Fuchs' Dystrophy

What is Fuchs' dystrophy?

Fuchs' (pronounced fewks) dystrophy is an eye condition that affects the cornea – the clear front surface of the eye – leading to vision problems. It occurs when excess fluid builds up in the cornea, causing it to swell and thicken. This can result in blurred or cloudy vision.

In Fuchs’ dystrophy, the endothelium – the innermost layer of the cornea – gradually stops working. The endothelium plays an important role in maintaining the correct amount of fluid within the cornea, by pumping out excess fluid. When it fails, the cornea becomes waterlogged, leading to vision problems.

One of the first symptoms people may notice is blurred or hazy vision in the morning, which gradually improves throughout the day. But as their condition progresses, their vision may start to remain blurry all day long.

Fuchs’ dystrophy usually affects both eyes and typically develops when people are in their 40s and 50s. It’s more common in women, and also in people with a family history of the condition. 

Researchers estimate that more than 7 out of 100 adults worldwide are affected by Fuchs’ dystrophy, which is expected to rise as the population ages.    

Other names for this corneal condition are Fuchs’ corneal dystrophy and Fuchs’ endothelial corneal dystrophy (FECD).

What causes Fuchs' dystrophy?

Fuchs’ dystrophy can sometimes be inherited, meaning it may be passed down from parents to their children. However, it can also develop in people with no known family history of the condition.  

Fuchs’ dystrophy affects the endothelium, the thin layer of cells lining the back of the cornea. In a healthy eye, endothelial cells act as a pump, continuously removing excess fluid to keep the cornea clear for sharp vision. However, in Fuchs’ dystrophy, these cells gradually start to die – leading to a build-up of fluid in the cornea, causing it to swell and thicken. This can cause blurred or cloudy vision, which usually worsens over time as more cells are lost.

While the earliest signs of the disease often start in a person’s 30s and 40s, many people don’t develop symptoms until their 50s or 60s. However, there’s a rare form of Fuchs’ dystrophy that starts in childhood. Some of these cases of this early-onset type are caused by faults in a gene called COL8A2, which provides the instructions for making a protein mainly found in the cornea. When the condition is caused by faults in this gene, it is inherited in an autosomal dominant pattern, which means a parent with the condition has a 50% chance of passing it to each of their children.

Certain risk factors can increase the risk of developing Fuchs’ dystrophy, or speed up its progression:

• Female sex – women are more likely to develop it than men.
• Genetics – a family history increases the likelihood of developing the condition.
• Smoking – exposure to cigarette smoke has been identified as a contributing factor.
• Ultraviolet (UV) light – regular exposure to UV light in sunlight or from artificial sources, such as sunbeds, may play a role.
• Diabetes – people with type 2 diabetes have a higher risk of developing the condition.

What are the signs and symptoms of Fuchs' dystrophy?

Fuchs' dystrophy often causes few, if any, noticeable symptoms at first. It’s unusual for any symptoms to appear before the age of 50.

One of the first signs of Fuchs’ dystrophy is blurred or cloudy vision in the morning, which slowly improves as the day goes on. This happens because the cornea naturally swells while the eyes are closed at night, trapping moisture inside. When the eyes open, the fluid begins to evaporate from the surface of the cornea, improving vision. However, as the condition progresses, people may find their vision takes longer to get better or it may remain blurry throughout the day.

As the disease progresses, additional symptoms may develop, including:

• Increased sensitivity to light – bright light may feel uncomfortable and may make it harder to see.
• Glare – which can reduce vision in dim or bright lighting.
• Seeing halos around lights – which may be more noticeable at night, especially when driving.
• Reduced contrast sensitivity – difficulty in distinguishing objects from their backgrounds, particularly when there isn’t much contrast between them.
• Eye discomfort or grittiness – caused by tiny blisters that can form on the surface of the cornea.
• Pain – if a blister bursts, it can cause sharp pain and irritation.

Is Fuchs' dystrophy serious?

For most people, Fuchs’ dystrophy progresses slowly and they may go for many years without any significant vision problems. However, as the disease progresses, vision problems can gradually deteriorate, making everyday activities – such as reading, watching TV or driving – increasingly challenging.

In the early stages of Fuchs’ dystrophy, certain medications and self-care measures can help manage mild symptoms. However, in advanced cases where vision loss becomes more severe, a corneal transplant is the most effective way to restore sight.

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How is Fuchs' linked to cataracts?

As both Fuchs’ dystrophy and cataracts become more common with age, some people may develop both of these eye conditions together.

Cataracts are a very common eye condition where the lens gradually becomes cloudy, leading to misty or blurred vision. They are typically treated with surgery to remove the cloudy lens and replace it with an artificial lens.

While cataract surgery is usually very successful, it can sometimes worsen Fuchs’ dystrophy by further reducing the number of healthy corneal endothelial cells. Because of this risk, a thorough cornea examination is necessary before this procedure is recommended.

