Updated: Jan 8, 2007
Keratoconus (KC) is a progressive, noninflammatory, bilateral (but usually asymmetrical) disease of the cornea, characterized by paraxial stromal thinning that leads to corneal surface distortion. Visual loss occurs primarily from irregular astigmatism and myopia and secondarily from corneal scarring.
All layers of the cornea are believed to be affected by KC, although the most notable features are the thinning of the corneal stroma, the ruptures in the Bowman layer, and the deposition of iron in the basal epithelial cells, forming the Fleischer ring. Breaks in and folds close to the Descemet membrane result in acute hydrops and striae, respectively.
Reported prevalence in the general population varies (50-200 per 100,000), perhaps with differences in diagnostic criteria. It is commonly an isolated ocular condition but sometimes coexists with other ocular and systemic diseases.
Commonly recognized ocular associations include vernal keratoconjunctivitis, retinitis pigmentosa, and Leber congenital amaurosis; systemic putative associations include many of the connective tissue disorders (eg, Ehlers-Danlos and Marfan syndromes), mitral valve prolapse, atopic dermatitis, and Down syndrome.
Particular risk factors include atopic history, especially ocular allergies, rigid contact lens (CL) wear, and vigorous eye rubbing.
Most KC cases appear spontaneously, although approximately 14% of them present with evidence of genetic transmission.
Anecdotal reports suggest an increase in prevalence in some parts of the world, Arabia, the Indian subcontinent, and New Zealand.
An equal incidence of KC occurs in males and females.
KC is divided into mild, moderate, and advanced.
Contact Lens Complications
Diplopia
Keratitis, Interstitial
Pellucid Marginal Degeneration
Consider other causes of irregular oblique astigmatism, imprecise variable subjective refractions and noninflammatory central corneal scarring, such as corneal surgery, healed infections, and trauma.
Stromal striations similar to Vogt striae occur when corneas swell (rather than thin) from either hypoxia (associated with wear of contact lenses) or with early Fuchs corneal dystrophy.
Iron depositions in the basal epithelium (similar to a Fleischer ring) occur in any area of relative depression in the corneal surface in association with pterygium, surgical scars, and central depressions from refractive surgery.
Pellucid marginal degeneration (PMD) is often considered a variant of KC, in which corneal thinning occurs about a millimeter above the inferior limbus, resulting in advanced against-the-rule corneal astigmatism that may be observed by keratometry or videokeratography.
Terrien marginal corneal degeneration is a mildly inflammatory disease, usually of the superior limbus, which also can induce irregular against-the-rule astigmatism and corneal thinning (often with vascularization and lipid deposits).
Keratoglobus (KG) is an extremely rare corneal disease in which the entire cornea, from limbus to limbus, thins, sometimes to the point where spontaneous perforation becomes possible. KG probably is unrelated to KC.
Posterior keratoconus is another extremely rare disease in which the posterior corneal surface suffers a loss of substance. This condition is probably unrelated to KC.
All layers of the cornea are affected by KC. Superficial epithelial cells located at the nodule are elongated and arranged in a whorl-like fashion. Iron deposition in the basal corneal epithelial cells form the characteristic Fleischer ring. Localized breaks are present in the basement membrane. A decrease in the number of stromal collagen lamellae is present, as well as a loss of the fibular arrangement within the lamellae. Folds and ruptures occur in the Descemet membrane. Some studies have reported endothelial cell loss in association with the rupture of the Descemet membrane.
Patients should avoid (vigorous) eye rubbing.
No direct pharmacological management of KC is available, although nonsteroidal anti-inflammatory (NSAID), antihistamine, or mast cell stabilizing topical medications are occasionally helpful in controlling the often concomitant signs of ocular allergies, especially pruritus, that can lead to eye rubbing.
Episodes of hydrops may require treatment with hyperosmotics to reduce corneal swelling or topical steroid drops to reduce inflammation. Topical antibiotics are used for suspected infection.
Used to manage signs and symptoms of long-term ocular allergies, which can lead to patient discomfort and increased vigorous eye rubbing.
Mast cell stabilizers are effective to treat long-term ocular allergies, specifically giant papillary conjunctivitis, which can accompany contact lens wear (especially in keratoconus). This is not effective for acute allergies.
1 gtt in each eye qid for 1 wk, then bid prn
<3 years: Not established
>3 years: Administer as in adults
None reported
Documented hypersensitivity
B - Usually safe but benefits must outweigh the risks.
Drops should be used prior to and following contact lens wear; most frequently reported adverse reactions include ocular stinging upon instillation
Inhibitor of histamine release from mast cell and devoid of effects on serotonin, alpha-adrenergic, muscarinic, and dopamine receptors.
1 gtt in affected eye(s) bid
<3 years: Not established
>3 years: Administer as in adults
None reported
Documented hypersensitivity
C - Safety for use during pregnancy has not been established.
Do not use while wearing contact lenses; not for injection
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
Site-specific steroid for acute severe allergic reactions.
1-2 gtt in affected eye(s) qid
Not established
Ophthalmic NSAIDs combined with corticosteroids may delay or slow ocular wound healing; adverse reactions can include dry eyes, watery eyes, foreign body sensation, itchy eyes, photophobia, headache, rhinitis, and pharyngitis
Documented hypersensitivity; contact lens wear; glaucoma; ocular infections (viral, fungal, bacterial); corneal epithelial defects
C - Safety for use during pregnancy has not been established.
Caution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate)
Reduce symptoms of itching that can lead to eye rubbing, thereby decreasing eye rubbing both in duration and intensity.
