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Available Options For
Keratoconus Management
BY
Dr. Amr Mounir , Lecturer of Ophthalmology
Sohag university
Definition
Keratoconus is a
progressive,
noninflammatory,
bilateral (but usually
asymmetric) ectatic
corneal disease,
characterized by
paraxial stromal
thinning and
weakening that leads
to corneal surface
distortion..
Etiology
-History of trauma that causes
weakness
-Recurrent trauma due to rubbing
from
Blepharitis, Vernal keratoconjuctivitis
-Inflammatory component!!!
Decrease proteinase inhibitors
Increase collagenase
Premature keratocytic apoptosis
Increase cytokine binding
Pathology
--Epithelial
basement
membrane
fragmentation and
scarring
--Axial stromal
thinning and
scarring
-Breaks in and folds
close to the
Descemet
membrane result in
acute hydrops
Clinical Picture
Patients with keratoconus (KC) often
report decreasing vision (distortions,
glare/flare, and monocular diplopia or
ghost images), with multiple
unsatisfactory attempts in obtaining
optimum spectacle correction
Signs
-Decrease in visual acuity
-Progressive myobia ,irrigular
Astigmatism
-Oil droplet sign by direct
ophthalmoscope - irregular
scissoring by retinoscopy
Slitlamp findings
FLEISCHER RING abrupt change in curvature
VOGT’S STRIAE 1st
Sign
STROMAL THINNING
STROMAL SCARS
ENLARGED CORNEAL NERVES
ACUTE HYDROPS
FLEISCHER RING
VOGT’S STRIAE
Healed aCUTE HYDROPS
Investigations
1-Old methods:
A- Placido Disc
B- Retinoscopy
2-New Methods:
A- Corneal topography
B- Pentacam
What Pentacam says to us???
1-Pachymetry map
2-Keratometry map ( K1,K2,Kmax(
3-Cone site and shape
4-Elevation map
5-Provisional diagnosis: Keratoconus
Summary ( Sirus device(
6-Aberration map
7-Scheimpflug imaging
Keratoconus summary
Scheimpflug imaging
Classifications of
keratoconus
Several classifications of keratoconus
based on:
1-Morphological Patterns
2-Topographical Patterns
Morphological Patterns
Nipple cone
Oval cone
Morphological Patterns
Topographical Patterns
Krumeich Classification of
Keratoconus:
Severity of KC is also classified by Krumeich.
This classification depends on mean K-
readings on the anterior curvature sagittal
map, thickness at the thinnest location, and
the refractive error of the patient.
Forme Fruste Keratoconus
Forme Fruste Keratoconus (FFKC(:
is a subclinical disease and is not a
variant of KC. Although clinicians use
many other terms such as mild KC,
early KC, and subclinical KC
Recently, there are two opinions
regarding the definition of this disease:
1.FFKC is a completely normal cornea
with neither clinical nor topographical
risk factors, but this cornea is able to
develop KC when treated by laser.
The fellow eye may be keratoconic or
there may be a family history of KC
2.FFKC is an abnormal cornea. Corneal
topography or corneal hysteresis or
both are abnormal; i.e., there are risk
factors but the case is still not a
clinically obvious KC.
Another Examble
Pellucid Marginal Degeneration (PMD)
and Pellucid-like Keratoconus
-PMD is a bilateral, non-inflammatory,
peripheral corneal thinning disorder
characterized by a peripheral band of
thinning of the inferior cornea. The
cornea in and adjacent to the thinned
area is ectatic.
-Patients usually are aged 20–40 years
at the time of clinical presentation.
Keratoglobus
Options For Treatment
Options For Treatment
Depend on:
1-Age
2-Refraction
3-Pachymetry
4-Keratometry
5-Cone Position
6-Corneal Opacifications.
Options
1-Glasses and follow up.
2-Hard Contact Lens.
3-CXL ( Transepithelial – Epi-off(.
4-Rings ( Kerarings- Myoring(.
5-Keratoplasty(Lamellar – penetrating(
Rules in treatment
1-Non of treatment options is satisfactory for the
patient.
2-The disease is progressive by its nature.
3-Follow up is mandatory.
4-Combination of treatment options can be done.
5-Keratoplasty can be a final destiny even with
treatment.
