MODULE 2A: KERATOCONUS

Definition

Cause

RF

Slowly progressive, non-inflammatory axial thinning and distortion of

cornea
Usually bilateral but asymmetric
Unknown
Classically described as associated with certain systemic diseases such
as atopy and CT DO
Usually manifests at 16y, but can start at any age
No sexual predilection

DIAGNOSIS signs and symptoms characteristic of keratoconus

1 Case Hx
1. Primary symptom: deteriorating vision more prominent in one eye
2. Monocular diplopia
3. Ghosting of images
4. Distortion
5. Blur
6. Owns many pairs of specs
7. History of K in family
2 Vision
Gradual decrease in VA (often first symptom)
Best correctable VA can deteriorate with time
Often no VA, and cant be compensated for
3 Retinoscopy
Poor reflex
Scissors-like motion
As condition progresses, reflex gets worse, and cannot do
retinoscopy effectively at all
4 Keratometry
Lack of parallelism of mires
Mire distortion ranges from moderate to severe
K readings often off scale in at least one meridian
Amount of mire distortion > corneal distortion
5 Cornal
How cornea is distorted
topography
Early > characteristic pear shaped pull of central keratoscopy ring
occurs
Steepening occurs inferiorly and in termporal quadrant
Superior cornea remains essentially normal
2 important contributors to understanding keratoconus and
diagnosing it:
Qualitative and quantitative indices that one can extract from
some topographers
Often do not work if K is bad
6 Biomicroscopy
1. Fleischers ring iron deposits in cornea
2. Munson sign
3. Thinning of cornea
4. Increased visibility of corneal nerves
5. Vogts striae
6. Breaks in descemets
7. Folds in stroma/endothelium
8. Scarring of apex of cornea
Signs and symptoms of keratoconus

Qualitative assessment
By evaluating topography pattern, can
make qualitative judgements relating
to appearance of pattern
Classification system:
A. Round
B. Oval
C. Superior steepening
D. Inferior steepening
E. Irregular
F. Symmetric bowtie
G. Symmetric bowtie with skewed radial
axes (SRAX)
H. Asymmetric bowtie with inferior
steepening
I. Asymmetric bowtie with superior
steepening
J. Asymmetric bowtie with skewed radial
axes

What does the KISA % index include

Quantitative indices
Topographers have built in indices that
vary between instruments
Tomy instrument:
Surface regularity index (SRI)
Surface asymmetry index (SAI)
Pentacam:
Index of surface variance (ISV)
Index of vertical asymmetry (IVA)
Keratoconus index (KI)
Centre keratoconus index (CKI)
Radii min (Rmin)
Index of height asymmetry (IHA)
Index of height decentration (IHD)
Aberration coefficient (ABR)
Calculate indices from raw height data:
Central K
I-S Index
SRAX index
KISA% index
K-value (> 47.2D = K)
I-S index (> 1.4 = K)
AST index (simk 1 simk 2)
SRAX index
KISA% = (K) x (I-S) x (AST) x (SRAX) x
100
Mean KISA significantly greater in
keratoconics
Index is highly sensitive and specific to
diagnosing keratoconics

MANAGEMENT
1. Spectacle correction
Cornea changes in K result in myopia and astigmatism
Limitations to use of specs:
1. High refractive astigmatism results in intolerable spec wear due to weight,
thickness, distortions, etc
2. Changing refractive state diurnally or week to week
3. Inadequate VA with specs
4. Anisometropia due to asymmetric nature of disease
2. Hydrogels
Rarely fitted as optical results are poor
Sometimes it is the only lens that is tolerated and gives better vision when used wit
spectacle overcorrection
Trapezoid lens
Fenestrated
Fit similar to scleral lenses
Difficult to manufacture
Needs to be thick (0.6mm)
Made of Hema
Optical effects poor
Soft-K
New soft lens designed and produced in Israel for K
Made of 67% water material with central thickness of 0.38mm
Thickness of lens allows lens to form uniform front surface on cornea
and allow adequate sight
Two large fenestrations present to allow for equalization of pressure
behind lens and exchange of tears (located over sclera)
Problem: thickness and related decreased amount of oxygen supply
to cornea
Draw the profile across the diameter of a negative hydrogel lens
- O2 arriving at point A has traveled through less lens material than O 2 arriving at point B
- O2 tension will be greater at A than B, causing diffusion from A to B
- In minus lenses this is from center to periphery
Draw the profile across the diameter of a positive hydrogel lens
- O2 arriving at point A has traveled through less lens material than O 2 arriving at point B
- O2 tension will be greater at A than B, causing diffusion from A to B
- In plus lenses this is from periphery to center
3. Combination
Soft perm
Rigid centre that becomes a soft skirt
DK/L of rigid centre = 5-10
Water content of skirt = 25%
ADV
DDV
As comfortable as a soft
permeability of rigid
lens due to its size and
centre too low to provide
soft skirt
adequate amounts of
oxygen for average cornea
Gives optics of a hard lens covers whole cornea
No tear exchange under
lens
Synergeyes
New lens in USA
Higher DK in rigid and hydrogel components
Piggy-back
In late stages of K, RGP lens used over soft lens may improve
system
comfort and more regular corneal surface
ADV
DDV
Useful system when hard
Increased potential for
lens continually abrades
hypoxia
epithelium
Soft lens acts as bandage
Neo common
lens and protects

