Fuchs' dystrophy

An inherited condition affecting the cornea.

What is Fuchs’ Endothelial Corneal Dystrophy (FECD)?

Fuchs’ Endothelial Corneal Dystrophy (or simply, Fuchs’ dystrophy) is a disease of the cornea and affects both eyes. The cornea is the clear, dome-shaped window of the front of your eye, that allows light to enter the eye as well as focus the light.

The cornea has three main layers – the outer epithelial layer, the middle stromal layer, and the inner endothelial layer. The Descemet's membrane separates the stroma from the endothelial layer.

The cornea needs to remain clear to function properly, and it is the cells on the inner endothelial layer of the cornea which are responsible for keeping it clear. These endothelial cells act as a pump and remove fluid from the cornea keeping it clear.

Fuchs’ Endothelial Corneal Dystrophy (FECD) diagram

As you age, endothelial cells are naturally lost over time, without regenerating. In FECD, however, these cells are lost at a faster rate. You are therefore left with fewer endothelial cells. As a result, their ability to pump fluid out of the cornea is reduced and fluid accumulates within the cornea. The cornea then becomes swollen and cloudy, which causes blurry vision – much like looking through a foggy window.

What are the symptoms of Fuchs’ endothelial corneal dystrophy (FECD)?

FECD develops slowly and in the early stages you might not notice any symptoms. At this stage, it is often diagnosed on a routine eye test as an incidental finding.

Early symptoms include mild blurring of vision, particularly when waking up first thing in the morning, which improves throughout the day.

As the condition progresses, the blurring of vision becomes worse, remains throughout the day, and does not improve as the day goes on.

The extra fluid in your cornea causes your eyes to be more sensitive to light (glare), and this can be particularly bothersome at night, for example when driving.

In more advanced cases, tiny blisters may form on the surface of the cornea. These blisters may break open causing eye pain and eventually corneal scarring.

What causes Fuchs’ dystrophy (FECD)?

FECD is usually inherited, which means it can be passed down from parents to children. If either of your parents has the disease, you have a 50% chance of developing the condition - but family members can be affected to varying degrees. However, the condition may also occur in people without a known family history of the disease.

FECD is more common in women than men. Whilst signs of FECD may be visible in people in their 30s and 40s, symptoms do not usually develop until their 50s or later. The actual reason why the corneal endothelial cells die at a faster rate is unknown.

FECD frequently asked questions

To help provide more information on Fuchs’ Endothelial Corneal Dystrophy (FECD), we have a list of our frequently asked questions. If you cannot find the answer to your question here, please contact our eye care experts who will be delighted to help you.

Do I need to have surgery?

If your symptoms are mild and your vision is unaffected, you do not need to have surgery. However, if you are having difficulty with your vision, despite treatment with eye drops, surgery may be an option for you.

Learn more about Fuchs' dystrophy treatments.

 

Is there a link between cataracts and Fuchs’ dystrophy?

Fuchs’ dystrophy and cataract are both conditions which affect your vision as you get older.

They are separate conditions and are not linked to each other. However, if you were to have cataract surgery to remove your cataracts, there is a chance that the Fuchs' dystrophy worsens. 

This is because the cataract surgery itself can accelerate the loss of the endothelial cells.

If you have mild Fuchs’ dystrophy and a cataract which is affecting your vision, your doctor might suggest removing the cataract first with cataract surgery. If the cornea remains cloudy after the surgery (due to worsening of the Fuchs’ dystrophy), then a corneal transplant may be needed. Usually, however, in mild Fuchs’ cases, the cornea usually clears without needing a corneal transplant.

For advanced cases of Fuchs’ dystrophy with cataracts, your doctor will plan for both cataract surgery and a corneal transplant to be done either on the same day or on different days.

 

Can Fuchs’ dystrophy come back after surgery?

Fuchs’ dystrophy cannot return in the newly-transplanted corneal transplant. Whilst there is no cure for Fuchs’ dystrophy, treatment in the form of corneal transplantation is very effective and has a high success rate. However, a repeat transplant may be required in certain circumstances. 

 

Treatment for  Fuchs’ Endothelial Corneal Dystrophy (FECD)

The treatment of FECD depends on your symptoms.

No symptoms:

  • No treatment or follow up is required.

Mild symptoms:

  • Eye drops (sodium chloride 5%) may be helpful. These eye drops work by drawing fluid out of the cornea, thereby reducing the swelling.

Moderate/severe symptoms:

As the condition progresses, the cornea becomes more swollen and a corneal transplant may be required.

The corneal transplant may be one of two types:

  • Endothelial Keratoplasty: where healthy endothelial cells from a donor are transplanted onto the back surface of your cornea.
  • Penetrating Keratoplasty: where the whole of your cornea is replaced with a healthy donor cornea.

Another surgical approach under investigation for FECD is a procedure called Descemet Stripping Only (DSO). DSO is the removal of the diseased area of the central cornea with no donor cells transplanted. Once the diseased area is removed from the centre of the cornea, the patient's own healthy endothelial cells move from the outer edges of the cornea to the centre of the cornea. DSO is not yet performed routinely but is currently under research.

How much does Fuchs’ dystrophy treatment cost?

Initial consultation

From £300

This includes an initial consultation and a visual acuity assessment.

If further outpatient tests and investigations are required, they will be charged at an additional rate. Your consultant will discuss this with you at your consultation.

Treatment

The cost of onward treatment will be provided after initial consultation,  based on your personalised treatment plan.

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