KERATOCONUS

The term keratoconus is derived from two Greek words: “kerato”, meaning cornea, and “konos”, meaning cone. Keratoconus is a disease of the cornea in which the cornea looses its natural round shape and becomes distorted with cone-like bulging, progressive thinning, and associated reduction in vision quality. Keratoconus is a progressive disease that can range from very mild to very severe. The progression is generally slow and may stop at any stage. Keratoconus is one of a group of corneal degenerations that is characterized by corneal thinning. These conditions are termed “Ectasias” of the cornea.

What Causes Keratoconus?

Although we have been aware of keratoconus for over two hundred years, we are still not certain about the cause of keratoconus. We are fairly certain that it is genetically programmed and family history is a risk factor. About 7% of patients with keratoconus have known relatives with the disease. Generally speaking, there is about a 1 in 10 chance of a patient with keratoconus having an offspring with the disease (unless there is evidence of keratoconus in successive generations, which increases the probability level). Additionally, there may be links to the endocrine (hormonal) system in that keratoconus tends to appear in its early stages at puberty.

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Associations with allergy (“atopy”) are common as well. Currently there are a number of studies going on in terms of determining the underlying causes and associations of and with keratoconus.

Although the exact prevalence of keratoconus is not known, a classic statistic often quoted is 1 in 2,000 persons in the general population suffer from the disease. This statistic is based on a very old study that utilized outmoded diagnostic techniques. Many new studies that utilize modern and more sensitive diagnostic technologies are finding that the actual prevalence of keratoconus may be as common as 1 in 375 individuals! Dr. Eiden and other keratoconus experts are involved in a research project that is looking at prevalence rates of keratoconus in a pediatric population. Initial results are showing very high frequencies. Since we now have treatments that can stop the progression of keratoconus (such as corneal cross linking) it is critically important to make a diagnosis as early as possible in order to preserve vision. At NSVC we screen for keratoconus regularly as part of comprehensive eye examinations.

Diagnosing Keratoconus

With new technologies that measure the shape and thickness of the cornea in exquisite detail, we are now able to detect the presence of keratoconus well before subjective symptoms develop. Corneal Topography has become the standard of care in diagnosing keratoconus. A computerized system images the shape of the cornea by taking tens of thousands of data points from the corneal surface. The results are instantly analyzed and a topography map is generated. The colors of the map correspond to the shape of the cornea. In keratoconus the cornea tends to be very steep in shape and quite irregular in shape.

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Quantitatively the topography values can be monitored for change over time to determine if the condition is progressive. NSVC is proud to use the most advanced corneal topography and analysis system in the world today, Pentacam Corneal Analysis.

Pentacam corneal analysis differs from all other topography systems in that it is the only system not only to measure the front surface of the cornea, but it also measures the back surface of the cornea, the thickness of the cornea, and images and analyzes the anterior ocular structures as well. This is very critical in keratoconus since we have discovered that the back surface of the cornea is affected earlier and to a greater degree than the front corneal surface is in keratoconus! Thickness variations from normal are also early hints in terms of the presence of keratoconus and in the determination of progression in keratoconus.

Early phases of keratoconus will typically have abnormalities found with Pentacam and topography, yet have no other detectable clinical signs or subjective patient symptoms. As keratoconus develops and progresses, clinical signs and subjective symptoms will eventually show up. At some point in the disease process your doctor will note visible thinning of the cornea under the biomicroscope along with folds in the cornea (called “striae”). Other clinical signs include the deposition of iron in the cornea (“Fleisher’s Rings”), corneal scares, large increases in astigmatism, and reduced correctable vision with glasses. Patients complain of distortion and progressive blur of their vision along with large and frequent changes in their prescription.

Treatment for Keratoconus

During the very early stages of keratoconus, conventional eyeglasses can correct the mild myopia (nearsightedness), and astigmatism that develops. At these stages vision is often correctable to 20/20. As the disease progresses a loss of best corrected glasses vision occurs. It is then that specialty keratoconus contact lenses are required to achieve clear vision. The contact lenses function to “mask” the irregularity of the corneal surface, thus increasing the quality of vision in comparison to the vision through glasses. For many years the only method to correct vision well in keratoconus was with the use of hard contact lenses and then eventually rigid gas permeable contact lenses.

