Keratoconus Causes, Symptoms and 10 Treatment Options
Keratoconus is a progressive eye disease in which the normally round cornea thins and begins to bulge into a cone-like shape. This cone shape deflects light as it enters the eye on its way to the light-sensitive retina, causing distorted vision.
Keratoconus can occur in one or both eyes and often begins during a person's teens or early 20s.
Keratoconus symptoms and signs
As the cornea becomes more irregular in shape, it causes progressive nearsightedness and irregular astigmatism to develop, creating additional problems with distorted and blurred vision. Glare and light sensitivity also may occur.
What causes keratoconus?
New research suggests the weakening of the corneal tissue that leads to keratoconus may be due to an imbalance of enzymes within the cornea. This imbalance makes the cornea more susceptible to oxidative damage from compounds called free radicals, causing it to weaken and bulge forward.
Risk factors for oxidative damage and weakening of the cornea include a genetic predisposition, explaining why keratoconus often affects more than one member of the same family.
FDA Approved Corneal Cross-Linking
For Progressive KC, Only One Cross-Linking Procedure Is FDA Approved
In April, 2016, Avedro's Photrexa Viscous® (riboflavin 5’-phosphate in 20% dextran ophthalmic solution), Photrexa® (riboflavin 5’-phosphate ophthalmic solution) and KXL® System were approved by the U.S. Food and Drug Administration (FDA) as the first and only epithelium off (epi-off) corneal cross-linking treatment for progressive keratoconus.
Since approval, more than 10,000 FDA-approved epi-off corneal cross-linking procedures have been completed at more than 300 leading practices nationwide. A growing number are now being covered by commercial insurers. More than 55 carriers, representing approximately 175 million insured Americans, now cover the treatment.
Supported by the National Keratoconus Foundation, this safe, minimally invasive outpatient procedure helps stiffen the cornea weakened by disease. Make sure your corneal cross-linking procedure is the FDA-approved epi-off cross-linking procedure. Click here to find a cornea specialist near you and to see the growing list of insurers covering the Avedro procedure.
Important Safety Information »
Photrexa Viscous® (riboflavin 5'-phosphate in 20% dextran ophthalmic solution) and Photrexa® (riboflavin 5'-phosphate ophthalmic solution) are used with the KXL® System in corneal cross-linking (CXL) to treat eyes in which the cornea, the clear dome shaped surface that covers the front of the eye, has been weakened from the progression of the disease keratoconus or following refractive surgery, a method for correcting or improving your vision.
What warnings should I know about corneal cross-linking?
Ulcerative keratitis, a potentially serious eye infection, can occur. Your doctor should monitor defects in the outermost corneal layer of the eye for resolution.
Who should not receive CXL?
The safety and effectiveness of CXL has not been studied in pregnant women, women who are breastfeeding, patients who are less than 14 years of age and patients 65 years of age or older.
What are the side effects of CXL?
In progressive keratoconus patients, the most common side effects in any CXL treated eye were haze, inflammation, fine white lines, disruption of surface cells, eye pain, reduced sharpness of vision, and blurred vision. In patients with corneal ectasia following refractive surgery, the most common side effects in any CXL treated eye were haze, disruption of surface cells, fine white lines, dry eye, eye pain, inflammation, light sensitivity, reduced sharpness of vision, and blurred vision.
These are not all of the side effects of the CXL treatment. Tell your healthcare provider if you have any side effect that bothers you or that does not go away.
You may report a side effect to Avedro by calling 1-844-528-3376, Option 1 or you may contact the U.S. Food and Drug Administration (FDA) directly at 1-800-FDA-1088.
For more information, ask your healthcare provider or see Prescribing Information.
In the mildest form of keratoconus, eyeglasses or soft contact lenses may help. But as the disease progresses and the cornea thins and becomes increasingly more irregular in shape, glasses and regular soft contact lens designs no longer provide adequate vision correction.
The aim of corneal cross-linking is to strengthen the cornea by increasing the number of "anchors" that bond collagen fibers together. (Diagram: Boxer Wachler Vision Institute)
Normal eye vs. eye with keratoconus. The keratoconic cornea bulges slightly, for a more cone-shaped surface. (Artist's re-creation of keratometry images.)
Treatments for progressive keratoconus include:
1. Corneal crosslinking
This procedure, also called corneal collagen cross-linking or CXL, strengthens corneal tissue to halt bulging of the eye's surface in keratoconus.