If a person’s recovery after cataract surgery is slow or limited, or their Fuchs’ dystrophy worsens, a corneal transplant may be necessary. In some cases, an ophthalmologist may recommend a combined approach, performing both cataract surgery and a corneal transplant at the same time. 

How is Fuchs' dystrophy diagnosed?

A specialist eye doctor (ophthalmologist) will often begin by taking a medical history, asking questions about symptoms and performing a thorough eye examination.

They may also use various specialised tests to help diagnose Fuchs dystrophy, including:

• Slit lamp microscopy – a special eye microscope (a slit lamp) is used to examine the cornea for tiny blisters on its front surface and droplet-like bumps (called guttae) at its back surface.
• Confocal/specular microscopy – a light-based imaging method that provide a detailed picture of the corneal endothelium. It can measure the number, density and shape of the endothelial cells.
• Corneal pachymetry – a painless test that measures the thickness of the cornea to detect swelling.
• Corneal tomography – a specialised imaging tests that creates a detailed map of the corneal surface to assess its shape and any signs of swelling.

What are the treatments for Fuchs' dystrophy?

While there is currently no cure for Fuchs’ dystrophy, treatments are available that can help manage the condition. Treatment options for Fuchs’ dystrophy will depend on the severity of symptoms. If the condition isn’t affecting their sight or is causing only mild symptoms, they may not need any treatment.

If a person with Fuchs’ dystrophy is experiencing mild symptoms, they may be prescribed non-surgical treatments including:

• Ointments and eye drops – applying saline (5% sodium chloride) medications can help to remove excess fluid in the cornea in some cases. 
• Soft contact lenses – which act as a covering to help alleviate pain from blisters that develop on the cornea.
• Medications – to help alleviate pain caused by blisters on the cornea.

For people with more advanced stages of Fuchs’ dystrophy, where vision problems are interfering with their daily lives, an ophthalmologist may recommend surgery. The main surgical option is a corneal transplant, also known as a keratoplasty or a corneal graft, which replaces damaged corneal tissue with healthy donor tissue to restore vision. 

There are different types of corneal transplants, which can replace all or only selected layers of the cornea. For Fuchs’ dystrophy, the preferred approach is a partial-thickness corneal transplant (or endothelial keratoplasty), which replaces only the innermost layers of the cornea.

The most common surgical procedures used for Fuchs’ dystrophy are:

• Descemet membrane endothelial keratoplasty (or DMEK) – the surgeon replaces only the damaged inner layer of the cornea with endothelial cells from a healthy donor.
• Descemet-stripping endothelial keratoplasty or Descemet-stripping automated endothelial keratoplasty (or DSEK/DSAEK) – the surgeon strips aways the damaged endothelium and replaces it with a very fine layer of endothelium and some supporting tissues from the cornea of a healthy donor.

DMEK and DSAEK are both ‘keyhole’ surgeries that are typically performed under local anaesthesia in hospital, taking less than an hour. Surgeons remove and replace the damaged corneal tissue through a small incision. Stitches, if needed, are usually removed within two to four weeks after the procedure. Most people will notice an improvement in their vision in the first six months after surgery.

What can help Fuchs' dystrophy?

People living with Fuchs’ dystrophy can take several steps to help manage their condition, including:

• Regular eye examinations – routine check-ups will help monitor how the condition progresses over time.
• Managing symptoms – using saline drops or ointments can help remove excess fluid in the cornea, improving vision problems for some people.
• Tinted glasses – for those with light sensitivity, wearing tinted glasses can help limit glare by reducing the amount of light entering their eyes.
• Pain relief – prescribed medications can help ease pain caused by corneal blisters. Wearing soft contact lenses day and night can also act as a protective bandage, reducing discomfort and promoting healing.

What research is there into Fuchs' dystrophy?

Research into Fuchs' dystrophy aims to uncover why this eye condition develops and what drives its progression. Scientists are also exploring a range of potential new treatments – both non-surgical and surgical – that could transform how the disease is managed in the future.

At Fight for Sight, we fund cutting-edge research to advance understanding of Fuchs’ dystrophy and develop new treatment approaches. Some of our current and past projects include:

• Dr Ciro Chiappini at King’s College London is developing an innovative gene therapy for inherited forms of Fuchs’ dystrophy. His approach involves using a dissolvable membrane embedded with nanoneedles – tiny, painless needles that deliver treatment directly into endothelial cells without causing damage. This technique aims to correct the underlying genetic fault, offering a potential alternative to corneal transplant surgery.
• Dr Alice Davidson at UCL Institute of Ophthalmology led a study to improve understanding of the genetic factors behind corneal dystrophies, including Fuchs’ dystrophy. As part of this project, she developed a new method to analyse a specific gene change linked with the condition. Her research could ultimately mean that more people with Fuchs dystrophy can receive a precise genetic diagnosis, helping them plan for the future.