Today gas permeable contact lens correction is still the main stay of keratoconus treatment, however very specialized designs have been developed that improve vision and comfort far beyond yesterdays lenses. In addition, customized soft lenses, hybrid, and sclera contact lenses can now be designed that can also provide excellent visual results in many cases of keratoconus. Some of the highly customized designs used by your contact lens specialists at NSVC include among many others:

EyePrint Pro
KBA bi-aspheric (gas perm)
Rose-K designs (gas perm)
IKone bi-aspheric (gas perm)
McGuire designs (gas perm)
Soper designs (gas perm)
HydraKone (soft lens)
AlterKone (soft lens)
Synergeyes Clear Kone and SynergEyes KC (hybrid soft and gas perm combination)
Jupiter Scleral (sclera gas perm)
MSD (mini sclera gas perm)
Macrolens (corneal-scleral gas perm)
Intra-Limbal design (large diameter gas perm)
“Piggy-back” lens systems (combinations of soft disposable lenses and gas permeable lenses fit over the soft lens for improved comfort and fitting)

Scleral Contact Lenses for Keratoconus

NSVC is one of the few specialty contact lens practices to have extensive experience in fitting sclera contact lenses for the management of keratoconus and other medical eye conditions that require contact lenses. Scleral contact lenses are large diameter gas permeable lenses that vault the cornea and align to the peripheral sclera of the eye. Since there are few nerve endings on the sclera, the lenses are very comfortable (even from the initial time they are placed on the eye). The vaulting of the cornea leaves a healthy tear layer between the back of the lens and the front of the cornea. This prevents any lens induced irritation or discomfort on the corneal surface and promotes excellent “wetting” of the front of the eye. These lenses are custom fit to the eye based on the anterior shape of the eye (determined at NSVC by corneal shape analysis obtained via Pentacam technology). We utilize numerous sclera lens designs and laboratories which are selected based on the specific requirements of the individual patient.

SynergEyes “UltraHealth” lens for Keratoconus

SynergEyes laboratories specialize in the development of hybrid contact lenses that have a highly oxygen permeable central area and a soft periphery. The goal is to provide the crisp optics of a rigid gas permeable lens and the comfort and positioning stability similar to a soft contact lens. The most recent development in hybrid designs is the UltraHealth. UltraHealth has unique geometry of both the rigid center and soft periphery. The result is a lens that vaults the central cornea and aligns with the soft periphery from the peripheral cornea out to the sclera. The lens has excellent centering, movement, and comfort characteristics. NSVC was involved in the initial pre-market evaluation of this exciting new lens design. Our results have been very positive. We have been able to fit patients with keratoconus who have failed at many other lens designs.

NSVC Keratoconus Diagnosis and Management Expertise

NSVC’s specialty contact lens service has extensive experience in the management of the most challenging contact lens cases. Dr. S. Barry Eiden is a co-investigator for the Collaborative Longitudinal Evaluation of Keratoconus (“CLEK”) study. In 2022 he was awarded the “Keratoconus Practitioner of the Year” from the National Keratoconus Foundation and the GPLI.

The CLEK Study

The NSVC doctors are nationally renowned for contact lens management for keratoconus patients. Their experience has allowed thousands of keratoconic patients to achieve clear vision, when less experienced lens fitters had failed. NSVC receives referrals for the treatment of keratoconic patients from throughout the United States. Dr. S. Barry Eiden is a co-investigator on the Collaborative Longitudinal Evaluation of Keratoconus (“CLEK”) study.

CLEK is the Collaborative Longitudinal Evaluation of Keratoconus (“CLEK”) study. CLEK is the largest most comprehensive study of keratoconus ever performed. Dr. S. Barry Eiden of NSVC is a co-investigator on the study.

CLEK is the largest, most comprehensive study of keratoconus ever performed. It is an ongoing multi-center study funded by the National Institute of Health and the National Eye Institute. Most of the study sites are affiliated with major university medical centers. NSVC’s study site (one of 14 in the U.S.) is at the University of Illinois at Chicago, where Dr. Eiden is an assistant clinical professor in the department of ophthalmology, cornea and contact lenses service.

The goal of CLEK is to study the various aspects of keratoconus and natural progression of the disease. In addition, certain aspects of contact lens fitting for keratoconus are being studied. Future phases of the study will evaluate various contact lens fitting methods for keratoconus and their outcome in terms of vision and eye health response. Click here to visit the study website.

NSVC’s doctors are also consultants to the National Keratoconus Foundation which serves to support research and education relating to the disease.

