Fuchs’ Dystrophy (corneal disease)

What is Fuchs’ Dystrophy?

Fuch’s dystrophy is also known as Fuch’s corneal endothelial dystrophy or FCED and is a slowly progressing corneal dystrophy that commonly affects both eyes and is a bit more common in women than men. Fuch’s dystrophy reduces the number of endothelial cells that make up the inner layer of the cornea and causes fluid buildup that makes the corneal tissue become thick and the cornea to become cloudy and swollen.

The role of the endothelial cells is to pump extra fluid from the cornea. Ernst Fuchs was an Austrian ophthalmologist who first described the condition in 1910. Fuch’s dystrophy can be inherited or occur in individuals with no family history of the condition.

What are the Symptoms of Fuchs’ Dystrophy?

Early signs of Fuchs’ dystrophy can include glare and an inability to see clearly in low light. Blurred vision may be experienced early in the morning or after waking up. Vision normally improves throughout the day until the condition progresses enough to permanently affect vision.

Symptoms include

  • Sensitivity to light
  • Difficulty seeing at night
  • Visibly cloudy corneas
  • Distorted vision

Fuchs’ Dystrophy¬†Causes

Normally, endothelial cells maintain a normal balance of fluid within the cornea to keep it from swelling. In Fuchs’ dystrophy, these cells slowly die, causing fluid to build up within the cornea. This can cause corneal thickening and blurred vision. In advanced cases, the condition can cause painful blisters to form on the surface of the cornea. Fuchs’ dystrophy is a progressive disease that usually takes years to appear and typically affects adults later in life. It can be an inherited condition, but tends to be more prevalent in women. Early onset Fuchs’ dystrophy can appear in children but it more commonly begins in a patient’s 20s or 30s, with symptoms appearing once an adult is in their 50s or 60s.

How is Fuchs’ Dystrophy Treated?

Diagnosis

Endothelial cells in the cornea prevent the build-up for fluid and keep the eyes’ windows clear. When less of these cells are produced, fluid accumulates enough to thicken tissues and reduce vision. It’s not known why some people develop the condition.

Since there could be multiple reasons for any changes in vision, diagnosis starts with an eye exam, to check:

  • Swelling around one or both corneas
  • Thickened corneas (measured with ultrasound)
  • Irregular bumps or guttae (on the back of the cornea)

Treatment includes

Medication often helps with mild forms of Fuchs’ dystrophy. If the condition isn’t detected until an advanced stage, a cornea transplant may be necessary to restore clear vision.

Fuchs’ dystrophy tends to develop later in life and may be confused with symptoms of cataracts due to the similarities with how the two conditions affect vision. It’s slightly more common in women, although anyone at any age may develop the condition. Any time vision is affected for no apparent reason, it’s time to see an eye doctor.

Fuchs’ Dystrophy¬†Prevention

The best prevention for Fuchs’ dystrophy is early diagnosis which allows for effective treatment methods to be prescribed. Regular vision exams are the best form of early diagnosis and can include a corneal pressure test, measuring corneal thickness, or performing a corneal cell count. Depending on how early the disease is diagnosed, various options can be prescribed, ranging from medications to surgery. If caught early, physicians may prescribe eye medications such as ointments or eye drops to help reduce excess fluid in the cornea. Sometimes soft contact lenses are recommended to help cover the cornea to reduce pain sensitivity.

In advanced cases of Fuchs’ dystrophy, surgery is recommended to correct the affected cornea. Surgical options can include the replacement of the inner layer of cornea, also known as an endothelial keratoplasty. In this situation, a local anesthetic is used and the procedure is usually performed at an outpatient facility. Although less commonly used, doctors may opt to perform a complete corneal transplant which requires sourcing a healthy cornea from a donor.