The cornea is the eye's outermost layer. It is the clear, dome-shaped surface that covers the front of the eye.
Structure of the Cornea
Although the cornea is clear and seems to lack substance, it is actually a highly organized group of cells and proteins. Unlike most tissues in the body, the cornea contains no blood vessels to nourish or protect it against infection. Instead, the cornea receives its nourishment from the tears and aqueous humor that fills the chamber behind it. The cornea must remain transparent to refract light properly, and the presence of even the tiniest blood vessels can interfere with this process. To see well, all layers of the cornea must be free of any cloudy or opaque areas.
About 120 million people in the United States wear eyeglasses or contact lenses to correct nearsightedness, farsightedness, or astigmatism. These vision disorders--called refractive errors-- affect the cornea and are the most common of all vision problems in this country.
Refractive errors occur when the curve of the cornea is irregularly shaped (too steep or too flat). When the cornea is of normal shape and curvature, it bends, or refracts, light on the retina with precision. However, when the curve of the cornea is irregularly shaped, the cornea bends light imperfectly on the retina. This affects good vision. The refractive process is similar to the way a camera takes a picture. The cornea and lens in your eye act as the camera lens. The retina is similar to the film. If the image is not focused properly, the film (or retina) receives a blurry image. The image that your retina "sees" then goes to your brain, which tells you what the image is.
When the cornea is curved too much, or if the eye is too long, faraway objects will appear blurry because they are focused in front of the retina. This is called myopia, or nearsightedness. Myopia affects over 25 percent of all adult Americans.
Hyperopia, or farsightedness, is the opposite of myopia. Distant objects are clear, and close-up objects appear blurry. With hyperopia, images focus on a point beyond the retina. Hyperopia results from an eye that is too short.
Astigmatism is a condition in which the uneven curvature of the cornea blurs and distorts both distant and near objects. A normal cornea is round, with even curves from side to side and top to bottom. With astigmatism, the cornea is shaped more like the back of a spoon, curved more in one direction than in another. This causes light rays to have more than one focal point and focus on two separate areas of the retina, distorting the visual image. Two-thirds of Americans with myopia also have astigmatism.
Refractive errors are usually corrected by eyeglasses or contact lenses. Although these are safe and effective methods for treating refractive errors, refractive surgeries are becoming an increasingly popular option.
Because the cornea is as smooth and clear as glass but is strong and durable, it helps the eye in two ways:
When light strikes the cornea, it bends--or refracts--the incoming light onto the lens. The lens further refocuses that light onto the retina, a layer of light sensing cells lining the back of the eye that starts the translation of light into vision. For you to see clearly, light rays must be focused by the cornea and lens to fall precisely on the retina. The retina converts the light rays into impulses that are sent through the optic nerve to the brain, which interprets them as images.
The refractive process is similar to the way a camera takes a picture. The cornea and lens in the eye act as the camera lens. The retina is similar to the film. If the image is not focused properly, the film (or retina) receives a blurry image.
The cornea also serves as a filter, screening out some of the most damaging ultraviolet (UV) wavelengths in sunlight. Without this protection, the lens and the retina would be highly susceptible to injury from UV radiation.
Some diseases and disorders of the cornea are:
An increasing number of eye allergy cases are related to medications and contact lens wear. Also, animal hair and certain cosmetics, such as mascara, face creams, and eyebrow pencil, can cause allergies that affect the eye. Touching or rubbing eyes after handling nail polish, soaps, or chemicals may cause an allergic reaction. Some people have sensitivity to lip gloss and eye makeup. Allergy symptoms are temporary and can eliminated by not having contact with the offending cosmetic or detergent.
At its onset, conjunctivitis is usually painless and does not adversely affect vision. The infection will clear in most cases without requiring medical care. But for some forms of conjunctivitis, treatment will be needed. If treatment is delayed, the infection may worsen and cause corneal inflammation and a loss of vision.
As a general rule, the deeper the corneal infection, the more severe the symptoms and complications. It should be noted that corneal infections, although relatively infrequent, are the most serious complication of contact lens wear.
Minor corneal infections are commonly treated with anti-bacterial eye drops. If the problem is severe, it may require more intensive antibiotic or anti-fungal treatment to eliminate the infection, as well as steroid eye drops to reduce inflammation. Frequent visits to an eye care professional may be necessary for several months to eliminate the problem.
