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Corneal Disease (cont.)

Part 2: Corneal Dystrophies

Lattice Dystrophy. Lattice dystrophy gets its name from an accumulation of amyloid deposits, or abnormal protein fibers, throughout the middle and anterior stroma. During an eye examination, the doctor sees these deposits in the stroma as clear, comma-shaped overlapping dots and branching filaments, creating a lattice effect. Over time, the lattice lines will grow opaque and involve more of the stroma. They will also gradually converge, giving the cornea a cloudiness that may also reduce vision.

In some people, these abnormal protein fibers can accumulate under the cornea's outer layer--the epithelium. This can cause erosion of the epithelium. This condition is known as recurrent epithelial erosion. These erosions: (1) Alter the cornea's normal curvature, resulting in temporary vision problems; and (2) Expose the nerves that line the cornea, causing severe pain. Even the involuntary act of blinking can be painful.

To ease this pain, a doctor may prescribe eye drops and ointments to reduce the friction on the eroded cornea. In some cases, an eye patch may be used to immobilize the eyelids. With effective care, these erosions usually heal within three days, although occasional sensations of pain may occur for the next six-to-eight weeks.

By about age 40, some people with lattice dystrophy will have scarring under the epithelium, resulting in a haze on the cornea that can greatly obscure vision. In this case, a corneal transplant may be needed. Although people with lattice dystrophy have an excellent chance for a successful transplant, the disease may also arise in the donor cornea in as little as three years. In one study, about half of the transplant patients with lattice dystrophy had a recurrence of the disease from between two to 26 years after the operation. Of these, 15 percent required a second corneal transplant. Early lattice and recurrent lattice arising in the donor cornea responds well to treatment with the excimer laser.

Although lattice dystrophy can occur at any time in life, the condition usually arises in children between the ages of two and seven.

Map-Dot-Fingerprint Dystrophy. This dystrophy occurs when the epithelium's basement membrane develops abnormally (the basement membrane serves as the foundation on which the epithelial cells, which absorb nutrients from tears, anchor and organize themselves). When the basement membrane develops abnormally, the epithelial cells cannot properly adhere to it. This, in turn, causes recurrent epithelial erosions, in which the epithelium's outermost layer rises slightly, exposing a small gap between the outermost layer and the rest of the cornea.

Epithelial erosions can be a chronic problem. They may alter the cornea's normal curvature, causing periodic blurred vision. They may also expose the nerve endings that line the tissue, resulting in moderate to severe pain lasting as long as several days. Generally, the pain will be worse on awakening in the morning. Other symptoms include sensitivity to light, excessive tearing, and foreign body sensation in the eye.

Map-dot-fingerprint dystrophy, which tends to occur in both eyes, usually affects adults between the ages of 40 and 70, although it can develop earlier in life. Also known as epithelial basement membrane dystrophy, map-dot-fingerprint dystrophy gets its name from the unusual appearance of the cornea during an eye examination. Most often, the affected epithelium will have a map-like appearance, i.e., large, slightly gray outlines that look like a continent on a map. There may also be clusters of opaque dots underneath or close to the map-like patches. Less frequently, the irregular basement membrane will form concentric lines in the central cornea that resemble small fingerprints.

Typically, map-dot-fingerprint dystrophy will flare up occasionally for a few years and then go away on its own, with no lasting loss of vision. Most people never know that they have map-dot-fingerprint dystrophy, since they do not have any pain or vision loss. However, if treatment is needed, doctors will try to control the pain associated with the epithelial erosions. They may patch the eye to immobilize it, or prescribe lubricating eye drops and ointments. With treatment, these erosions usually heal within three days, although periodic flashes of pain may occur for several weeks thereafter. Other treatments include anterior corneal punctures to allow better adherence of cells; corneal scraping to remove eroded areas of the cornea and allow regeneration of healthy epithelial tissue; and use of the excimer laser to remove surface irregularities.

Ocular Herpes. Herpes of the eye, or ocular herpes, is a recurrent viral infection that is caused by the herpes simplex virus and is the most common infectious cause of corneal blindness in the U.S. Previous studies show that once people develop ocular herpes, they have up to a 50 percent chance of having a recurrence. This second flare-up could come weeks or even years after the initial occurrence.

Ocular herpes can produce a painful sore on the eyelid or surface of the eye and cause inflammation of the cornea. Prompt treatment with anti-viral drugs helps to stop the herpes virus from multiplying and destroying epithelial cells. However, the infection may spread deeper into the cornea and develop into a more severe infection called stromal keratitis, which causes the body's immune system to attack and destroy stromal cells. Stromal keratitis is more difficult to treat than less severe ocular herpes infections. Recurrent episodes of stromal keratitis can cause scarring of the cornea, which can lead to loss of vision and possibly blindness.

Like other herpetic infections, herpes of the eye can be controlled. An estimated 400,000 Americans have had some form of ocular herpes. Each year, nearly 50,000 new and recurring cases are diagnosed in the United States, with the more serious stromal keratitis accounting for about 25 percent. In one large study, researchers found that recurrence rate of ocular herpes was 10 percent within one year, 23 percent within two years, and 63 percent within 20 years. Some factors believed to be associated with recurrence include fever, stress, sunlight, and eye injury.





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