There are two versions of corneal crosslinking: epithelium-off and epithelium-on.
With epithelium-off crosslinking, the outer layer of the cornea (called the epithelium) is removed to allow entry of riboflavin, a type of B vitamin, into the cornea, which then is activated with UV light.
With the epithelium-on method (also called transepithelial crosslinking), the corneal epithelium is left intact during the treatment. The epithelium-on method requires more time for the riboflavin to penetrate into the cornea, but potential advantages include less risk of infection, less discomfort and faster visual recovery, according to supporters of this technique.
In April 2016, the FDA granted approval of Avedro's KXL System corneal collagen cross-linking treatment of progressive keratoconus. The approval includes use of the pharmaceutical and medical device company's Photrexa and Photrexa Viscous riboflavin solutions with the KXL System during the procedure.
Avedro’s Photrexa/KXL System currently is the only FDA-approved platform and procedure for CXL treatment of progressive keratoconus in the United States.
Corneal crosslinking may reduce significantly the need for corneal transplants among keratoconus patients. It also is being investigated as a way to treat or prevent complications following LASIK or other vision correction surgery.
Using a combination of corneal crosslinking and Intacs implants also has demonstrated promising results for treating keratoconus. Also, progressive mild to moderate keratoconus has been safely and successfully treated with a combination of corneal crosslinking and implantation of a toric phakic IOL.
2. Custom soft contact lenses
Recently, contact lens manufacturers have introduced custom soft contact lenses specially designed to correct mild-to-moderate keratoconus. These lenses are made-to-order based on detailed measurements of the person's keratoconic eye(s) and may be more comfortable than gas permeable lenses (GPs) or hybrid contact lenses for some wearers.
In the United States, custom soft contacts that are available for the correction of keratoconus include:
- KeraSoft lenses (Bausch + Lomb). These high-water silicone hydrogel lenses can correct up to 20 diopters (D) of nearsightedness or farsightedness and up to -12 D of astigmatism.
- NovaKone lenses (Bausch + Lomb). These medium-water hydrogel lenses can correct up to 30 D of nearsightedness or farsightedness and up to -10 D of astigmatism.
Both lenses have a very wide range of fitting parameters for a customized fit and are larger in diameter than regular soft lenses for greater stability on a keratoconic eye.
In a recent study of the visual performance of toric soft contacts and rigid gas permeable lenses for the correction of mild keratoconus, though GP lenses provided better visual acuity in low-contrast situations, soft toric lenses performed equally well in high-contrast acuity testing.
Custom toric soft contacts for keratoconus are significantly more expensive than regular soft contacts, but vision insurance may cover some of the costs associated with contact lenses for keratoconus.
3. Gas permeable contact lenses
If eyeglasses or soft contact lenses cannot control keratoconus, then gas permeable contact lenses usually are the preferred treatment. GP lenses vault over the cornea, replacing its irregular shape with a smooth, uniform refracting surface to improve vision.
Fitting contact lenses on an eye with keratoconus often is challenging and time-consuming. You can expect frequent return visits to your eye care provider so he or she can fine-tune the fit and your prescription, especially if your keratoconus continues to progress.
4. "Piggybacking" contact lenses
Because fitting a gas permeable contact lens over a cone-shaped cornea can sometimes be uncomfortable for a person with keratoconus, some eye care practitioners advocate "piggybacking" two different types of contact lenses on the same eye.
For keratoconus, this method involves placing a soft contact lens, such as one made of silicone hydrogel, over the eye and then fitting a GP lens over the soft lens. This approach increases wearer comfort because the soft lens acts like a cushioning pad under the rigid GP lens.
Your eye care practitioner will monitor closely the fitting of "piggyback" contact lenses to make sure enough oxygen reaches the surface of your eye, which can be a problem when two lenses are worn on the same eye. However, most modern contacts — both GP and soft — typically have adequate oxygen permeability for a safe "piggyback" fit.
5. Hybrid contact lenses
The UltraHealth and ClearKone hybrid contact lenses manufactured by SynergEyes combine a highly oxygen-permeable rigid center with a soft peripheral "skirt." These lenses were designed specifically for keratoconus, and the central GP zone of the lens vaults over the cone-shaped cornea for increased comfort.
Hybrid contact lenses provide the crisp optics of a gas permeable contact lens and wearing comfort that rivals that of soft lenses, according to SynergEyes. UltraHealth and ClearKone are available in a wide variety of parameters to provide a fit that conforms well to the irregular shape of a keratoconic eye.
