Keratoconus

Diagnosis

To diagnose keratoconus, an eye doctor will review a medical and family history and do an eye exam. Other tests also may be done to find out more about the shape of the cornea.

Tests to diagnose keratoconus include:

• Eye refraction. This test uses special equipment that measures the eyes. It may involve looking through a device that contains wheels of different lenses, called a phoropter. This device helps judge which combination offers the sharpest vision. Some eye doctors may use a hand-held instrument called a retinoscope to check the eyes.
• Slit-lamp examination. This test involves directing a vertical beam of light on the surface of the eye and using a low-powered microscope to view the eye. The eye doctor evaluates the shape of the cornea and looks for other potential problems in the eye.
• Keratometry. This exam involves focusing a circle of light on the cornea and measures the reflection. This determines the basic shape of the cornea.
• Computerized corneal mapping. Special photographic tests, such as corneal tomography and corneal topography, record images to create a detailed shape map of the cornea. Corneal tomography also can measure the thickness of the cornea. This type of testing can often detect early signs of keratoconus before the disease is visible by slit-lamp examination.

Treatment

Treatment for keratoconus depends on the severity of the condition and how quickly the condition is progressing. Generally, there are two approaches to treating keratoconus: slowing the progression of the disease and improving vision.

If keratoconus is progressing, corneal collagen cross-linking may be recommended to slow it or stop it from getting worse. This treatment aims to stabilize the structure of the cornea. It may decrease the bulging of the cornea and help achieve better vision with glasses or contact lenses. This treatment also has the potential to prevent needing a cornea transplant in the future.

Improving vision depends on the severity of keratoconus. Mild to moderate keratoconus can be treated with eyeglasses or contact lenses. This will likely be a long-term treatment, especially if the cornea becomes stable with time or from cross-linking.

In some people with keratoconus, the cornea becomes scarred with advanced disease. For others, wearing contact lenses becomes difficult. In these people, cornea transplant surgery might be necessary.

Lenses

• Eyeglasses or soft contact lenses. Glasses or soft contact lenses can correct blurry or distorted vision in early keratoconus. But people often need to change their prescription for eyeglasses or contacts as the shape of their corneas change.
• Hard contact lenses. Hard contact lenses are often the next step in treating more advanced keratoconus. Hard lenses include rigid, gas permeable types. Hard lenses may feel uncomfortable at first, but many people adjust to wearing them and they can provide excellent vision. This type of lens can be made to fit the corneas.
• Piggyback lenses. If rigid lenses are uncomfortable, an eye doctor may recommend "piggybacking" a hard contact lens on top of a soft one.
• Hybrid lenses. These contact lenses have a rigid center with a softer ring around the outside for increased comfort. People who can't tolerate hard contact lenses may prefer hybrid lenses.
• Scleral lenses. These lenses are useful for very irregular shape changes in the cornea in advanced keratoconus. Instead of resting on the cornea like traditional contact lenses do, scleral lenses sit on the white part of the eye, called the sclera, and vault over the cornea without touching it.

If you're using rigid or scleral contact lenses, make sure to have them fitted by an eye doctor with experience in treating keratoconus. You'll also need to have regular checkups to determine whether the lenses still fit well. An ill-fitting lens can damage the cornea.

Therapies

• Corneal cross-linking. In this procedure, the cornea is saturated with riboflavin eye drops and treated with ultraviolet light. This causes cross-linking of the cornea, which stiffens the cornea to prevent further shape changes. Corneal cross-linking may help to reduce the risk of progressive vision loss by stabilizing the cornea early in the disease.

Surgery

Surgery may be necessary if there is corneal scarring, extreme thinning of the cornea, poor vision with the strongest prescription lenses or an inability to wear any type of contact lenses.

Depending on the location of the bulging cone and the severity of the condition, surgical options include:

• Intrastromal corneal ring segments (ICRS). For mild to moderate keratoconus, an eye doctor may recommend inserting small synthetic rings in the cornea. This treatment can help flatten the cornea, which can help improve vision and make contact lenses fit better. Sometimes this procedure is done in combination with corneal cross-linking.
• Cornea transplant. If there is corneal scarring or extreme thinning, a cornea transplant may be needed. Depending on the situation, an eye doctor may recommend replacing all or part of the cornea with healthy donor tissue. A cornea transplant is known as a keratoplasty.

Cornea transplant for keratoconus generally is very successful. Possible complications include graft rejection, poor vision, infection and astigmatism. Astigmatism is often managed by wearing hard contact lenses again, which is usually more comfortable after a cornea transplant.

Preparing for your appointment

If you're having difficulty with your vision, you'll likely start by seeing an eye doctor, called an ophthalmologist or optometrist. If your eye doctor determines that you might have keratoconus, you may be referred to an ophthalmologist who has had special training in corneal disease and surgery. A trained ophthalmologist can interpret corneal imaging studies and determine if you need cross-linking or a cornea transplant.

Here's some information to help you get ready for your appointment.

What you can do

Before your appointment make a list of:

• Symptoms you've been having and for how long.
• Recent major stresses or life changes.
• All medicines, eye drops, vitamins and supplements you take, including the doses.
• Questions to ask your eye doctor.

For keratoconus some basic questions to ask include:

• What's the most likely cause of my symptoms?
• What are other possible causes?
• Do I need any tests?
• Is this condition temporary?
• What treatments are available, and which do you recommend?
• What are the alternatives to the primary approach you're suggesting?
• I have other health conditions. How can I best manage them together?
• Do you have any brochures or other printed material I can take with me? What websites do you recommend?

What to expect from your doctor

Your eye doctor is likely to ask you a few questions, such as:

• What types of symptoms have you been having?
• When did you begin experiencing symptoms?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• Does anything seem to improve your symptoms?
• Do you rub your eyes?
• What, if anything, appears to worsen your symptoms?
• Does anyone in your family have keratoconus?