Keratoconus Treatment & Management

  • Author: Barry A Weissman, OD, PhD, FAAO; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Nov 23, 2011
 

Medical Care

Rigid contact lenses (CLs) are the mainstay of keratoconus (KC) treatment.

Patients with early keratoconus may successfully use spectacles or spherical/toric soft contact lenses. They may even rarely find that spectacle vision is superior to rigid contact lenses.

Patients with modest-to-advanced keratoconus almost always require rigid contact lenses. When rigid contact lenses are no longer tolerated, some patients can maintain contact lens wear and usable visions with hydrogel contact lenses, piggyback contact lenses, or scleral (haptic) contact lenses but usually at a physiological or visual cost.

Contact lens wear is often complicated by episodes of intolerance, allergic reactions (eg, giant papillary conjunctivitis), corneal abrasions,[30] neovascularization, and other problems, sometimes leading to total intolerance.

Though not FDA approved in the United States, corneal collagen cross-linking (CXL) is a procedure used to increase the rigidity of the cornea by inducing additional cross-links within or between collagen fibers using UVA light and a photomediator, riboflavin, with the goal of slowing down, possibly stabilizing, and even perhaps reversing, the progression of corneal ectasia in patients with keratoconus.[31, 32]

Long-term studies are still needed to determine the success and adverse effects of the procedure, as well as the long-term biomechanical effect of CXL. Currently, there is no effective way to measure collagen turnover and, hence, the stability of the collagen cross-links remains a concern.[33] More studies are also needed to identify high-risk patients perhaps related to their age, diagnosis, corneal shape, and/or stages of ectasia.

Next

Surgical Care

Surgically removing central nodular scars by shaving the corneal surface (superficial keratectomy with a blade or excimer laser phototherapeutic keratectomy) may improve contact lens tolerance, decrease the rate of associated corneal abrasions, and preclude the need for corneal transplant.

Intrastromal corneal rings (Intacs) have been implanted for patients who have become intolerant to contact lenses, but these have been found to be more successful in modest than advanced disease.[18, 19]

Lamellar keratoplasty, or various types, especially deep anterior lamellar keratoplasty (DLAK) with "big bubble" technique, is being reconsidered as a surgical treatment for keratoconus.[16, 17]

More traditionally, these patients, including those whose vision is not correctable to better than 20/40, are referred for corneal transplants penetrating keratoplasty (PKP). PKP is very successful in keratoconus, resulting in clear visual axes in greater than 90% of all cases. PKP for KC exhibits excellent visual and survival results, but young patients may require 1 or more grafts during their lifetime.[34] Contact lenses are often still required post graft for optimum vision.

PKP requires continuing professional care to watch for rejection, suture-related problems, wound dehiscence, and other difficulties.

Although extremely rare, keratoconus can recur in a graft.

Previous
Next

Consultations

Consult with a cornea specialist (a graduate of a cornea fellowship program) and/or contact lens specialist who provides appropriate (primarily rigid gas permeable [GP]) contact lens care.

An ophthalmologist who is a cornea specialist assists in identifying appropriate clinical conditions and timing for surgical intervention, such as superficial keratectomy, PKP, implantation of plastic corneal ring segments, or collagen crosslinking. Alternatively, topography-guided conductive keratoplasty has been shown to be modestly effective in reshaping the cornea in keratoconus eyes, at least temporarily.[35]

A specialty contact lens practitioner (usually an optometrist but can be an optician or ophthalmologist) monitors contact lens care to optimize vision while minimizing complications of contact lens wear. The practitioner also helps to establish the appropriate clinical conditions and timing of surgical intervention, should this become necessary.

Previous
Next

Activity

Patients should avoid (vigorous) eye rubbing.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Barry A Weissman, OD, PhD, FAAO  Chief of Contact Lens Service, Professor, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine; Adjunct Professor of Optometry, Southern California College of Optometry

Barry A Weissman, OD, PhD, FAAO is a member of the following medical societies: American Academy of Optometry, American Optometric Association, California Optometric Society, and International Society for Contact Lens Research

Disclosure: VSP None Speaking and teaching; Alcon None Speaking and teaching; Vistakon/The Vision Care Institute Grant/research funds support of Fellowship program

Coauthor(s)

Karen K Yeung, OD, FAAO  Director of Optometry, Arthur Ashe Student Health and Wellness Center, University of California, Los Angeles, David Geffen School of Medicine

Karen K Yeung, OD, FAAO is a member of the following medical societies: American Academy of Optometry

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Institute

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; RPS Ownership interest Other; EyeGate Pharma Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting; Merck Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

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.

References
  1. Gondhowiardjo TD, van Haeringen NJ. Corneal aldehyde dehydrogenase, glutathione reductase, and glutathione S-transferase in pathologic corneas. Cornea. Jul 1993;12(4):310-4. [Medline].

