Keratoconus

Synonyms

3

Overview

Keratoconus is the degeneration of the structure of the cornea, which is the clear tissue, dome-shaped covering the front of the eye. In this condition, the shape of the cornea slowly changes from the normal round shape to a cone shape. Most people who develop keratoconus start out nearsighted, which tends to become worse over time. The earliest symptom is a slight blurring of vision that cannot be corrected with glasses. Over time, there may be eye halos, glare, or other night vision problems. Keratoconus usually affects both eyes and generally begins to first affect people ages 10 to 25. The condition may progress slowly for 10 years or longer.

In the early stages of keratoconus, you can correct vision problems with glasses or soft contact lenses. Later you may have to be fitted with rigid gas permeable contact lenses or other types of lenses. If your condition progresses to an advanced stage, you may need a cornea transplant.

Risk factors:
These factors can increase your chances of developing keratoconus:

• Having a family history of keratoconus
• Rubbing your eyes vigorously
• Having certain conditions, such as retinitis pigmentosa, Down syndrome, Ehlers-Danlos syndrome, hay fever and asthma

Complications:

In some situations, your cornea may swell quickly and cause sudden reduced vision and scarring of the cornea. This is caused by a condition in which the inside lining of your cornea breaks down, allowing fluid to enter the cornea (hydrops).

In advanced keratoconus, your cornea may become scarred, particularly where the cone forms. A scarred cornea causes worsening vision problems and may require corneal transplant surgery.

Symptoms

Signs and symptoms of keratoconus may change as the disease progresses. They include:

• Blurred or distorted vision
• Increased sensitivity to bright light and glare, which can cause problems with night driving
• A need for frequent changes in eyeglass prescriptions
• Sudden worsening or clouding of vision

Causes

The exact cause of keratoconus is unknown. Both genetic and environmental factors may play a role in the development of keratoconus.

The genetic factors involve abnormalities in the structure of collagen, which result in a weak and flexible cornea. Keratoconus is more common in people with Down syndrome, Marfan syndrome, and Leber congenital amaurosis, and certain genetic conditions. In these cases, the cause depends on the specific condition.

Environmental factors may include living in sunny, hot areas of the world, while eye-rubbing is a major behavioral factor in the disease. Malfunctioning enzymes that normally help maintain the health of the cornea may play a role. All of these factors contribute to the main problem in keratoconus, which is the defective collagen structure that results in thinning and irregularity of the cornea. Keratoconus occurs more frequently in patients with atopy (asthma and eczema) or severe ocular allergies. It may also be linked to hormonal factors because it is more frequent during puberty and also may progress during pregnancy.

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

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.

Diagnosis

To diagnose keratoconus, your eye doctor (ophthalmologist) will review your medical and family history and conduct an eye exam. He or she may conduct other tests to determine more details regarding the shape of your cornea. Tests to diagnose keratoconus include:

Eye refraction. In this test your eye doctor uses special equipment that measures your eyes to check for vision problems. He or she may ask you to look through a device that contains wheels of different lenses (phoropter) to help judge which combination gives you the sharpest vision. Some doctors may use a hand-held instrument (retinoscope) to evaluate your eyes.

Slit-lamp examination. In this test your doctor directs a vertical beam of light on the surface of your eye and uses a low-powered microscope to view your eye. He or she evaluates the shape of your cornea and looks for other potential problems in your eye.

The doctor may repeat the test after you've had eyedrops applied to dilate your pupils. This helps with viewing the back of your eye.

Keratometry. In this test your eye doctor focuses a circle of light on your cornea and measures the reflection to determine the basic shape of your cornea.

Computerized corneal mapping. Special photographic tests, such as optical coherence tomography and corneal topography, record images of your cornea to create a detailed shape map of your cornea's surface. The tests can also measure the thickness of your cornea.