The tear film consists of three layers--an outer, oily (lipid) layer that keeps tears from evaporating too quickly and helps tears remain on the eye; a middle (aqueous) layer that nourishes the cornea and conjunctiva; and a bottom (mucin) layer that helps to spread the aqueous layer across the eye to ensure that the eye remains wet. As we age, the eyes usually produce fewer tears. Also, in some cases, the lipid and mucin layers produced by the eye are of such poor quality that tears cannot remain in the eye long enough to keep the eye sufficiently lubricated.
The main symptom of dry eye is usually a scratchy or sandy feeling as if something is in the eye. Other symptoms may include stinging or burning of the eye; episodes of excess tearing that follow periods of very dry sensation; a stringy discharge from the eye; and pain and redness of the eye. Sometimes people with dry eye experience heaviness of the eyelids or blurred, changing, or decreased vision, although loss of vision is uncommon.
Dry eye is more common in women, especially after menopause. Surprisingly, some people with dry eye may have tears that run down their cheeks. This is because the eye may be producing less of the lipid and mucin layers of the tear film, which help keep tears in the eye. When this happens, tears do not stay in the eye long enough to thoroughly moisten it.
Dry eye can occur in climates with dry air, as well as with the use of some drugs, including antihistamines, nasal decongestants, tranquilizers, and anti-depressant drugs. People with dry eye should let their health care providers know all the medications they are taking, since some of them may intensify dry eye symptoms.
People with connective tissue diseases, such as rheumatoid arthritis, can also develop dry eye. It is important to note that dry eye is sometimes a symptom of Sjögren's syndrome, a disease that attacks the body's lubricating glands, such as the tear and salivary glands. A complete physical examination may diagnose any underlying diseases.
Artificial tears, which lubricate the eye, are the principal treatment for dry eye. They are available over-the-counter as eye drops. Sterile ointments are sometimes used at night to help prevent the eye from drying. Using humidifiers, wearing wrap-around glasses when outside, and avoiding outside windy and dry conditions may bring relief. For people with severe cases of dry eye, temporary or permanent closure of the tear drain (small openings at the inner corner of the eyelids where tears drain from the eye) may be helpful.
Fuchs' dystrophy occurs when endothelial cells gradually deteriorate without any apparent reason. As more endothelial cells are lost over the years, the endothelium becomes less efficient at pumping water out of the stroma. This causes the cornea to swell and distort vision. Eventually, the epithelium also takes on water, resulting in pain and severe visual impairment.
Epithelial swelling damages vision by changing the cornea's normal curvature, and causing a sight-impairing haze to appear in the tissue. Epithelial swelling will also produce tiny blisters on the corneal surface. When these blisters burst, they are extremely painful.
At first, a person with Fuchs' dystrophy will awaken with blurred vision that will gradually clear during the day. This occurs because the cornea is normally thicker in the morning; it retains fluids during sleep that evaporate in the tear film while we are awake. As the disease worsens, this swelling will remain constant and reduce vision throughout the day.
When treating the disease, doctors will try first to reduce the swelling with drops, ointments, or soft contact lenses. They also may instruct a person to use a hair dryer, held at arm's length or directed across the face, to dry out the epithelial blisters. This can be done two or three times a day.
When the disease interferes with daily activities, a person may need to consider having a corneal transplant to restore sight. The short-term success rate of corneal transplantation is quite good for people with Fuchs' dystrophy. However, some studies suggest that the long-term survival of the new cornea can be a problem.
A corneal dystrophy is a condition in which one or more parts of the cornea lose their normal clarity due to a buildup of cloudy material. There are over 20 corneal dystrophies that affect all parts of the cornea. These diseases share many traits:
Corneal dystrophies affect vision in widely differing ways. Some cause severe visual impairment, while a few cause no vision problems and are discovered during a routine eye examination. Other dystrophies may cause repeated episodes of pain without leading to permanent loss of vision.
Some of the most common corneal dystrophies include Fuchs' dystrophy, keratoconus, lattice dystrophy, and map-dot-fingerprint dystrophy.
