Other names for Fuchs’ dystrophy include Fuchs’ endothelial dystrophy and Fuchs’ endothelial corneal dystrophy.

Read on to learn more about the causes, symptoms, diagnostic process, and treatments for Fuchs’ dystrophy.

What are the causes of Fuchs’ dystrophy?

A blurry picture of a person on a bicycle
Lauren Lee/Stocksy United

The exact cause of Fuchs’ dystrophy is unclear. Some people inherit the condition from their parents, while others develop it without a known family history.

In Fuchs’ dystrophy, endothelial cells in the cornea begin to malfunction due to the thickening of their supporting layer. These cells line the back of the cornea and remove fluid to keep your vision clear. When endothelial cells malfunction and begin to die off, fluid builds up in the cornea. This buildup causes swelling and affects your vision and sensitivity to light.

What are the stages of Fuchs’ dystrophy?

There are 4 stages of Fuchs’ dystrophy.

Description
Stage 1In this stage, endothelial cells produce scattered guttae, or collagen droplets, in the cornea’s middle. This stage is usually asymptomatic.
Stage 2As the condition progresses, the guttae start to join together. The endothelial cells become larger and lose their characteristic six-sided shape. Blurry vision may be worse in the morning due to overnight fluid buildup in the cornea.
Stage 3In this stage, doctors may be able to see epithelial bullae or blisters on the cornea. If the bullae rupture, they can cause pain.
Stage 4People with stage 4 Fuchs’ dystrophy experience significant vision deterioration due to subepithelial scarring.

What are the symptoms of Fuchs’ dystrophy?

A close-up of an eye with a cloudy cornea
Fuchs dystrophy results in fluid buildup, which can make the cornea look cloudy. Copyright International Journal of Ophthalmology Press CC-BY-NC-ND 4.0 International

The symptoms of Fuchs’ dystrophy worsen as the condition progresses into the later stages.

In its early stages, Fuchs’ dystrophy may not cause noticeable symptoms. Blurry or hazy vision may be present after you wake up but will improve over the day.

In the later stages, your vision may remain blurry or hazy throughout the day and will continue to deteriorate. If blisters on the cornea rupture, you may experience pain.

Other symptoms can include increased sensitivity to light and a gritty feeling in the eyes.

Most people develop late-onset Fuchs’ dystrophy after age 40. The early onset form is much less common.

How do doctors diagnose Fuchs’ dystrophy?

In addition to assessing your medical history, there are a few methods doctors can use to diagnose Fuchs’ dystrophy:

  • Slit-lamp examination: This examination allows doctors to determine the presence of guttae. In the later stages, blisters and scarring may also be visible.
  • Specular microscopy: Doctors use this test to assess if the endothelial cells are dying off.
  • Pachymetry: Pachymetry helps determine the thickness of the central cornea. Increased thickness can indicate fluid buildup.

What are the treatments for Fuchs’ dystrophy?

There is currently no way to cure Fuchs’ dystrophy. However, treatments are available to manage pain and vision difficulties.

Topical treatments

Hypertonic sodium chloride eye drops or ointments may help the cornea dry out. Some people with early stage Fuchs’ dystrophy may also benefit from using a hair dryer to blow warm air onto the cornea and encourage drying.

Additional topical medications may reduce eye pressure and treat ruptured blisters.

Surgery

Some symptoms may require surgery to treat.

Keratoplasty is surgery on the cornea. A subtype of keratoplasty, the Descemet stripping automated endothelial keratoplasty (DSAEK), is currently the most common surgery for Fuchs’ dystrophy.

DSAEK removes part of the endothelial layer and the Descemet membrane separating the endothelial layer from the thickest part of the cornea. Doctors then transplant a section of the endothelial layer from a healthy donor into the eye.

A full corneal transplant may be necessary to treat significantly impaired vision or extensive corneal scarring.

What is the outlook for people with Fuchs’ dystrophy?

Fuchs’ dystrophy develops slowly. As such, many people may be able to go without treatment for several decades.

Fuchs’ dystrophy is incurable. However, people who undergo surgery to repair part or all of the cornea typically experience significant vision improvement.

What are some potential complications of Fuchs’ dystrophy?

The main complications of Fuchs’ dystrophy are vision impairment and corneal scarring. In addition, surgery to address the condition may cause complications, such as:

  • detachment of the transplanted tissues
  • rejection or failure of the transplanted tissues
  • fluid leakage
  • infection
  • formation of cataracts
  • astigmatism

What are the risk factors for Fuchs’ dystrophy?

Although not everyone with a family history of Fuchs’ dystrophy will develop the condition, it may increase your risk.

There are also some nonhereditary risk factors, including:

  • smoking
  • being exposed to UV light
  • having diabetes
  • being assigned female at birth
  • being over 40 years old

Other frequently asked questions

These are a few other questions people commonly ask about Fuchs’ dystrophy. Dr. Leela Raju has reviewed the answers.

Is Fuchs’ dystrophy a disability?

Fuchs’ dystrophy may or may not be a disability, depending on the severity of the symptoms. In the early stages, many people may be asymptomatic or may only experience mild vision impairment. As the condition progresses, worsening vision and pain can occur.

Is Fuchs’ dystrophy common?

Experts estimate that around 4% of people ages 40 and up develop Fuchs’ dystrophy. It is rare for people under 40 to develop the condition.

Can you cure Fuchs’ dystrophy?

There is currently no known cure for Fuchs’ dystrophy. However, there are treatments available to relieve the symptoms. People who undergo surgery for the condition typically experience a noticeable improvement in their vision.

How fast does Fuchs’ dystrophy progress?

Fuchs’ dystrophy progresses slowly, and symptoms may appear over several decades. Many people do not require surgical correction until ages 60–70.

Summary

Fuchs’ dystrophy is a progressive eye condition affecting your vision and light sensitivity. While there is no cure, treatment can help relieve the symptoms.

Many people who undergo surgery to correct Fuchs’ dystrophy experience significant vision improvement. Your doctor will determine the appropriate procedure based on the condition’s progression.

Contact your doctor if you are experiencing visual impairment or pain.

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Medical Reviewer: Leela Raju, MD
Last Review Date: 2022 Oct 7
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THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
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  3. Fuchs’ endothelial dystrophy - Europe. (2015). https://www.aao.org/topic-detail/fuchs-endothelial-dystrophy-europe
  4. Moshirfar, M., et al. (2022). Fuchs endothelial dystrophy. https://www.ncbi.nlm.nih.gov/books/NBK545248/