Fuchs’ Corneal Dystrophy

What is Fuchs’ Dystrophy?

This is an inherited, progressive disease of the cornea. Specifically, Fuchs’ Dystrophy affects the posterior layer of the cornea known as the endothelial cell layer. The endothelial cells contain pumps that help remove fluid from the cornea, keeping it optically clear. Fuchs’ Dystrophy causes death and decline of these water pumping cells, resulting in the loss of transparency of the cornea from swelling. Also seen in Fuchs’ Dystrophy are anvil shaped outgrowths called guttata in the layer just underneath the endothelial cells layer. Fuchs’ dystrophy usually occurs after age 40.

What are the symptoms of Fuchs’ Dystrophy?

Besides cloudy vision, a patient with Fuchs’ usually progresses through two stages:

Stage One: Involves swelling of the cornea while sleeping overnight that gradually clears as the day goes on. As a result, vision is worse in the morning. Hypertonic saline eye drops are usually helpful during this stage to control symptoms.

Stage Two: The swelling progresses and vision becomes cloudy until later in the day. With this, the patient may have sensitivity to light and see a variety of visual phenomena like haloes and starbusts when looking at light. Humid climates tend to make this condition even worse. As the disease progresses, the center of the cornea can become scarred, further reducing vision.

How is Fuchs’ Dystrophy Diagnosed?

Using a special microsope (i.e. slitlamp), an ophthalmologist can detect subtle changes in the cornea. Special instruments, such as a pachymeter, may be used to measure the thickness of the cornea to detect and monitor swelling. A device known as a specular microscope can provide an image of the endothlelial cells to detect the extent of cell loss as well their characteristics. All of these diagnostic devices are available at OCC.

How is Fuchs’ Treated?

Fortunately, many patients will never need a corneal transplant for this condition. In fact, a fair percentage of individuals have this condition and remain asymptomatic. As surgery can cause endothelial cell loss, it is important to determine if Fuchs’ Dystrophy exists when planning other intraocular procedures, such as cataract surgery. In cases where there is already extensive cell loss, it may be prudent to do a combined procedure (i.e cataract surgery with a corneal transplant) if your ophthalmologist feels the cornea will be too compromised after just a single surgery. If saline eye drops are no longer an option to maintain good vision with Fuchs’, a corneal transplant is usually required. The preferred technique is to perform a partial thickness transplant (Endothelial, or DSAEK, Keratoplasty) to remove just the diseased cells. However, in certain cases, this many not be possible and a full thickness transplant will be required. Your ophthalmologist will carefully outline your treatment options in order to maximize your visual potential.