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Corneal Disease (cont.)

Part 1: Corneal Dystrophies

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:

  • They are usually inherited.
  • They affect the right and left eyes equally.
  • They are not caused by outside factors, such as injury or diet.
  • Most progress gradually.
  • Most usually begin in one of the five corneal layers and may later spread to nearby layers.
  • Most do not affect other parts of the body, nor are they related to diseases affecting other parts of the eye or body.
  • Most can occur in otherwise totally healthy people, male or female.

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.

Herpes Zoster (Shingles). This infection is produced by the varicella-zoster virus, the same virus that causes chickenpox. After an initial outbreak of chickenpox (often during childhood), the virus remains inactive within the nerve cells of the central nervous system. But in some people, the varicella-zoster virus will reactivate at another time in their lives. When this occurs, the virus travels down long nerve fibers and infects some part of the body, producing a blistering rash (shingles), fever, painful inflammations of the affected nerve fibers, and a general feeling of sluggishness.

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.

Iridocorneal Endothelial Syndrome. More common in women and usually diagnosed between ages 30-50, iridocorneal endothelial (ICE) syndrome has three main features: (1) Visible changes in the iris, the colored part of the eye that regulates the amount of light entering the eye; (2) Swelling of the cornea; and (3) The development of glaucoma, a disease that can cause severe vision loss when normal fluid inside the eye cannot drain properly. ICE is usually present in only one eye.

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.

Keratoconus. This disorder--a progressive thinning of the cornea--is the most common corneal dystrophy in the U.S., affecting one in every 2000 Americans. It is more prevalent in teenagers and adults in their 20s. Keratoconus arises when the middle of the cornea thins and gradually bulges outward, forming a rounded cone shape. This abnormal curvature changes the cornea's refractive power, producing moderate to severe distortion (astigmatism) and blurriness (nearsightedness) of vision. Keratoconus may also cause swelling and a sight-impairing scarring of the tissue.

Studies indicate that keratoconus stems from one of several possible causes:

  • An inherited corneal abnormality. About seven percent of those with the condition have a family history of keratoconus.
  • An eye injury, i.e., excessive eye rubbing or wearing hard contact lenses for many years.
  • Certain eye diseases, such as retinitis pigmentosa, retinopathy of prematurity, and vernal keratoconjunctivitis.
  • Systemic diseases, such as Leber's congenital amaurosis, Ehlers-Danlos syndrome, Down syndrome, and osteogenesis imperfecta.

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.






Medical Dictionary