The cornea is the transparent, dome-shaped structure of the eye that covers the iris and pupil. It can be thought of as the front-most window into the eye. In order to have sharp vision, the cornea needs to be clear, have a smooth surface, and be round in shape. Conditions that affect any of these properties will lead to blurry vision.
The cornea has several layers, as shown in the above diagram. The innermost layer, known as the endothelium, is a layer of cells which normally function to pump fluid out of the cornea to keep it clear. These endothelium cells naturally die off as we age. In Fuchs’ dystrophy, these cells die off at a faster-than-normal rate. If too many endothelial cells are lost, the cornea will not be able to keep up with pumping fluid out and will become swollen and cloudy.
Although there is a very rare form of Fuchs’ that presents in early adulthood, most cases start when a patient is in their 30’s to 40’s but don’t cause symptoms until many years – often decades – later. Fuchs’ dystrophy can be hereditary and we recommend family members of affected individuals be screened.
Most people who have Fuchs’ dystrophy never have any symptoms because their disease is mild throughout life. The earliest symptom is blurry vision upon awakening which improves throughout the day as the cornea dries out while the eyes are open. As the condition worsens, the blurriness may not improve over the course of the day. Patients may have glare especially while out in the bright sun and while driving at night. The cornea may become so swollen with fluid that it actually develops blisters on the surface which can rupture and be quite painful.
The diagnosis of Fuchs' is usually made when your eye doctor performs a standard microscopic examination of your eye. At InVision, we utilize additional testing such as computer-automated counters of corneal endothelial cells as well as corneal thickness scanning to assess the severity of Fuchs', which helps us decide on the need for intervention.
Fortunately most cases Fuchs' dystrophy do not require urgent intervention.
Early in the disease, treatment is often not necessary. There are several non-surgical treatments to manage the symptoms of Fuchs' as it becomes more advanced. Blue-light-blocking lenses or polarized lenses may reduce glare symptoms. A special eye medication may help draw fluid out of the cornea to temporarily allow for clearer vision. A soft bandage contact lens may provide temporary relief from corneal blisters, should they develop.
The only current solution for Fuchs’ dystrophy is to replace the diseased corneal endothelial cells with healthy ones. This is done surgically by transplanting layers of healthy corneal endothelial cells from human (deceased) donors into a patient (recipient's) eye. These partial corneal transplant surgeries, known by their acronyms DMEK and DSEK, are performed in an outpatient setting in less than an hour under sedation. Following these surgeries, most patients successfully experience improvement in their symptoms and vision in a matter of months or even weeks.
Should you need a corneal transplant surgery, Dr. Ragam can perform it at our state-of-the art ambulatory surgery center, Seashore Surgical Institute.