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By: Marika Mohammed
 Progressive, non-imflammatory ectatic disorder of the
cornea
 Usually bilateral but asymmetric
 Paraxial stromal thinning and weakening leading to
corneal surface distortion
 Primary- irregular astigmatism
- myopia
 Secondary- corneal scarring
 Presents at puberty or early adulthood
 50-230 per 100,000
 Equal prevalence in both sexes and all races
 Generally unknown, likely multifactorial
 Suspected:
 Family history in 6-8% of cases
 x15-67 higher incidence if first degree relative
 Eye rubbing
 Contact lens use
 Systemic disorders eg. Downs Syndrome, Ehlers-Danlos,
Osteogenesis Imperfecta
 All layers of the cornea believed to be affected
 Epithelial cells may be enlarged and elongated
 Early degeneration of basal epithelial cells
 Disruption of basement membrane
 Growth of epithelium posterior to Bowman’s layer
forming z-shaped interruptions or breaks
 Scarring of Bowman’s layer and anterior stroma
 Stromal thinning due to normal-sized fibres but low
numbers of llamelae
Symptoms:
 Progression until 4th decade
 Asymmetric visual complaints
 Blur and distortions
 Glare/flare
 Monocular diplopia
 Photophobia
 Initial correction by spectacles then soft contact lenses
Signs:
 Slit lamp:
 Fleisher ring: Iron deposits in epithelial layer at cone base
 Vogt striae: Vertical stress lines at thinnest part of cornea
 Central and inferior paracentral corneal thinning
 Corneal scarring
 Scissor reflex on retinoscopy due to irregular astigmatism
 Rizzutti’s sign: conical reflection on the nasal cornea
when light is shone temporally
 Munson’s sign: corneal protrusion may cause angulation
of the lower lid on downgaze (advanced)
 Corneal Hydrops: stromal edema due to leakage of
aqueous through a tear in descemet membrane
Vogt Striae
Corneal HydropsMunson’s Sign
 Complete history and clinical examination
 Visual acuity testing
 Slit lamp examination
 Retinoscopy- for scissoring reflex
 Keratometry- may demonstrate irregular mires and
progressive corneal steepening
 Diagnostic rigid contact lenses
 Corneal Topography
 Maps the corneal curvature
 Indicates any distortions or scarring
 Common characteristics:
 Asymmetrical bowtie
 Inferior corneal steepening
 Skewed radia axes
 K value – Measures central steepening of the cornea; ≥
47.20 D suggests keratoconus
 I-S value – Measures inferior-versus-superior corneal
dioptric asymmetry; ≥ 1.4 D suggests keratoconus
 KISA% - Incorporates K and I-S values quantifying
regular and irregular astigmatism into a single index;
60-100% suggests keratoconus, ≥ 100% strongly
suggests frank keratoconus
Non-Surgical:
 Spectacle correction- early, as long as visual acuity
allows
 Contact lens-
 With progressive astigmatism
 Soft-tonic initially
 Rigid gas-permeable lenses most common
 Until corneal irregularity becomes too advanced
 Collagen cross-linking
Surgical:
 Intrastromal corneal ring segments:
 thin, semi-circular plastic inserts implanted into the
mid-corneal layers to flatten the cornea
 Keratoplasty – 10-15% patients
 penetrating keratoplasty (full thickness corneal
transplant) : most common
 Deep anterior lamellar keratoplasty (partial thickness
corneal transplant)
Thank you!

 References
 1. Espandar L, Meyer J. Keratoconus: Overview and Update on Treatment.
Middle East Afr J Ophthalmol [Internet]. 2010 [cited 9 January 2015];. Available
from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2880369/
 2. Wayman L, Trobe J, Park L. Keratoconus. [Internet]. 2014 [cited 9 January
2015];. Available from:
http://www.uptodate.com.ezproxy.sastudents.uwi.tt:2048/contents/keratocon
us?source=search_result&search=keratoconus&selectedTitle=1~13
 3. Weissman B, Roy H. Keratoconus [Internet]. Medscape. 2014 [cited 9 January
2015]. Available from: http://emedicine.medscape.com/article/1194693-
overview#showall
 4. Romero-Jiménez M M, Santodomingo-Rubido J, Wolffsohn J. Keratoconus: a
review. Elsevier [Internet]. 2010 [cited 9 January 2015];. Available from:
http://www.ncbi.nlm.nih.gov/pubmed/20537579
 5. OphthaClass. Amsler-Krumeich Classification for Grading Keratoconus -
OphthaClass [Internet]. 2015 [cited 9 January 2015]. Available from:
http://ophthaclassification.altervista.org/krumeichclass/
 6. Sinjab M. Quick Guide to the Management of Keratoconus A Systematic
Step-by-Step Approach. New York: Springer; 2012.