6-Rings mostly will be followed by glasses.
7-Don't judge on improvement of VA without correction.
6-Financial aspect should be taken into consideration.
Glasses and follow up
When?????
1-Age > 28 ys old.
2-Stable and low refraction with BCVA
>6/24
3-Clear cornea
4-Favorable Pentacam:
1(Average Keratometry <46 Ds
2(Thinnest Pachymetry > 480 um
Hard Contact Lens
--GP lenses are not the same as the
old hard lenses. For one thing, GP
lens materials allow oxygen to pass
through the lens and reach the
cornea.
-With advances in manufacturing, GP
lenses are made in thinner designs,
larger diameters, and with more
consistently smooth edges than ever
before.
-GP contact lenses are custom made for
each individual.
-Parameters which are needed for GP
contact lens request.
1(Keratometry: for initial fitting
2(Refraction.
-Soft lenses do provide better initial comfort,
while GP lenses require a brief adaptation
period. But this is due to the size of the
lens — not the lens material.
-Soft lenses are larger in diameter than GP
lenses and "tuck under" the eyelids. As a
result, you don't feel the lens edges when
you blink. But since GP lenses are smaller,
during blinking your eyelids will experience
initial "lens awareness.
Hybrid lenses
This is a lens design combination that
has an RGP center surrounded by a
soft peripheral “skirt”. Hybrid contact
can provide the crisp optics of a GP
lens and wearing comfort of soft
contact lenses. They are available in
a wide variety of parameters to
provide a fit that conforms well to the
irregular shape of a keratoconic eye.
CXL (Transepithelial – Epi-off(.
Why CXL is important???
-The only actual therapy for
keratoconus.
-Main effect is stiffening and flattening.
-Long term effect.
-Minimal optical effect.
1-Transepithelial CXL
Important hints:
-Less effective than Epi-off.( Less
flattening effect(
-Less complications rate.
-More comfortable for the patient.
When to do?????
-Early Keratoconus. 46 Ds<Mean K <
48 Ds
-Middle aged patients.
-Very thin corneas.
-After ICRS.
-Inadequate follow up.
2-Epi-Off CXL
When to do???
-In moderate and advanced cases.
-In young patients < 25 ys old.
Intracorneal Rings
Types of Rings
Ideal patient for rings
-High errors.
-Mean Keratometry > 48 Ds
-K Max > 50 Ds
-BCVA < 6/30
-High patient motivation
Which type?????
Kerarings
-Non central cones.
-High cylinder.
-High difference between K1, K2.
-Thickness at insertion site >400 um
Pre and post Kerarings Pentacam
Myoring
-Central cones ( Nipple ,Oval , Globus(
-High K readings K1, K2 with low
difference.
-High errors with high sphere.
-Thinnest location > 400 um.
-Epi-off like CXL.
-Eye without refraction.
Pre and post Myoring Pentacam
Central cone - Refraction : -8 Ds -7 Dc
-Very high K readings For Myoring implantation
-Shifted cone , Refraction : -9Ds -4.5 Dc
-K2 @ 68 For Kerarings implantation
Combined CXL with rings
When to do???
With Myoring:
-It should be done in the same session
( intrapocket CXL(.
-Epi-off like effect as it crosses the epithelium
With Kerarings:
-It should be done in the same session or after
ring implantation not before.
Age as a guideline for decision
Young age < 25 ys ---------- be
more aggressive
Early Keratoconus: Epi-off CXL
stabilization and follow up
Moderate and severe Keratoconus:
CXL  stabilization + Rings 
Regularization and Flattening
Advanced opacified cornea :
Keratoplasty
Middle age > 25 ys ---------- be less
aggressive
Early Keratoconus: Epi-on CXL or follow
up
Moderate and severe Keratoconus:
Rings  Regularization and Flattening with
follow up if progression  Stabilization by
CXL
Advanced opacified cornea :
Keratoplasty
Rings in advanced cases
Aim:
-To delay corneal grafting.
-To make the eye refractable.
-To decrease coma aberrations.
N.B: Keratoplasty is still an option
Home message
-Many guidelines affect our decision in
keratoconus management.
-Pentacam is an important tool in evaluation
of Keratoconus patient.
