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FUCH’S CORNEAL ENDOTHELIAL DYSTROPHY (FUCHS)
The cornea is the window in the front of the eye. It covers the iris (colored part of the eye) and the pupil (black hole in the iris). The cornea focuses light as it enters 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.
Layers of the cornea
Source: American Academy of Ophthalmology ®
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 generally made on a routine eye exam, usually before the patient is symptomatic from it. On exam your eye doctor will see little dots, called guttata, on the back of the cornea which is a sign that the endothelial cells are dying off. At InVision we utilize computer-automated counters of corneal endothelial cells to give us a more accurate evaluation of how advanced the Fuchs’ is. We also are one of the few practices in New Jersey to have a specialized device from Spain called the HD Analyzer that uses scanning lasers to help us quantify the amount of glare someone is experiencing. The results from these tests may influence our decision on when to surgically intervene for Fuchs’ dystrophy.
Fortunately for most people with Fuchs’ it is generally not an emergency situation and the course of treatment may be planned over a convenient time period. Early in the disease no treatment is necessary.
There are several non-surgical treatments to manage the symptoms of Fuchs’ dystrophy as it becomes more advanced. Blue-light-blocking lenses or polarized lenses may reduce glare symptoms. A special eye ointment before bedtime may help draw fluid out of the cornea overnight to allow for clear vision in the morning. Similarly, the use the use of a blow dryer in a low setting at arm’s length in the morning may help clear vision. This should only be done under the direction of your doctor. A soft bandage contact lens may provide temporary relief from corneal blisters, should they develop.
However, the only current solution for Fuchs’ dystrophy is to replace the sick corneal endothelial cells with healthy ones. This is done by surgical procedures called DSEK and DMEK which involve transplanting human donor endothelial cells onto the back of the cornea. After these procedures, most patients successfully experience improvement in their symptoms and vision in a matter of weeks. It is an extraordinary surgical advance over what was available a few years ago, when corneal transplant surgery routinely required up to one year to recover from.
There are several procedures currently under investigation that hold the promise in the future of not requiring human donor tissue.
Should you need DSEK or DMEK surgery, it will be performed at our state-of-the art ambulatory surgery center, Seashore Surgical Institute.
Dr. Ragam is experienced in all facets of corneal surgery and has published research on corneal disease. We are fortunate on the Jersey shore to have such a talented and caring corneal specialist. We welcome Dr. Ragam to our multispecialty practice.