Surgical Treatments for Keratoconus

Cornea Transplant Less than 10% of patients with keratoconus progress to the point where contact lens treatment is insufficient to correct vision adequately or contact lens intolerance develop. These patients require some form of corneal transplantation. Eye banks in most major cities have a great supply of corneas for transplantation, so waiting on a recipient list is generally a thing of the past. Corneal transplantation is one of the most successful forms of organ transplantation. Rejection of corneal tissue is relatively infrequent and is very successfully managed with topical medication eye drops. The outcome for corneal transplantation in cases of keratoconus is very good. Following corneal transplants, most patients do require glasses and often contact lenses for proper visual correction.

NSVC is affiliated with a number of the most highly respected corneal transplant surgeons in the country. They are on staff at respected institutions such as Northwestern University Medical Center, the University of Illinois Eye and Ear Infirmary, Rush University Medical Center, and North Shore University Medical Center. Should any of our keratoconus require corneal transplantation, your doctor will make the most appropriate referral for you.

INTACS for Keratoconus are small arc shape implants that can be surgically placed within the corneal tissue to effect a purposeful change in the surface shape of the cornea. Initially developed for the correction of myopia (nearsightedness), doctors began to utilize Intacs for certain cases of keratoconus to provide a relatively more regular front surface. This allows for some degree of improvement in vision and in some instances, more stable contact lens fitting. There are certain specific criteria that make a patient with keratoconus a candidate for Intacs. Your keratoconus specialist at NSVC is able to make that determination. We also work with some of the most experienced Intacs surgeons in the world and as such will make the appropriate referral when indicated.

Corneal Cross Linking (CXL) For Keratoconus

New research indicates that keratoconus may be treated with a procedure called Corneal Cross-linking (CXL). This treatment increases the stiffness and rigidity of the cornea and stabilizes ectasia. Some patients who previously had progressive keratoconus have now been treated and followed for years without evidence of any further change in their condition. Over 95% of patients treated with CXL show no signs of disease progression and in fact a significant percentage of cases may show some degree of improvement (or reduction is disease severity), although it must be understood that the primary goal of treatment is to halt progression of keratoconus. The bulk of the cornea is made from collagen fibers which are arranged in bundles. The strength and rigidity of the cornea is partly determined by how strongly the fibers are linked together. Over the course of a lifetime the cornea becomes progressively stiffer due to natural cross-linking between the fibers. As such, CXL is indicated for patients who have demonstrated progression or are at high risk for progression. As one ages the risk of keratoconus progression decreases. At NSVC we strongly suggest CXL treatment for younger persons with keratoconus who are at high risk for progression. For older individuals with keratoconus we typically monitor the disease and if we document progression, we would perform CXL.

Riboflavin (vitamin B2) is a naturally occurring compound which strongly absorbs UV light. By applying riboflavin to the cornea at the same time as exposing it to a UV light source, the riboflavin not only enhances the cross-linking effect of the UV light, but also absorbs the light to an extent that the inner layers of the cornea and intra-ocular structures are protected from the potentially damaging effects of the UV light rays. There are two basic methods to perform CXL. The original procedure is called epithelium off CXL (Epi-off). This involves removal of the surface cells of the cornea prior to applying riboflavin and UV light. This method (often referred to as the “Dresden protocol” since the treatment was developed at the University of Dresden in Germany over 20 years ago) has been shown to be highly effective in halting keratoconus progression and is currently the only FDA approved method of CXL in the United States. More and more medical insurance carriers are covering this form of CXL. There are potential downsides to epi-off CXL that include; significant post treatment discomfort, slow vision recuperation, delayed time to get back into contact lenses and greater potential for complications such as post treatment infection and/or inflammation (although the likelihood is very low). A second method of CXL has been developed and performed over the past 10 years. This is called transepithelial CXL or “Epi-on CXL”. Due to the development of riboflavin formulations that can penetrate an intact corneal surface epithelium, this method has been developed and is showing in many studies equal efficacy to epi-off CXL. The advantage of epi-on CXL is almost no discomfort, rapid vision recovery, quick return to contact lens wear and a significantly lower chance for treatment complications. We are currently waiting on FDA approval for Epi-on CXL as they analyze outcomes of clinical trials. NSVC is involved in CXL and we manage CXL through our affiliated keratoconus specialty practice Keratoconus Specialists of Illinois (KSI). Our results over the past number of years have been excellent.