-Age is a guiding factor in treatment with
aggressive attitude in young age.
-Customization should be done for every
patient in keratoconus management.
Thank you

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Available options for keratoconus management

  • 1. Available Options For Keratoconus Management BY Dr. Amr Mounir , Lecturer of Ophthalmology Sohag university
  • 2.
  • 3. Definition Keratoconus is a progressive, noninflammatory, bilateral (but usually asymmetric) ectatic corneal disease, characterized by paraxial stromal thinning and weakening that leads to corneal surface distortion..
  • 4. Etiology -History of trauma that causes weakness -Recurrent trauma due to rubbing from Blepharitis, Vernal keratoconjuctivitis -Inflammatory component!!! Decrease proteinase inhibitors Increase collagenase Premature keratocytic apoptosis Increase cytokine binding
  • 5. Pathology --Epithelial basement membrane fragmentation and scarring --Axial stromal thinning and scarring -Breaks in and folds close to the Descemet membrane result in acute hydrops
  • 6. Clinical Picture Patients with keratoconus (KC) often report decreasing vision (distortions, glare/flare, and monocular diplopia or ghost images), with multiple unsatisfactory attempts in obtaining optimum spectacle correction
  • 7. Signs -Decrease in visual acuity -Progressive myobia ,irrigular Astigmatism -Oil droplet sign by direct ophthalmoscope - irregular scissoring by retinoscopy
  • 8. Slitlamp findings FLEISCHER RING abrupt change in curvature VOGT’S STRIAE 1st Sign STROMAL THINNING STROMAL SCARS ENLARGED CORNEAL NERVES ACUTE HYDROPS
  • 12. Investigations 1-Old methods: A- Placido Disc B- Retinoscopy 2-New Methods: A- Corneal topography B- Pentacam
  • 13. What Pentacam says to us??? 1-Pachymetry map 2-Keratometry map ( K1,K2,Kmax( 3-Cone site and shape 4-Elevation map 5-Provisional diagnosis: Keratoconus Summary ( Sirus device( 6-Aberration map 7-Scheimpflug imaging
  • 16. Classifications of keratoconus Several classifications of keratoconus based on: 1-Morphological Patterns 2-Topographical Patterns
  • 21. Topographical Patterns Krumeich Classification of Keratoconus: Severity of KC is also classified by Krumeich. This classification depends on mean K- readings on the anterior curvature sagittal map, thickness at the thinnest location, and the refractive error of the patient.
  • 22.
  • 23. Forme Fruste Keratoconus Forme Fruste Keratoconus (FFKC(: is a subclinical disease and is not a variant of KC. Although clinicians use many other terms such as mild KC, early KC, and subclinical KC
  • 24. Recently, there are two opinions regarding the definition of this disease: 1.FFKC is a completely normal cornea with neither clinical nor topographical risk factors, but this cornea is able to develop KC when treated by laser. The fellow eye may be keratoconic or there may be a family history of KC
  • 25. 2.FFKC is an abnormal cornea. Corneal topography or corneal hysteresis or both are abnormal; i.e., there are risk factors but the case is still not a clinically obvious KC.
  • 26.
  • 27.
  • 29.
  • 30. Pellucid Marginal Degeneration (PMD) and Pellucid-like Keratoconus -PMD is a bilateral, non-inflammatory, peripheral corneal thinning disorder characterized by a peripheral band of thinning of the inferior cornea. The cornea in and adjacent to the thinned area is ectatic. -Patients usually are aged 20–40 years at the time of clinical presentation.
  • 31.
  • 34. Options For Treatment Depend on: 1-Age 2-Refraction 3-Pachymetry 4-Keratometry 5-Cone Position 6-Corneal Opacifications.
  • 35. Options 1-Glasses and follow up. 2-Hard Contact Lens. 3-CXL ( Transepithelial – Epi-off(. 4-Rings ( Kerarings- Myoring(. 5-Keratoplasty(Lamellar – penetrating(
  • 36. Rules in treatment 1-Non of treatment options is satisfactory for the patient. 2-The disease is progressive by its nature. 3-Follow up is mandatory. 4-Combination of treatment options can be done. 5-Keratoplasty can be a final destiny even with treatment. 6-Rings mostly will be followed by glasses. 7-Don't judge on improvement of VA without correction. 6-Financial aspect should be taken into consideration.