6. Scleral and semi-scleral lenses
These are large-diameter gas permeable contacts — large enough that the periphery and edge of the lens rest on the "white" of the eye (sclera). Scleral lenses cover a larger portion of the sclera, whereas semi-scleral lenses cover a smaller area.
Because the center of scleral and semi-scleral lenses vaults over the irregularly shaped cornea, these lenses don't apply pressure to the eye's cone-shaped surface for a more comfortable fit. These larger lenses also are more stable than conventional gas permeable contact lenses, which move with each blink because they cover only a portion of the cornea.
7. Prosthetic lenses
Because keratoconic eyes are so challenging to fit, patients with severe disease often require an advanced scleral lens design that doubles as a prosthetic shell.
One example of a prosthetic lens is the BostonSight PROSE, a prosthetic replacement of the ocular surface ecosystem that's been in use since the 1990's. This treatment restores visual function, supports healing, reduces symptoms and improves quality of life for patients suffering with complex corneal disease such as keratoconus.
To qualify for PROSE, you must have a severe, unusual or otherwise untreatable condition. In cases of financial need, the non-profit Boston Foundation for Sight, which developed PROSE, may help subsidize the cost of the fitting and lens, which typically runs several thousand dollars. Also, PROSE is currently only available at a few top-ranked academic medical centers.
A second type of lens is the EyePrintPRO (EyePrint Prosthetics). This prosthetic scleral cover shell is designed utilizing Elevation Specific Technology to exactly match the unique irregularities of each individual eye. The process begins with an EyePrint Impression, which captures the precise curvatures of the entire ocular surface. Doctors then send the resulting impression to an EyePrint lab for 3-D scanning. The scanned data is then fed to a numerically controlled machining system to produce a lens that matches the impression exactly and therefore fits the individual cornea and sclera perfectly. Because of the precise nature of the back surface fit, high quality and individualized optics can be placed on the front surface of the device.
"The EyePrint is like a fingerprint—it's completely individual," says EyePrint developer Dr. Christine Sindt, Clinical Associate Professor of Ophthalmology and Visual Sciences at University of Iowa.
The EyePrintPRO can only be fit by doctors who undergo specific training and certification. However, the device is now available at nearly 50 practices throughout the US and Canada.
Intacs (Addition Technology) are FDA-approved clear, arc-shaped corneal inserts that are surgically positioned within the peripheral cornea to reshape the front surface of the eye for clearer vision.
Intacs may be needed when keratoconus patients no longer can obtain functional vision with contact lenses or eyeglasses.
Several studies show that Intacs can improve the best spectacle-corrected visual acuity (BSCVA) of a keratoconic eye by an average of two lines on a standard eye chart. The implants also have the advantage of being removable and exchangeable. The surgical procedure takes only about 10 minutes.
Intacs might delay but can't prevent a corneal transplant if keratoconus continues to progress.
9. Topography-guided conductive keratoplasty
While more study is needed, early results of a small study involving topography-guided conductive keratoplasty (CK) show this procedure might help smooth irregularities in the corneal surface.
This treatment uses energy from radio waves, applied with a small probe at several points in the periphery of the cornea to reshape the eye's front surface. A topographic "map" created by computer imaging of the eye's surface helps create individualized treatment plans.
10. Corneal transplant
Some people with keratoconus can't tolerate a rigid contact lens, or they reach the point where contact lenses or other therapies no longer provide acceptable vision.
The last remedy to be considered may be a cornea transplant, also called a penetrating keratoplasty (PK or PKP). Even after a transplant, you most likely will need glasses or contact lenses for clear vision.
More Keratoconus Articles
Keratoconus | Keratoconus FAQ | Intacs Treatment for Keratoconus | Scleral Lenses
Corneal Crosslinking for Keratoconus | Answers from an Eye Doctor About Keratoconus
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How can I prevent my keratoconus from getting worse?
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Is keratoconus covered by health insurance?
Is keratoconus genetic?
Is there a cure for keratoconus?
What are the best contact lenses for keratoconus?
What is corneal cross-linking for keratoconus?
What is keratoconus?
About the Reviewer: William Trattler, MD, is a board-certified ophthalmologist specializing in refractive, corneal and cataract eye surgery at the Center for Excellence in Eye Care — a private practice in Miami that treats a wide range of vision disorders and provides laser vision correction. More about Dr. Trattler
Page updated December 2018