Varicella-zoster virus may travel to the head and neck, perhaps involving an eye, part of the nose, cheek, and forehead. In about 40 percent of those with shingles in these areas, the virus infects the cornea. Doctors will often prescribe oral anti-viral treatment to reduce the risk of the virus infecting cells deep within the tissue, which could inflame and scar the cornea. The disease may also cause decreased corneal sensitivity, meaning that foreign matter, such as eyelashes, in the eye are not felt as keenly. For many, this decreased sensitivity will be permanent.
Although shingles can occur in anyone exposed to the varicella-zoster virus, research has established two general risk factors for the disease: (1) Advanced age; and (2) A weakened immune system. Studies show that people over age 80 have a five times greater chance of having shingles than adults between the ages of 20 and 40. Unlike herpes simplex I, the varicella-zoster virus does not usually flare up more than once in adults with normally functioning immune systems.
Be aware that corneal problems may arise months after the shingles are gone. For this reason, it is important that people who have had facial shingles schedule follow-up eye examinations.
ICE syndrome is actually a grouping of three closely linked conditions: iris nevus (or Cogan-Reese) syndrome; Chandler's syndrome; and essential (progressive) iris atrophy (hence the acronym ICE). The most common feature of this group of diseases is the movement of endothelial cells off the cornea onto the iris. This loss of cells from the cornea often leads to corneal swelling, distortion of the iris, and variable degrees of distortion of the pupil, the adjustable opening at the center of the iris that allows varying amounts of light to enter the eye. This cell movement also plugs the fluid outflow channels of the eye, causing glaucoma.
The cause of this disease is unknown. While we do not yet know how to keep ICE syndrome from progressing, the glaucoma associated with the disease can be treated with medication, and a corneal transplant can treat the corneal swelling.
Studies indicate that keratoconus stems from one of several possible causes:
Keratoconus usually affects both eyes. At first, people can correct their vision with eyeglasses. But as the astigmatism worsens, they must rely on specially fitted contact lenses to reduce the distortion and provide better vision. Although finding a comfortable contact lens can be an extremely frustrating and difficult process, it is crucial because a poorly fitting lens could further damage the cornea and make wearing a contact lens intolerable.
In most cases, the cornea will stabilize after a few years without ever causing severe vision problems. But in about 10 to 20 percent of people with keratoconus, the cornea will eventually become too scarred or will not tolerate a contact lens. If either of these problems occur, a corneal transplant may be needed. This operation is successful in more than 90 percent of those with advanced keratoconus. Several studies have also reported that 80 percent or more of these patients have 20/40 vision or better after the operation.
The National Eye Institute is conducting a natural history study--called the Collaborative Longitudinal Evaluation of Keratoconus Study--to identify factors that influence the severity and progression of keratoconus.
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.
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 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.
The National Eye Institute supported the Herpetic Eye Disease Study, a group of clinical trials that studied various treatments for severe ocular herpes.
Pterygia are more common in sunny climates and in the 20-40 age group. Scientists do not know what causes pterygia to develop. However, since people who have pterygia usually have spent a significant time outdoors, many doctors believe ultraviolet (UV) light from the sun may be a factor. In areas where sunlight is strong, wearing protective eyeglasses, sunglasses, and/or hats with brims are suggested. While some studies report a higher prevalence of pterygia in men than in women, this may reflect different rates of exposure to UV light.
Because a pterygium is visible, many people want to have it removed for cosmetic reasons. It is usually not too noticeable unless it becomes red and swollen from dust or air pollutants. Surgery to remove a pterygium is not recommended unless it affects vision. If a pterygium is surgically removed, it may grow back, particularly if the patient is less than 40 years of age. Lubricants can reduce the redness and provide relief from the chronic irritation.
Scientists are not certain why SJS develops. The most commonly cited cause of SJS is an adverse allergic drug reaction. Almost any drug--but most particularly sulfa drugs--can cause SJS. The allergic reaction to the drug may not occur until 7-14 days after first using it. SJS can also be preceded by a viral infection, such as herpes or the mumps, and its accompanying fever, sore throat, and sluggishness. Treatment for the eye may include artificial tears, antibiotics, or corticosteroids. About one-third of all patients diagnosed with SJS have recurrences of the disease.
SJS occurs twice as often in men as women, and most cases appear in children and young adults under 30, although it can develop in people at any age.
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