Fuchs' Dystrophy

What is Fuchs' dystrophy?

Fuchs' dystrophy is one type of corneal dystrophy - a group of inherited disorders that affect the cornea, which is the clear front surface of the eye. In Fuchs' (pronounced fewks) dystrophy the innermost layer of the cornea lets too much water in, so the cornea becomes swollen and cloudy.

It is a fairly common cause of blindness worldwide and in the UK, often leads to corneal transplant surgery. It is classed as a posterior corneal dystrophy, which means it affects the innermost layer of the cornea. 

What causes Fuchs' dystrophy?

The cornea has five different layers, and Fuchs' dystrophy affects the inner layer (called the endothelium). In a healthy eye the endothelium prevents fluid from entering into the cornea from the middle layer of the eye, but in Fuchs dystrophy, the cornea takes in fluid and swells.

This makes the cornea become less clear, which affects vision. Fuchs' dystrophy can be caused by an inherited genetic fault but may also happen in people with no family history of the condition.

It is more common in women and in people over 40. 

What are the signs and symptoms of Fuchs' dystrophy?

Fuchs' dystrophy tends to affect both eyes at the same time, causing vision to become misty or cloudy. Sight loss happens gradually and tends to be worse first thing in the morning, and as the condition progresses symptoms can include pain and eventually serious sight loss.

Is Fuchs' dystrophy serious?

Fuchs' dystrophy doesn't always cause problems, but it can cause pain or discomfort and can lead to progressive sight loss, or may require surgery such as corneal transplant. Unfortunately, there is currently no cure for the underlying cause of the condition.

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How is Fuchs' dystrophy diagnosed?

Corneal dystrophies are usually diagnosed using a slit light microscope to shine a thin, bright light into the eye, enabling the ophthalmologist to study the front part of the eye. They may also ask about family history and may recommend genetic testing to identify the gene responsible for the condition.

What are the treatments for Fuchs' dystrophy?

Fuchs' corneal dystrophy may need transplant surgery to remove the damaged cornea or corneas and replace them with a healthy one from a donor. In some cases this can be done as keyhole surgery to replace just the faulty endothelial layer. 

What can help Fuchs' dystrophy?

Managing symptoms through prescribed treatments, regular monitoring, and in severe cases surgery, can help. Genetic counselling can provide guidance for families with a history of Fuchs' dystrophy.

What research is there into Fuchs' dystrophy?

Research on Fuchs' dystrophy aims to learn more about how and why the cornea’s inner layer stops working and the links to genetic and environmental factors. Ultimately it may one day be possible to develop a gene therapy to replace the faulty genes.

One of our research projects at University College London is looking at genetic data in people with Fuchs' dystrophy, to understand how many people with a certain genetic fault develop the condition and how it changes as it is passed from parent to child. 

This research could ultimately lead to the development of new treatments – such as innovative gene therapies – which could help prevent or slow down sight loss in this condition. It could also lead to more accurate genetic counselling for families affected by Fuchs' dystrophy – helping them to better plan for the future.