Causes, incidence, and risk factors

The cause is unknown, but the tendency to develop keratoconus is probably present from birth. Keratoconus is thought to involve a defect in collagen, the tissue that makes up most of the cornea.

Some researchers believe that allergy and eye rubbing may play a role.

Also, there is an association between keratoconus and Down syndrome.

Symptoms

The earliest symptom is a subtle blurring of vision that cannot be corrected with glasses. (Vision can generally be corrected to 20/20 with rigid, gas-permeable contact lenses.)

Most people who develop keratoconus start out nearsighted. The nearsightedness tends to become worse over time.

Signs and tests

Keratoconus is often discovered during adolescence. It can usually be diagnosed with a slit-lamp examination of the cornea. The most accurate test is called corneal topography, which creates a map of the curve of the cornea.

When keratoconus is advanced, the cornea will be thinner at the point of the cone. This can be measured with a painless test called pachymetry.

Treatment

Contact lenses are the main treatment for most patients with keratoconus. Severe cases may require corneal transplantation as a last resort.

The following newer technologies may delay or prevent the need for corneal transplantation:

• Using high-frequency radio energy or corneal implants called intracorneal ring segments, the shape of the cornea can be changed so that vision with contact lenses is improved.
• An experimental treatment called corneal cross-linking, which will soon be available, causes the cornea to become hard and stops the condition from getting worse. The cornea can then be reshaped with laser vision correction.

Expectations (prognosis)

In most cases, vision can be corrected with rigid gas-permeable contact lenses.

If corneal transplantation is needed, results are usually good (read my corneal surgery diary.) The recovery period can be long, and patients often still need contact lenses.

Complications

There is a risk of rejection after corneal transplantation, but the risk is much lower than with other organ transplants.

Patients with even borderline keratoconus should not have laser vision correction. Corneal topography is done before laser vision correction to rule out people with this condition.

Calling your health care provider

Young persons whose vision cannot be corrected to 20/20 with glasses should be evaluated by an eye doctor experienced with keratoconus.

Prevention

There are no preventive measures. Some specialists believe that patients with keratoconus should have their eye allergies aggressively treated and should be instructed not to rub their eyes.

Review Date: 7/28/2010.

Reviewed by: Linda J. vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; and Franklin W. Lusby, MD, Ophthalmologist, Lusby Vision Institute, La Jolla, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.