  2. Behndig A, Karlsson K, Johansson BO, Brannstrom T, Marklund SL. Superoxide dismutase isoenzymes in the normal and diseased human cornea. Invest Ophthalmol Vis Sci. Sep 2001;42(10):2293-6. [Medline].

  3. Buddi R, Lin B, Atilano SR, Zorapapel NC, Kenney MC, Brown DJ. Evidence of oxidative stress in human corneal diseases. J Histochem Cytochem. Mar 2002;50(3):341-51. [Medline].

  4. Atilano SR, Coskun P, Chwa M, et al. Accumulation of mitochondrial DNA damage in keratoconus corneas. Invest Ophthalmol Vis Sci. Apr 2005;46(4):1256-63. [Medline].

  5. 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].

  6. Udar N, Atilano SR, Brown DJ, et al. SOD1: a candidate gene for keratoconus. Invest Ophthalmol Vis Sci. Aug 2006;47(8):3345-51. [Medline].

  7. Lema I, Duran JA. Inflammatory molecules in the tears of patients with keratoconus. Ophthalmology. Apr 2005;112(4):654-9. [Medline].

  8. Lema I, Duran JA, Ruiz C, Diez-Feijoo E, Acera A, Merayo J. Inflammatory response to contact lenses in patients with keratoconus compared with myopic subjects. Cornea. Aug 2008;27(7):758-63. [Medline].

  9. Krachmer JH, Feder RS, Belin MW. Keratoconus and related noninflammatory corneal thinning disorders. Surv Ophthalmol. Jan-Feb 1984;28(4):293-322. [Medline].

  10. Rabinowitz YS. Keratoconus. Surv Ophthalmol. Jan-Feb 1998;42(4):297-319. [Medline].

  11. Macsai MS, Varley GA, Krachmer JH. Development of keratoconus after contact lens wear. Patient characteristics. Arch Ophthalmol. Apr 1990;108(4):534-8. [Medline].

  12. 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].

  13. Wagner H, Barr JT, Zadnik K. Collaborative Longitudinal Evaluation of Keratoconus (CLEK) Study: methods and findings to date. Cont Lens Anterior Eye. Sep 2007;30(4):223-32. [Medline].

  14. Moodaley LC, Woodward EG, Liu CS, Buckley RJ. Life expectancy in keratoconus. Br J Ophthalmol. Oct 1992;76(10):590-1. [Medline].

  15. Lass JH, Lembach RG, Park SB, et al. Clinical management of keratoconus. A multicenter analysis. Ophthalmology. Apr 1990;97(4):433-45. [Medline].

  16. Shimmura S, Tsubota K. Deep anterior lamellar keratoplasty. Curr Opin Ophthalmol. Aug 2006;17(4):349-55. [Medline].

  17. Fontana L, Parente G, Tassinari G. Clinical outcomes after deep anterior lamellar keratoplasty using the big-bubble technique in patients with keratoconus. Am J Ophthalmol. Jan 2007;143(1):117-124. [Medline].

  18. Colin J, Malet FJ. Intacs for the correction of keratoconus: two-year follow-up. J Cataract Refract Surg. Jan 2007;33(1):69-74. [Medline].

  19. Rabinowitz YS. Intacs for keratoconus. Curr Opin Ophthalmol. Jul 2007;18(4):279-83. [Medline].

  20. Riaskup-Wolf F, Hoyer A, Spoerl E, et al. Collagen crosslinking with riboflavin and ultra violet A light in keratoconus: long term results. J of Cataract and Refractive Surgery. 2008;34(5):796-801.

  21. Kymionis GD, Portaliou DM, Kounis GA, et al. Simultaneous Topography-Guided Photorefractive Keratectomy Followed by Corneal Collagen Cross-linking for Keratoconus. Am J Ophthalmol. Nov 2011;152(5):748-55. [Medline].

  22. Visser ES, Visser R, van Lier HJ, Otten HM. Modern scleral lenses part I: clinical features. Eye Contact Lens. Jan 2007;33(1):13-20. [Medline].

  23. Karseras AG, Ruben M. Aetiology of keratoconus. Br J Ophthalmol. Jul 1976;60(7):522-5. [Medline].

  24. 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].

  25. Maeda N, Klyce SD, Smolek MK. Comparison of methods for detecting keratoconus using videokeratography. Arch Ophthalmol. Jul 1995;113(7):870-4. [Medline].

  26. Alio JL, Shabeyek MH. Corneal higher order aberrations: a method to grade keratoconus. Journal of Refractive Surgery. 2006;22(6):539-45.

  27. Jafri B, Li X, Yang H, Rabinowitz YS. Higher order wavefront aberrations and topography in early and suspected keratoconus. J Refract Surg. Oct 2007;23(8):774-81. [Medline].

  28. Kosaki R, Maeda N, Bessho K, Hori Y, Nishida K, Suzaki A. Magnitude and orientation of Zernike terms in patients with keratoconus. Invest Ophthalmol Vis Sci. Jul 2007;48(7):3062-8. [Medline].