4.

epithelium from hard lens

RGP provides optics
Two lenses to keep clean
needed for K
Rigid lenses
Resurface irregular cornea and allow intervening fluid lens to correct corneal irregular
astigmatism
Major rigid lens fitting techniques:
Apical
With primary lens support being provided by apex of
bearing
cornea
Apical
Lens support and bearing directed off apex and onto
clearing
paracentral cornea with clearance of apex of cornea
Three point
Lens support with bearing shard between apex and
touch
paracentral cornea
Semi-scleral
Complete vaulting of cornea with semi-scleral lenses
Lenses are large (13.5 18mm diameter)
Mini lenses
Rose-K
8.6mm diameter
Fitting pearls:
1. Have variety of trial lenses
Small diameter < 8.6mm
Large diameters > 9.2mm up to 19mm
2. Choice of fitting philosophy and therefore lens choice is one of necessity
3. New research indicates that most optoms do not fit lenses steep enough
4. Often, the lens you fit is not related to the K readings
5. Always use a retinoscope when doing an over-refraction

Discuss the fitting of rigid lenses in keratoconus

Rigid lenses
Most often used in the management of keratoconus
Resurface irregular cornea and allow intervening fluid lens to correct corneal irregular astigmatism
Major rigid lens fitting techniques
Apical bearing: with primary lens support provided by the apex of the cornea
Apical clearing: with lens support and bearing directed off the apex and onto the paracentral cornea with
clearance of the corneal apex
Three point touch: lens support and bearing shared between the apex and the paracentral cornea
Guidelines
1. Variety of trail lenses diameter between 8.6 and 9.2mm
2. Lens choice of necessity. May end up fitting a patient in a manner you did not expect to.
3. Research lenses not fitted steep enough
4. Often lens you fit is not related to K-readings
5. Always use ret with over-refraction
CORNEA WITH K CAN VARY IN THE FOLLOWING WAYS:
1. Cone position
2. Cone size
3. Degree of myopia
4. Amount of corneal toricity
5. Steepness of K readings
6. Corneal topography
7. Disease progression
8. Achievement of corrected VA
9. CL tolerance
List the variations that can occur in a keratoconic cornea

SEVERAL FORMS OF LENS DESIGN USED IN K TREATMENT

1. Aspheric
2. Three point touch
3. Apical clearance
4. Large flat lenses
5. Rose-K type lenses
6. Large steep, vaulting lenses that are large in diameter
7. Scleral lenses
Aspheric

Three point
touch

Good
1.
2.
3.

Fitted much steeper than flatter corneal meridian

Overall diameter controls amount of lens movement and
displacement over cone on blinking
As base curve is made steeper, diameter needs to be decreased
Parameter changes are evaluated by trial and error fitting and
observation process
Aspheric lenses fitted to K should rest on cone and move away
radially from cone towards periphery
ADV over spherical BC designs > progressive flattening from apex to
periphery allows lens to fit without excessive para-apical or peripheral
bearing
fit:
Minimal movement and displacement
Complete peripheral edge clearance
Light apical bearing
Popular fitting philosophy
Lens fitted slightly flatter than flattest K
2.3mm of mild apical bearing and at least two other areas
approximately 180 apart at corneal midperiphery
Weight of lens distributed across cornea and not concentrated in one
area
As little movement as possible, but still allow tear flow under lens
Centration not always possible as cone can be markedly displaced >
lens should be centred over cone

Slide 6

Large flat
lenses

Apical
clearance

Rose-K type
Large vaulting
lens

Done in an attempt to halt progression of the cone

Excessive bearing results in scarring of cone
Doesnt work very well
Corneal scarring can be initiated/accelerated by the way a lens
relates to the cornea
If bearing is used, area of contact between lens and cornea should be
kept minimum
Attempt total avoidance of bearing on cone
Apical clearance doesnt promote apex scarring
Most practitioners fit K too flat and need to steepen their fits
Attempt to fit lens that is not going to compromise cornea, give px
adequate vision and be comfortable
Lens should be stable on eye and not result in excessive bearing on
cornea
Rose K and Rose K2 are small (8.7mm)
Designed to fit over cone and into moat that surrounds cone
Fairly new mainly due to large gas permeable blanks becoming
available
Labs: ABBA (ABBA semi-scleral 13.5mm) and Medlens (Jupiter 15 and
18mm)
Lens is fitted such that lens vaults cornea completely
No tear exchange as lens sits on sclera and needs to e filled with fluid
when inserted
Should be no touch on cornea; only Fl
No blanching of limbal/scleral vessels
Useful for px with conditions:
1. Host Vs graft diseas
2. Terriens marginal degeneration
3. Sjogrens syndrome
4. Steven Johnsons syndrome
5. Dry eye

