Keratoconus (KC, KTCN) is a degenerative disorder of the eye in which structural changes within the cornea cause it to thin and change to a more conical shape than the more normal gradual curve. Keratoconus can cause substantial distortion of vision, with multiple images, streaking and sensitivity to light all often reported by the person. It is typically diagnosed in the person’s adolescent years. If both eyes are significantly affected, the deterioration in vision can affect the person’s ability to drive a car or read normal print.
In most cases, corrective lenses fitted by a specialist are effective enough to allow the person to continue to drive legally and likewise function normally. Further progression of the disease may require surgery, for which several options are available, including intrastromal corneal ring segments, corneal collagen cross-linking, mini asymmetric radial keratotomy, corneal intrastromal implantation system (CISIS), topography-guided photorefractive keratectomy (PRK), topography-guided conductive keratoplasty, phakic intraocular lenses and, in 25% of cases, corneal transplantation.
Estimates of the rates for keratoconus range from 1 in 500 to 1 in 2000 people, but difficulties with differential diagnosiscause uncertainty. It seems to occur in populations throughout the world, although it is observed more frequently in certain ethnic groups, such as South Asians. Environmental and genetic factors are considered possible causes, but the exact cause is uncertain. It has been associated with detrimental enzyme activity within the cornea. The word is from Greek: kérashorn, cornea; and Latin: cōnus cone.
Keratoconus Signs and Symptoms
People with early keratoconus typically notice a minor blurring of their vision and come to their clinician seeking corrective lenses for reading or driving. At early stages, the symptoms of keratoconus may be no different from those of any other refractive defect of the eye. As the disease progresses, vision deteriorates, sometimes rapidly. Visual acuity becomes impaired at all distances, and night vision is often poor. Some individuals have vision in one eye that is markedly worse than that in the other. The disease is often bilateral, though asymmetrical. Some develop photophobia (sensitivity to bright light), eye strain from squinting in order to read, or itching in the eye,but there is normally little or no sensation of pain. It may cause luminous objects to appear as cylindrical pipes with the same intensity at all points.
The classic symptom of keratoconus is the perception of multiple “ghost” images, known as monocular polyopia. This effect is most clearly seen with a high contrast field, such as a point of light on a dark background. Instead of seeing just one point, a person with keratoconus sees many images of the point, spread out in a chaotic pattern. This pattern does not typically change from day to day, but over time, it often takes on new forms. People also commonly notice streaking and flaring distortion around light sources. Some even notice the images moving relative to one another in time with their heart beat. The predominant optical aberration of the eye in keratoconus is coma. The visual distortion experienced by the person comes from two sources, one being the irregular deformation of the surface of the cornea, and the other being scarring that occurs on its exposed highpoints. These factors act to form regions on the cornea that map an image to different locations on the retina. The effect can worsen in low light conditions, as the dark-adapted pupil dilates to expose more of the irregular surface of the cornea.
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