Fuchs’ Dystrophy: causes, symptoms, and treatment options

What is Fuchs’ Dystrophy?

Fuchs’ endothelial dystrophy is a degenerative disease affecting the corneal endothelial cells, which form a single layer on the back surface of the cornea. These cells are crucial for pumping excess fluid out of the cornea to keep it clear. When these cells become damaged or dysfunctional, the cornea begins to swell with fluid, leading to visual disturbances and a gradual loss of vision.

Unlike other corneal conditions, the endothelial cells in Fuchs' dystrophy do not regenerate. As a result, the cornea becomes progressively cloudier, which can severely affect vision over time.

What are the symptoms?

The symptoms of Fuchs’ dystrophy often develop slowly and may initially be mild. Typical symptoms include:

• Foggy vision: Vision may appear hazy or blurred, especially in the morning when you first wake up. This fogginess often improves as the day progresses.
• Glare and halos: Bright lights can cause glare and halos around them, making it difficult to see clearly, particularly at night.
• Visual improvement by evening: While early symptoms can be managed, the vision may improve towards the end of the day. In advanced cases, however, this may not occur.

Individuals in their fifties or older are more likely to experience noticeable symptoms, although early signs can be detected earlier by an eye specialist.

What causes Fuchs’ Dystrophy?

Fuchs’ dystrophy is primarily caused by genetic mutations that affect the endothelial cells. Over time, these cells deteriorate faster than they should, leading to a reduction in their number and function. The condition can occur with no family history or may be inherited in some cases.

Normal ageing results in a gradual loss of endothelial cells, but in Fuchs’ dystrophy, this loss is accelerated. A healthy individual may have 1500-2500 endothelial cells per square millimetre by age 80, whereas a person with Fuchs’ dystrophy might have fewer than 500 cells by age 50. Abnormal proteins and small bumps, known as guttata, accumulate on the back of the cornea, further disrupting vision.

What are the treatment options?

Medical Treatment

In the early stages, treatment may involve using sodium chloride (salt) drops or ointments to draw excess fluid out of the cornea through osmosis. While this approach can delay the need for surgery, it does not cure Fuchs’ dystrophy.

Surgical Treatment

1. DSAEK (Descemet’s Stripping Automated Endothelial Keratoplasty): This procedure involves replacing the damaged endothelial layer with a thin layer of donor tissue. Performed through small incisions, DSAEK does not require stitches and involves positioning an air bubble to keep the graft in place. It is generally performed under local or general anaesthesia.
2. DMEK (Descemet’s Membrane Endothelial Keratoplasty): DMEK involves transplanting a sheet of pure endothelial cells, which is thinner and results in faster visual recovery compared to DSAEK. It also has a lower rejection rate. This procedure is highly specialised and may not be suitable for all individuals.

Cataract surgery and Fuchs’ Dystrophy

Routine cataract surgery can sometimes accelerate corneal failure in individuals with Fuchs’ dystrophy. Careful surgical techniques, such as the soft shell technique or femtosecond laser cataract surgery, can minimise endothelial cell damage. In more advanced cases, cataract surgery may be combined with endothelial corneal transplantation.

Why choose us for your treatment?

At OCL Vision, we specialise in advanced corneal transplantation techniques, including DSAEK and DMEK. Our team of expert surgeons performs over 5% of all corneal transplants in England, with extensive experience in handling complex cases.

To learn more about our surgical procedures and how they can help you, please explore our dedicated pages on corneal transplants.