Lens designs in treatment of keratoconus

1. Aspheric
2. Three point touch
3. Apical clearance
4. Large flat lenses
1. Aspheric
Fitted much steeper than the flattest corneal meridian
Overall diameter controls the amount of lens movement and displacement over the cone on blinking
As the base curve gets steeper the diameter decrease
Should rest on the cone, move away rapidly from the cone towards the periphery
Prerequisites
o Minimal movement and displacement
o Complete peripheral edge clearance
o Light apical bearing
2. Three point touch
one of the most popular
fitted slightly flatter than the flattest K
with 2-3 mm of mild apical bearing
at least 2 other areas approximately 180 degrees apart at the corneal midperiphery
weight distributed across the cornea
little as possible movement, but still allow tear flow under the lens
centration is not always possible, but lens should be centred over the cone
3. Large flat lenses

 In attempt to stop progression of the cone

Excessive bearing scarring of the cone
Method does not work well
4. Apical clearance
Total avoidance of bearing of the cone
Most practitioners fit the cornea too flat

SCLERAL LENSES
Haptic Scleral lenses
Specs
Large (18 25mm diameter)
Until recently were made of PMMA
Cover whole cornea and surrounding sclera
PMMA
RGP
Often moulded from positive mould
Preformed (material breaks when
of eye
trying to mould lens over an
impression
Fenestration necessary
RGP > non ventilated
Uses
Keratoconus
Post-LASIK
Post-LASEK
Keratoplasty
Distorted cornea
ADV, DDV
ADV
DDV
1. Scleral bearing (not corneal
1. Bulk
bearing)
2. Large size
2. Pre-corneal reservoir of tears
3. Reduce oxygen supply but
provide optical uniformity
RGP materials have made big
3. High powers available
difference
4. Cannot be dislodged easily
4. Because corneal clearance, VA
5. Comfort because lens sits
and stability may be
under lids
compromised
Indications
When nothing else works
Fitting
Limited to preformed, non-ventilated RGP lenses
Advantages of scleral lenses

Innovative sclerals
Basic designs
3 zones to each lens:
1. Peripheral spherical scleral zone
2. Broad aspheric transition zone overlying peri and limbal region
3. Spherical optic zone
Diagrams
Controlled clearance scleral lens fitting
Controlled clearance scleral lens
Optic, transition and scleral zones
Scleral radius and optic zone projecti

OZP

Trial lenses

Testing

Important parameter
All corneas project forward
Normal corneas project 0.8 2mm from sclera
Abnormal corneas may project up to 4mm
In the fitting set, lenses have increasing OZPs
Allows one to fit lens that just doesnt touch cornea
Two sets: 13.5mm (N) and 14.5mm (F) scleral zone radius lenses
Each set has 9 lenses o increasing OZP
Usually normal (N) scleral zone radius lens tried first, and then corneal
compression is assessed and the OZP is changed as needed
Keratometry and topography useless

 Assess projection of cornea > optic section with slitlamp

Insertion
Saline fills optic zone > px keeps head down
Concept of optic zone projection
- Relates to innovative scleral lenses
- All corneas project forward
- Normal 0.8 to 2mm from the sclera
- Abnormal corneas may project up to 4mm
- Limbal and apical
- Projection of cornea allowed for by optic zone projection of the lens
SURGERY
Cross-linking:
Used to create new cross links between collagen fibres of stroma
Epithelium is abraded (in total/strips)
Riboflavin is dripped onto cornea and cornea is exposed to UV
Cornea has to be at least 400 microns thick so UV doesnt penetrate eye
Tx doesnt eliminate keratoconus but retards/halts progression
Collagen in keratoconus/ abnormal corneas
Collagen fibrils not arranged perpendicular to one another and this causes vision not be clear
Collagen fibers are weakened and pull out their cross-links
Reduced number of fibroblasts and keratocytes
The deficiency of keratocytes cause changes in the corneal structure and transparency
Reduced GAGs and proteoglycans
Apex of cone creates disturbance
Lower intermolecular spacing
Collagen in normal corneas
Collagen is arranged perpendicular to one another
Thick and healthy fibers tight cross-links
Contain fibroblasts and keratocytes
Large number of keratocytes
Keratocytes produce collagen and proteoglycans which maintain the integrity of the cornea
Arrangement is important for clear vision in normal cornea it is arranged perpendicular to one another
GAGs and proteoglycans in high numbers