  29. Sorbara L, Dalton K. The use of video-keratoscopy in predicting contact lens parameters for keratoconic fitting. Cont Lens Anterior Eye. Feb 1 2010;[Medline].

  30. Weissman BA, Chun MW, Barnhart LA. Corneal abrasion associated with contact lens correction of keratoconus-a retrospective study. Optom Vis Sci. 1994;71(11):677-81.

  31. Spoerl E, Huhle M, Seiler T. Induction of cross-links in corneal tissue. Exp Eye Res. Jan 1998;66(1):97-103. [Medline].

  32. Raiskup F, Spoerl E. Corneal Cross-linking with Hypo-osmolar Riboflavin Solution in Thin Keratoconic Corneas. Am J Ophthalmol. Jul 2011;152(1):28-32.e1. [Medline].

  33. Wollensak G, Iomdina E. Biomechanical and histological changes after corneal crosslinking with and without epithelial debridement. J Cataract Refract Surg. Mar 2009;35(3):540-6. [Medline].

  34. Kelly TL, Williams KA, Coster DJ. Corneal Transplantation for Keratoconus: A Registry Study. Arch Ophthalmol. Jun 2011;129(6):691-697. [Medline].

  35. Kato N, Toda I, Kawakita T, Sakai C, Tsubota K. Topography-guided conductive keratoplasty: treatment for advanced keratoconus. Am J Ophthalmol. Oct 2010;150(4):481-489.e1. [Medline].

  36. Bartlett JD, Howes JF, Ghormley NR, et al. Safety and efficacy of loteprednol etabonate for treatment of papillae in contact lens-associated giant papillary conjunctivitis. Curr Eye Res. Apr 1993;12(4):313-21. [Medline].

  37. [Guideline] American Academy of Ophthalmology Refractive Management/Intervention Panel. Refractive errors & refractive surgery. San Francisco (CA): American Academy of Ophthalmology; 2007.

  38. Chwa M, Atilano SR, Hertzog D, Zheng H, Langberg J, Kim DW. Hypersensitive response to oxidative stress in keratoconus corneal fibroblasts. Invest Ophthalmol Vis Sci. Oct 2008;49(10):4361-9. [Medline].

  39. Kenney MC, Chwa M, Atilano SR, et al. Increased levels of catalase and cathepsin V/L2 but decreased TIMP-1 in keratoconus corneas: evidence that oxidative stress plays a role in this disorder. Invest Ophthalmol Vis Sci. Mar 2005;46(3):823-32. [Medline].

  40. 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].

  41. Kymes SM, Walline JJ, Zadnik K, Sterling J, Gordon MO. Changes in the quality-of-life of people with keratoconus. Am J Ophthalmol. Apr 2008;145(4):611-617. [Medline].

  42. Machkiewicz Z, Maatta M, Stenman M, et al. Colagenolytic proteinases in keratoconus. Cornea. 2006;25(5):603-10.

  43. Maguire LJ, Bourne WM. Corneal topography of early keratoconus. Am J Ophthalmol. Aug 15 1989;108(2):107-12. [Medline].

  44. McMahon TT, Edrington TB, Szczotka-Flynn L, et al. Longitudinal changes in corneal curvature in keratoconus. Cornea. Apr 2006;25(3):296-305. [Medline].

  45. Moodaley L, Buckley RJ, Woodward EG. Surgery to improve contact lens wear in keratoconus. CLAO J. Apr 1991;17(2):129-31. [Medline].

  46. Rabinowitz YS. The genetics of keratoconus. Ophthalmol Clin North Am. Dec 2003;16(4):607-20, vii. [Medline].

  47. Sherwin T, Brookes NH. Morphological changes in keratoconus: pathology or pathogenesis. Clin Experiment Ophthalmol. Apr 2004;32(2):211-7. [Medline].

  48. Vogt A. Reflexlinien durch faltung spiegelnder grenzflachen im bereiche von corneo, linsenkapsel und netzhaut. Albrecht von Graefes Arch Ophthalmol. 1919;99:296-338.

  49. Wilson SE, Lin DT, Klyce SD. Corneal topography of keratoconus. Cornea. Jan 1991;10(1):2-8. [Medline].

  50. Wollensak G. Crosslinking treatment of progressive keratoconus: new hope. Curr Opin Ophthalmol. Aug 2006;17(4):356-60. [Medline].

  51. Zadnik K, Barr JT, Steger-May K, et al. Comparison of flat and steep rigid contact lens fitting methods in keratoconus. Optom Vis Sci. Dec 2005;82(12):1014-21. [Medline].

Previous
Next
 
An optic section of a keratoconic cornea shows corneal thinning. Vogt striae and some scarring can also be seen centrally; superiorly, a small (brown) section of the Fleischer ring is noted.
The fluorescein pattern of a rather flat-fitted rigid contact lens on an advanced keratoconic cornea.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.