Fuchs’ dystrophy is an eye condition that causes the death of cells in the inner lining of the cornea. This may lead to the accumulation of fluid in the cornea and negatively affect vision.

One of the first symptoms people may experience is glare from bright sunlight or car headlights at night. Later, people with this condition may develop blurry vision and a gritty feeling in the eye.

It’s possible that genetics play a role. In fact, most individuals with Fuchs’ dystrophy have a family history of the condition. Treatment in the early stage involves saline eye drops comprising 2–5% salt to dry out the cornea. For comparison, typical saline solutions, such as contact solutions, only contain 0.9% salt.

Late stage treatment typically involves surgical replacement of part of the cornea.

Keep reading to learn more about the symptoms, causes, diagnosis, treatment, complications, and home care for Fuchs’ dystrophy.

A cross-section diagram of the eye showing where the cornea is.Share on Pinterest
Illustrated by Jason Hoffman

Fuchs’ dystrophy is a condition that affects the cornea, which is the transparent outer layer of the eye that covers the pupil and the iris. It causes the gradual death of cells in the corneal endothelium, the layer of cells on the cornea’s inner surface.

In a healthy eye, the endothelium pumps fluid away from the cornea to keep it clear. When the endothelial cells die, the fluid accumulates, which results in swelling of the cornea. As a result, vision becomes cloudy.

Fuchs’ dystrophy also causes bumps, known as guttata, to form on the endothelium. As the condition progresses, an increasing number of guttata form in the center of the cornea, which also impairs vision.

Once endothelial cells die, they do not regenerate. As such, this dystrophy is not reversible. The condition can affect people in their 30s and 40s, but vision problems may not become apparent until age 50 or later.

The symptoms of Fuchs’ dystrophy differ between the early and late stages.

Early stage

In the early stage of Fuchs’ dystrophy, people may notice few or no symptoms. One of the first symptoms that they may experience is glare from bright sunlight or headlights at night.

As the condition advances, a person may have foggy vision immediately after waking, which clears with time. This is because fluid can only evaporate from the cornea when the eyes are open. When a person sleeps, the fluid accumulates on the cornea again.

Late stage

In the late stage of Fuchs’ dystrophy, the vision stays blurry all day without clearing. Blisters may also form and cause pain. Other symptoms include:

  • extra sensitivity to light
  • gritty or sandy feeling in the eye
  • symptoms that get worse in humid conditions

Most people with Fuchs’ dystrophy have a family history of the condition. Researchers have identified one gene variation that links to the early stage of Fuchs’ dystrophy. Similarly, they have found several variations that link to the late stage. However, research on how genetics influence the condition is still ongoing.

Fuchs’ dystrophy is more common in females and people aged 40 or over. Risk factors that affect severity include:

  • exposure to UV light
  • smoking
  • diabetes

A rare form of Fuchs’ dystrophy that begins earlier in life affects males and females equally.

To diagnose this condition, doctors will ask about a person’s symptoms and medical or family history. Doing so can help determine if eye conditions run in the family.

The doctor may then carry out a microscopic examination. This exam can determine if there is a loss of endothelial cells and a thickening of the cornea.

Optical coherence tomography can detect guttata in the eye. However, this does not necessarily prove a person has Fuchs’ dystrophy. People can have guttata without having Fuchs’ dystrophy, so a doctor will also look for swelling in the cornea.

Treatment will depend on whether the condition is in the early or late stage.

Early stage

Treatment for early stage Fuchs’ dystrophy involves drying the cornea. A doctor may recommend 2–5% saline drops or ointment to help pull water out of the cornea. The eye drop that doctors most commonly recommend is Muro 128.

It may also help to gently blow warm air onto the face by holding a hair dryer at arm’s length.

Late stage

Treatment for late stage Fuchs’ dystrophy requires a cornea transplant to improve vision. To understand the surgical options, it helps to be familiar with the anatomy of the cornea. It consists of three layers:

  • the epithelium, or outer layer
  • the stroma, or middle layer
  • the endothelium, or inner layer

The surgeries that may help improve the symptoms of Fuchs’ dystrophy include:

  • Penetrating keratoplasty (PK): This involves removing all three layers of the cornea and replacing them with a healthy donor cornea. PK was the first surgery doctors developed for Fuchs’ dystrophy. However, it is no longer the standard because of its potential complications.
  • Descemet’s stripping automated endothelial keratoplasty (DSAEK): This replaces the endothelium and stroma layers with part of a donor cornea. DSAEK offers advantages over PK, and the recovery time varies from 3–6 months.
  • Descemet membrane endothelial keratoplasty (DMEK): This replaces only the endothelial layer with part of a donor cornea. DMEK is the most advanced procedure, and the recovery time ranges from 1.5–3 months.

Over time, Fuchs’ dystrophy can cause scarring and reduced visual acuity, meaning a person cannot see things clearly. Once these occur, the only treatment that can help is surgery.

Corneal transplants have their own risks, although the procedures have become safer and more advanced. Some people may experience:

  • infection
  • poor wound healing
  • graft detachment, rejection, and failure
  • leakage from the cornea
  • high blood pressure in the eye due to steroid medication
  • cataract formation
  • problems that affect the retina, which is the light-sensitive structure in the back of the eye
  • problems with the vitreous, which is the gel-like fluid in the eye

Doctors can generally treat graft detachments by using air or sulfur hexafluoride to reattach the graft.

Fuchs’ dystrophy advances slowly, so many people do not manifest symptoms for years. During this time, they may not need any specific self-care to manage the condition.

However, certain actions can prevent the symptoms from worsening and generally promote eye health. These include:

  • stopping smoking
  • wearing sunglasses to protect the eyes from UV light
  • taking steps to lower the risk of type 2 diabetes

Later, when symptoms develop, a person may receive relief from using saline eye drops. A person can also dry their eyes using gentle heat, such as from a hair dryer on the lowest setting.

Driving

If a person experiences any vision changes, they need to consider how this may affect daily activities, such as driving.

Ask about the vision requirements for keeping a driver’s license. If a person’s vision does not meet the threshold required for driving, they might keep their driver’s license with a restriction, such as a limitation to daytime driving.

Work and reading

As vision changes take place, people can use assistive devices to help them continue reading and working. These may include:

  • large-print books
  • visual aids, such as magnifiers
  • screen readers

People can also use these tools at work. Employers are required to make reasonable adjustments to the workplace that allow people with visual impairments to continue in their job.

Other adjustments that may help include:

  • adjusting the lighting in the workplace to reduce glare
  • changing the position of a person’s workstation
  • providing software that helps a person use their computer

Fuchs’ dystrophy is a condition that causes cells in the lining of the cornea to die. It also increases the formation of guttata, or bumps, in the cornea. Both of these can reduce visual acuity, or how well a person can see.

In the early stage of Fuchs’ dystrophy, a person may experience few or no symptoms. However, as the condition progresses, it can cause pain, a gritty feeling in the eye, and blurred vision.

Early treatment may involve nonsurgical interventions, such as saline eye drops. However, the late stage requires surgery to replace the cornea.