  • 37. Glasses and follow up When????? 1-Age > 28 ys old. 2-Stable and low refraction with BCVA >6/24 3-Clear cornea 4-Favorable Pentacam: 1(Average Keratometry <46 Ds 2(Thinnest Pachymetry > 480 um
  • 38. Hard Contact Lens --GP lenses are not the same as the old hard lenses. For one thing, GP lens materials allow oxygen to pass through the lens and reach the cornea. -With advances in manufacturing, GP lenses are made in thinner designs, larger diameters, and with more consistently smooth edges than ever before.
  • 39. -GP contact lenses are custom made for each individual. -Parameters which are needed for GP contact lens request. 1(Keratometry: for initial fitting 2(Refraction.
  • 40.
  • 41. -Soft lenses do provide better initial comfort, while GP lenses require a brief adaptation period. But this is due to the size of the lens — not the lens material. -Soft lenses are larger in diameter than GP lenses and "tuck under" the eyelids. As a result, you don't feel the lens edges when you blink. But since GP lenses are smaller, during blinking your eyelids will experience initial "lens awareness.
  • 42. Hybrid lenses This is a lens design combination that has an RGP center surrounded by a soft peripheral “skirt”. Hybrid contact can provide the crisp optics of a GP lens and wearing comfort of soft contact lenses. They are available in a wide variety of parameters to provide a fit that conforms well to the irregular shape of a keratoconic eye.
  • 43.
  • 45. Why CXL is important??? -The only actual therapy for keratoconus. -Main effect is stiffening and flattening. -Long term effect. -Minimal optical effect.
  • 46. 1-Transepithelial CXL Important hints: -Less effective than Epi-off.( Less flattening effect( -Less complications rate. -More comfortable for the patient.
  • 47. When to do????? -Early Keratoconus. 46 Ds<Mean K < 48 Ds -Middle aged patients. -Very thin corneas. -After ICRS. -Inadequate follow up.
  • 48.
  • 49. 2-Epi-Off CXL When to do??? -In moderate and advanced cases. -In young patients < 25 ys old.
  • 50.
  • 52.
  • 54. Ideal patient for rings -High errors. -Mean Keratometry > 48 Ds -K Max > 50 Ds -BCVA < 6/30 -High patient motivation
  • 56. Kerarings -Non central cones. -High cylinder. -High difference between K1, K2. -Thickness at insertion site >400 um
  • 57. Pre and post Kerarings Pentacam
  • 58. Myoring -Central cones ( Nipple ,Oval , Globus( -High K readings K1, K2 with low difference. -High errors with high sphere. -Thinnest location > 400 um. -Epi-off like CXL. -Eye without refraction.
  • 59. Pre and post Myoring Pentacam
  • 60. Central cone - Refraction : -8 Ds -7 Dc -Very high K readings For Myoring implantation
  • 61. -Shifted cone , Refraction : -9Ds -4.5 Dc -K2 @ 68 For Kerarings implantation
  • 63. When to do??? With Myoring: -It should be done in the same session ( intrapocket CXL(. -Epi-off like effect as it crosses the epithelium With Kerarings: -It should be done in the same session or after ring implantation not before.
  • 64. Age as a guideline for decision
  • 65. Young age < 25 ys ---------- be more aggressive Early Keratoconus: Epi-off CXL stabilization and follow up Moderate and severe Keratoconus: CXL  stabilization + Rings  Regularization and Flattening Advanced opacified cornea : Keratoplasty
  • 66. Middle age > 25 ys ---------- be less aggressive Early Keratoconus: Epi-on CXL or follow up Moderate and severe Keratoconus: Rings  Regularization and Flattening with follow up if progression  Stabilization by CXL Advanced opacified cornea : Keratoplasty
  • 68. Aim: -To delay corneal grafting. -To make the eye refractable. -To decrease coma aberrations. N.B: Keratoplasty is still an option
  • 69.
  • 70.
  • 71. Home message -Many guidelines affect our decision in keratoconus management. -Pentacam is an important tool in evaluation of Keratoconus patient. -Age is a guiding factor in treatment with aggressive attitude in young age. -Customization should be done for every patient in keratoconus management.