Relatively selective H1-receptor antagonist.
1 gtt in affected eye(s) qid
<3 years: Not established
>3 years: Administer as in adults
None reported
Documented hypersensitivity
C - Safety for use during pregnancy has not been established.
Adverse reactions include headaches.
May reduce inflammation in cornea by creating an osmotic gradient across an intact blood barrier.
Used for temporary relief of corneal edema.
Solution: 1-2 gtt in affected eye(s) q3-4h or as directed
Ointment: Apply 0.25-inch ribbon of ointment inside lower lid q3-4h or as directed
Not established
None reported
Documented hypersensitivity
A - Safe in pregnancy
May cause temporary burning and irritation upon use; if pain, change in vision, continued redness or irritation of the eye(s) occurs, or if initial condition/problem worsens or persists, reevaluate therapy; do not use product if it changes color or becomes cloudy
Boxer Wachler BS, Christie JP, Chandra NS, et al. Intacs for keratoconus. Ophthalmology. May 2003;110(5):1031-40. [Medline].
Karseras AG, Ruben M. Aetiology of keratoconus. Br J Ophthalmol. Jul 1976;60(7):522-5. [Medline].
Kennedy RH, Bourne WM, Dyer JA. A 48-year clinical and epidemiologic study of keratoconus. Am J Ophthalmol. Mar 15 1986;101(3):267-73. [Medline].
Korb DR, Finnemore VM, Herman JP. Apical changes and scarring in keratoconus as related to contact lens fitting techniques. J Am Optom Assoc. Mar 1982;53(3):199-205. [Medline].
Krachmer JH, Feder RS, Belin MW. Keratoconus and related noninflammatory corneal thinning disorders. Surv Ophthalmol. Jan-Feb 1984;28(4):293-322. [Medline].
Lass JH, Lembach RG, Park SB, et al. Clinical management of keratoconus. A multicenter analysis. Ophthalmology. Apr 1990;97(4):433-45. [Medline].
Macsai MS, Varley GA, Krachmer JH. Development of keratoconus after contact lens wear. Patient characteristics. Arch Ophthalmol. Apr 1990;108(4):534-8. [Medline].
Maeda N, Klyce SD, Smolek MK. Comparison of methods for detecting keratoconus using videokeratography. Arch Ophthalmol. Jul 1995;113(7):870-4. [Medline].
Maguire LJ, Bourne WM. Corneal topography of early keratoconus. Am J Ophthalmol. Aug 15 1989;108(2):107-12. [Medline].
Moodaley L, Buckley RJ, Woodward EG. Surgery to improve contact lens wear in keratoconus. CLAO J. Apr 1991;17(2):129-31. [Medline].
Moodaley LC, Woodward EG, Liu CS, Buckley RJ. Life expectancy in keratoconus. Br J Ophthalmol. Oct 1992;76(10):590-1. [Medline].
Rabinowitz YS. Keratoconus. Surv Ophthalmol. Jan-Feb 1998;42(4):297-319. [Medline].
Rabinowitz YS, Garbus J, McDonnell PJ. Computer-assisted corneal topography in family members of patients with keratoconus. Arch Ophthalmol. Mar 1990;108(3):365-71. [Medline].
Rabinowitz YS. The genetics of keratoconus. Ophthalmol Clin North Am. Dec 2003;16(4):607-20, vii. [Medline].
Sherwin T, Brookes NH. Morphological changes in keratoconus: pathology or pathogenesis. Clin Experiment Ophthalmol. Apr 2004;32(2):211-7. [Medline].
Vogt A. Reflexlinien durch faltung spiegelnder grenzflachen im bereiche von corneo, linsenkapsel und netzhaut. Albrecht von Graefes Arch Ophthalmol. 1919;99:296-338.
Wilson SE, Lin DT, Klyce SD. Corneal topography of keratoconus. Cornea. Jan 1991;10(1):2-8. [Medline].
Wollensak G. Crosslinking treatment of progressive keratoconus: new hope. Curr Opin Ophthalmol. Aug 2006;17(4):356-60. [Medline].
Zadnik K, Barr JT, Gordon MO, Edrington TB. Biomicroscopic signs and disease severity in keratoconus. Collaborative Longitudinal Evaluation of Keratoconus (CLEK) Study Group. Cornea. Mar 1996;15(2):139-46. [Medline].
KC, corneal disease, stromal thinning, corneal scarring, irregular astigmatism, myopia, vision loss, Fleischer ring, Descemet membrane, intracorneal plastic rings, Intacs, corneal transplantation, penetrating keratoplasty, PKP, contact lens, contact lenses, CL, CL wear, contact lens wear, eye allergies, ocular allergies, eye rubbing
Barry A Weissman, OD, PhD, FAAO, Chief of Contact Lens Service, Professor, Department of Ophthalmology, Jules Stein Eye Institute, University of California at Los Angeles
Barry A Weissman, OD, PhD, FAAO is a member of the following medical societies: American Academy of Optometry and Phi Beta Kappa
Disclosure: Nothing to disclose.
Karen K Yeung, OD, FAAO, Director of Optometry, Arthur Ashe Student Health and Wellness Center, University of California at Los Angeles
Karen K Yeung, OD, FAAO is a member of the following medical societies: American Academy of Optometry
Disclosure: Nothing to disclose.
Fernando H Murillo-Lopez, MD, Senior Surgeon, Unidad Privada de Oftalmologia CEMES
Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.
Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.
Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Hospital
Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Eye Bank Association of America, Pennsylvania Medical Society, and Philadelphia County Medical Society
Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other
Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.
Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.
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