Introduction  

Fuchs' dystrophy refers to the death of cells in the endothelium (one of the layers in the cornea). The cells function to keep the cornea clear by pumping fluid from the cornea. Fluid builds up in the cornea when these cells die. The cornea then gets puffy (corneal edema) leading to a hazy or cloudy vision. 
This slowly progressing disease can also lead to glare and discomfort. It affects both eyes and can cause a deterioration of vision. Fuchs’ dystrophy can cause changes in the cornea’s shape leading to more vision problems.
Fuchs' dystrophy can occur in two stages. In the first stage, an individual experiences a cloudy vision in the morning. The vision, however, improves as the day progresses. The reason is that the eye is ordinarily moist when it is closed during sleep but the fluid dries when awake. 
In the second stage, the individual has blurry vision throughout the day. Excess fluid builds up during sleep but doesn’t dry up well during the day. During this stage, small blisters may form in the cornea. These blisters cause pain when they enlarge and break open.

Also Known As

  • Fuchs' endothelial dystrophy
  • Fuchs' corneal dystrophy
     

Sub-Types

 

Causes and Risk Factors

Fuchs’ dystrophy occurs when the endothelial cells gradually die which results in edema within the cornea. The disease can be inherited. However, it can also occur in individuals without a known family history of Fuchs’ dystrophy. 
The following factors can increase the risk of developing Fuchs’ dystrophy:

  • Age - Those in their 30s and 40s even though they may not be aware of it. Problems with vision might start showing when they reach age 50 and above 
  • Gender - The disease affects more women than men
  • Genetics – A family history of the disease
  • Other diseases such as diabetes 
  • Habits such as smoking
     

Signs & Symptoms

The symptoms of Fuchs’ dystrophy can be observed in the two stages. They may include:

  • Pain in the eye if one has tiny blisters on the cornea
  • Problems in the eye that get worse in humid conditions
  • Sandy or gritty feeling in the eye
  • Extra sensitivity to bright light
  • Very blurry vision if the center of the cornea is scarred
  • Glare which can decrease vision in dim and bright light
  • Difficulty seeing at night
  • Distorted vision
  • Seeing colored halos around light
     

Diagnosis

The eye care professional will conduct a thorough eye examination. He/she may do the following:

  • A pachymetry test which measures the thickness of the cornea
  • Staging where he/she checks for guttae (irregular bumps) on the cornea using a slit lamp (microscopic instrument). This test will determine whether it is stage 1 or 2
  • Count the cells in the endothelium by use of a special device to record the size, shape and number of the corneal cells
  • Take a special photograph of the cornea
  • Conduct a corneal pressure test using a special instrument
     

Treatment 

Treatment of Fuchs’ dystrophy is directed towards clearing the swelling in the cornea.

Medical Treatment

Treatment depends on how the disease has affected the cells in the eye. For stage 1 Fuchs’ dystrophy, the patient can take eye drops or ointment to reduce the swelling in the eye. 
The patient can also use ‘bandage’ contact lenses which may relieve eye pain.

Surgical Treatment

For stage 2 Fuchs’ dystrophy that causes poor vision or where the cornea is scarred, a corneal transplant is necessary. Descemet’s stripping endothelial keratoplasty (DSEK) or Descemet’s stripping automated endothelial keratoplasty (DSAEK) is a surgical procedure where a patient’s cornea receives healthy endothelial cells. 

The surgeon can also do a full corneal transplant (penetrating keratoplasty) in which a patient is given a healthy cornea from a donor.

Home Care

The patient can use a hair dryer and while holding it at arm’s length, direct warm air to his/her face. The warm air may help to dry out the surface of the cornea.

Prognosis/Long-term outlook

Surgery is the only cure for Fuchs’ dystrophy. DSEK is preferred to penetrating keratoplasty because recovery is quick with fewer complications. This is because there are no stitches involved.

Fuchs’ dystrophy gets worse with time. Without a transplant, it can lead to blindness or reduced vision with pain.

Fuchs' dystrophy can worsen after cataract surgery. The surgeon may need to evaluate the risks involved.

Complications from surgery include infection, rejection and poor wound healing. Using steroids to prevent rejection of the donor tissue can in turn cause glaucoma and cataract.

Patients with mild Fuchs’ dystrophy need a medical follow-up at least once a year. Those with a severe form of the disease, however, need a  follow-up every four months. 

Patients using ‘bandage’ contact lenses need more monitoring due to the risk of infection. 
Surgical cases need follow-up in the first few weeks after surgery and subsequent follow-ups due to the risk of rejection.

Prevention/Follow Up

There is no known prevention.