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Keratoconus

  • 2.  Progressive, non-imflammatory ectatic disorder of the cornea  Usually bilateral but asymmetric  Paraxial stromal thinning and weakening leading to corneal surface distortion
  • 3.
  • 4.  Primary- irregular astigmatism - myopia  Secondary- corneal scarring
  • 5.  Presents at puberty or early adulthood  50-230 per 100,000  Equal prevalence in both sexes and all races
  • 6.  Generally unknown, likely multifactorial  Suspected:  Family history in 6-8% of cases  x15-67 higher incidence if first degree relative  Eye rubbing  Contact lens use  Systemic disorders eg. Downs Syndrome, Ehlers-Danlos, Osteogenesis Imperfecta
  • 7.  All layers of the cornea believed to be affected  Epithelial cells may be enlarged and elongated  Early degeneration of basal epithelial cells  Disruption of basement membrane
  • 8.  Growth of epithelium posterior to Bowman’s layer forming z-shaped interruptions or breaks  Scarring of Bowman’s layer and anterior stroma  Stromal thinning due to normal-sized fibres but low numbers of llamelae
  • 9. Symptoms:  Progression until 4th decade  Asymmetric visual complaints  Blur and distortions  Glare/flare  Monocular diplopia  Photophobia  Initial correction by spectacles then soft contact lenses
  • 10. Signs:  Slit lamp:  Fleisher ring: Iron deposits in epithelial layer at cone base  Vogt striae: Vertical stress lines at thinnest part of cornea  Central and inferior paracentral corneal thinning  Corneal scarring
  • 11.  Scissor reflex on retinoscopy due to irregular astigmatism  Rizzutti’s sign: conical reflection on the nasal cornea when light is shone temporally  Munson’s sign: corneal protrusion may cause angulation of the lower lid on downgaze (advanced)  Corneal Hydrops: stromal edema due to leakage of aqueous through a tear in descemet membrane
  • 13.  Complete history and clinical examination  Visual acuity testing  Slit lamp examination  Retinoscopy- for scissoring reflex  Keratometry- may demonstrate irregular mires and progressive corneal steepening  Diagnostic rigid contact lenses  Corneal Topography
  • 14.  Maps the corneal curvature  Indicates any distortions or scarring  Common characteristics:  Asymmetrical bowtie  Inferior corneal steepening  Skewed radia axes
  • 15.  K value – Measures central steepening of the cornea; ≥ 47.20 D suggests keratoconus  I-S value – Measures inferior-versus-superior corneal dioptric asymmetry; ≥ 1.4 D suggests keratoconus  KISA% - Incorporates K and I-S values quantifying regular and irregular astigmatism into a single index; 60-100% suggests keratoconus, ≥ 100% strongly suggests frank keratoconus
  • 16.
  • 17.
  • 18. Non-Surgical:  Spectacle correction- early, as long as visual acuity allows  Contact lens-  With progressive astigmatism  Soft-tonic initially  Rigid gas-permeable lenses most common  Until corneal irregularity becomes too advanced  Collagen cross-linking
  • 19. Surgical:  Intrastromal corneal ring segments:  thin, semi-circular plastic inserts implanted into the mid-corneal layers to flatten the cornea  Keratoplasty – 10-15% patients  penetrating keratoplasty (full thickness corneal transplant) : most common  Deep anterior lamellar keratoplasty (partial thickness corneal transplant)
  • 21.  References  1. Espandar L, Meyer J. Keratoconus: Overview and Update on Treatment. Middle East Afr J Ophthalmol [Internet]. 2010 [cited 9 January 2015];. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2880369/  2. Wayman L, Trobe J, Park L. Keratoconus. [Internet]. 2014 [cited 9 January 2015];. Available from: http://www.uptodate.com.ezproxy.sastudents.uwi.tt:2048/contents/keratocon us?source=search_result&search=keratoconus&selectedTitle=1~13  3. Weissman B, Roy H. Keratoconus [Internet]. Medscape. 2014 [cited 9 January 2015]. Available from: http://emedicine.medscape.com/article/1194693- overview#showall  4. Romero-Jiménez M M, Santodomingo-Rubido J, Wolffsohn J. Keratoconus: a review. Elsevier [Internet]. 2010 [cited 9 January 2015];. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20537579  5. OphthaClass. Amsler-Krumeich Classification for Grading Keratoconus - OphthaClass [Internet]. 2015 [cited 9 January 2015]. Available from: http://ophthaclassification.altervista.org/krumeichclass/  6. Sinjab M. Quick Guide to the Management of Keratoconus A Systematic Step-by-Step Approach. New York: Springer; 2012.