Fuchs’ dystrophy is caused by the loss of corneal endothelial cells. The cornea is the clear window on the front of the eye, and the endothelial cells are on the back surface of the cornea. Endothelial cells help keep the cornea clear by pumping out excess fluid. 

“We’re born with a given number of endothelial cells and gradually lose those cells over time as natural part of the aging process,” explains ophthalmologist Brian Armstrong, MD. “In Fuchs' dystrophy, these cells die off more rapidly than normal, leading to fluid buildup and corneal swelling.” 

It’s believed that genetic mutations in the corneal endothelial cells or surrounding tissue may contribute to their premature death, resulting in Fuchs’ dystrophy.

When too many cells die, and the endothelial cell density reaches a certain minimal threshold, fluid can build up in the cornea. The cornea thickens and becomes cloudy, causing vision to become blurry – like looking through a foggy bathroom mirror. 

“Fuchs' dystrophy is more common in women and tends to run in families,” says Dr. Armstrong. “If either of your parents has this condition, you have a 50% chance of developing it too.” 

Other risk factors for developing the disease include: 

• Age (symptoms usually appear later in life when a person reaches their 50s or 60s) 
• European descent 
• Diabetes (a possible risk factor)

While there's no way to prevent Fuchs' dystrophy, you can manage certain risk factors by: 

• Avoiding smoking.
• Eating a well-balanced diet with lots of leafy greens and colorful vegetables. 
• Protecting your eyes from UV light by wearing sunglasses.
• Getting regular eye check-ups.

What are the symptoms of Fuchs’ dystrophy?

In the early stages of Fuchs’ dystrophy, people often have no symptoms. As the disease progresses, people may notice blurry vision, especially in the morning. If the condition progresses, blurry vision may continue later into the day. A person may see glare and halos around lights. In later stages, a person may feel irritation in their eye or feel pain caused by blisters in the cornea. 

Onset of Fuchs’ dystrophy can vary, but symptoms typically become noticeable when a person reaches their 50s or 60s. In some patients, the condition remains mild, and symptoms never develop. Sometimes, routine eye surgery, like cataract surgery, can cause the disease to progress.

How is Fuchs’ dystrophy treated?

An eye doctor, usually an ophthalmologist or optometrist, diagnoses Fuchs' dystrophy through a comprehensive eye exam. They may use a slit lamp to look for specific changes in the cornea, measure corneal thickness, and count the endothelial cells via specialized imaging. 

Early treatments include saline eye drops or ointments to reduce corneal swelling. The salt in these saline drops can draw excess fluid out of the cornea.

“You should see a corneal specialist if you experience significant vision changes or discomfort,” says Dr. Armstrong. “Surgery is usually considered when vision is severely affected, and other treatments are no longer effective.” 

About 15% of people with Fuchs' dystrophy may eventually need surgery. Surgical options include:

• Descemet membrane endothelial keratoplasty: DMEK is a partial-thickness cornea transplant that replaces only the inner layer of the cornea with a thin sheet of healthy donor cells.
• Descemet stripping endothelial keratoplasty: DSEK is a partial-thickness cornea transplant that replaces a thicker layer of tissue than DMEK.
• Penetrating keratoplasty: This is a full-thickness cornea transplant used in more severe cases.

There are also newer, less invasive surgical procedures being developed and awaiting FDA approval.