Prognosis

Patients with keratoconus typically present initially with mild astigmatism and myopia, commonly at the onset of puberty, and are diagnosed by the late teenage years or early 20s. The disease can, however, present or progress at any age; in rare cases, keratoconus can present in children or not until later adulthood. A diagnosis of the disease at an early age may indicate a greater risk of severity in later life. Patients' vision will seem to fluctuate over a period of months, driving them to change lens prescriptions frequently, but as the condition worsens, contact lenses are required in the majority of cases. The course of the disorder can be quite variable, with some patients remaining stable for years or indefinitely, while others progress rapidly or experience occasional exacerbations over a long and otherwise steady course. Most commonly, keratoconus progresses for a period of 10 to 20 years before the course of the disease generally ceases in the third and fourth decades of life.

Corneal hydrops:
In advanced cases, bulging of the cornea can result in a localized rupture of Descemet's membrane, an inner layer of the cornea. Aqueous humor from the eye's anterior chamber seeps into the cornea before Descemet's membrane reseals. The patient experiences pain and a sudden severe clouding of vision, with the cornea taking on a translucent milky-white appearance known as a corneal hydrops. Although disconcerting to the patient, the effect is normally temporary and after a period of six to eight weeks, the cornea usually returns to its former transparency. The recovery can be aided nonsurgically by bandaging with an osmotic saline solution. Although a hydrops usually causes increased scarring of the cornea, occasionally it will benefit a patient by creating a flatter cone, aiding the fitting of contact lenses. corneal transplantation is not usually indicated during corneal Hydrops.

Treatment

Treatment for keratoconus depends on the severity of your condition and how quickly the condition is progressing.

Mild to moderate keratoconus can be treated with eyeglasses or contact lenses. For most people, the cornea will become stable after a few years. If you have this type, you likely won't experience severe vision problems or require further treatment.

In some people with keratoconus, the cornea becomes scarred or wearing contact lenses becomes difficult. In these cases, 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 frequently need to change their prescription for eyeglasses or contacts as the shape of their corneas change.
• Hard contact lenses. Hard (rigid gas permeable) contact lenses are often the next step in treating progressing keratoconus. 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 your corneas.
• Piggyback lenses. If rigid lenses are uncomfortable, your 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 your 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 (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 fitting remains satisfactory. An ill-fitting lens can damage your cornea.

Surgery:
You may need surgery if you have corneal scarring, extreme thinning of your cornea, poor vision with the strongest prescription lenses or an inability to wear any type of contact lenses. Several surgeries are available, depending on the location of the bulging cone and the severity of your condition.

Surgical options include:

• Corneal inserts. During this surgery, your doctor places tiny, clear, crescent-shaped plastic inserts (intracorneal ring sigments) into your cornea to flatten the cone, support the cornea's shape and improve vision. Corneal inserts can restore a more normal corneal shape, slow progress of keratoconus and reduce the need for a cornea transplant. This surgery may also make it easier to fit and tolerate contact lenses. The corneal inserts can be removed, so the procedure can be considered a temporary measure. This surgery carries risks, such as infection and injury to the eye.
• Cornea transplant. If you have corneal scarring or extreme thinning, you'll likely need a cornea transplant (keratoplasty).

Lamellar keratoplasty is a partial-thickness transplant, in which only a section of the cornea's surface is replaced.

Penetrating keratoplasty is a full-cornea transplant. In this procedure, doctors remove a full-thickness portion of your central cornea and replace it with donor tissue.

A deep anterior lamellar keratoplasty (DALK) preserves the inside lining of the cornea (endothelium). It helps avoid rejection of this critical inside lining that can occur with a full-thickness transplant.

Recovery after keratoplasty can take up to one year, and you may need to continue wearing rigid contact lenses to have clear vision. Full improvement of vision may occur several years after your transplant.

Cornea transplant for keratoconus generally is very successful, but possible complications include graft rejection, poor vision, astigmatism, inability to wear contact lenses and infection.

Implants:
Keratoconus can also be treated with Intacs, which are small curved implantable corneal devices that can reshape the cornea. Intacs are FDA approved and can help flatten the steep cornea found in keratoconus.

Potential future treatment:
A treatment called collagen cross-linking shows promise for people with keratoconus. The process involves using special eyedrops and ultraviolet A (UVA) light to strengthen (cross-link) the tissues of the cornea. The treatment is still in the testing phase in the United States, and additional study is needed before it becomes widely available.