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Keratoconus - Dr Shylesh B Dabke
1. Dr Shylesh B Dabke
Resident, Dept Of Ophthalmology
KMC, Mangalore
Keratoconus
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2. • Keratoconus a noninflammatory corneal ectasia, is characterized by
progressive corneal thinning and apical protrusion
• Described first by Burchard Mauchart(Staphyloma Diaphanum). John
Nottingham(1854) clearly described keratoconus and distinguished it
from other ectasia’s of cornea
• Preoperative topographic screening prior to keratorefractive surgery has
largely focused on keratoconus
Introduction
3. • Earlier reported to be higher in women than in men
• However recent studies suggested it to be higher in men or that there is
no significant difference
• It is bilateral in over 90% of patients
• Prevalence - 54.5 per one lakh population
• Asians have a fourfold higher incidence and are younger at presentation
compared to westerns
Sex Predilection
Prevalence & Statics
5. • Biomechanical hypothesis proposes keratoconus to result from
interlamellar and interfibrillar slippage of collagen within stroma*
• In addition to biomechanical changes, the concept of tissue degradation
based on the demonstration of collagen loss and proteoglycan changes
in affected cornea and on the up-regulation of degradative enzymes
have also been postulated
Pathophysiology
6. 1
•Same collagen content like normal cornea
2
•Decreased corneal stability
3
•2 times higher rate of degradation of cornea
Keratoconus
Reduced amount of cross linking
7. • Although can present in any age group, it more commonly affects
patients in their late teens or early twenties
• The condition almost always progressive but the rate of progression and
severity is variable
• Tends to progress more rapidly in young patients
• About 10-20% eventually require corneal transplant
Clinical Features
8. • Symptoms are highly variable and depend on the stage of the
progression of the disorder
• Early in the disease there may be no symptoms & may be noted simply
because the patient cannot refracted to a clear 6/6 corrected vision
Symptoms
9. Deteriorating visual acuity, distortions, glare
Frequent change in refraction
Visual acuity not correctable to 6/6
Monocular polyopia or Ghosting
Symptoms
16. • Keratoconus
- Clinical slit-lamp signs of keratoconus
- Scissoring on retinoscopy
- Keratoconus topography pattern
• Early/subclinical keratoconus
- No slit lamp findings
- Scissoring of reflex of the retinoscopy
- Keratoconus topography pattern
• Keratoconus-suspect
- No clinical signs, no scissoring
- Keratoconus topography pattern
Terminology
17. • Increased area of corneal power surrounded by concentric areas of
decreasing power
• Inferior-superior power asymmetry
• Skewing of the steepest radial axes above and below the horizontal
meridian(asymmetric bow tie with skewed radial axes (AB/SRAX)
pattern)
Characteristic Keratoconus Topographic Pattern*
23. • Keratometry
- mires commonly are steep, highly astigmatic, irregular and often
appear egg shaped.
- Also shows increased keratometry values between 45-52D or more
• Videokeratography
- Commonly shows inferior corneal steeping
Diagnosis
24. • Consists of three corneal topographic derived indices which when
abnormal should alert the clinician to consider keratoconus as diagnosis
• The indices are:
- Keratometry value quantifies the central steepening of cornea.
(A value of 47.2D or more)
- I-S value quantifies inferior versus superior corneal dioptric asymmetry
(Value 1.4D or more)
KISA percent incorporates the K and I-S values with a measure
quantifying the regular and irregular astigmatism into one index
• KISA percentage of >100 - Frank Keratoconus
60-100 - Kerataconus suspect
The Rabinowitz Diagnostic Criteria
25. • The Orbscan Corneal Topography II system uses the combination of
placido and scanning optical slit design to calculate the corneal
thickness and posterior surface of the entire cornea.
28. • The patient’s refractive error can often be successfully managed with
spectacle in the early stages
• However Contact lens provides better visual acuity than that obtained
by spectacles
Spectacle Correction
29. • Traditionally lenses for keratoconus have been hard or rigid gas
permeable contact lens variety
• For most of patients three point touch contact lens is ideal
• The most accurate way to fit keratoconic patients is to place a diagnostic
lens on the eye, check the fit and then modify the fit*
Contact Lenses
30. • In mild to moderate keratoconus the lens diameter selected is usually
7.5-8.5mm
- small size facilitates tear exchange and allows a steeper fit to
accommodate the cone
• Central nipple cones do best with small diameter lenses
• When the cone is displaced peripherally as with oval or globus cones
fitting a larger flatter lens may be required
31. • Soper lenses : One of best known lens with bicurve design with a deep
central curve to accommodate the steep central cone and a flat
peripheral curve to align with the peripheral cornea
• Hybrid lenses : Lenses with rigid gas permeable optic zone surrounded
by a soft skirt to ensure comfortable fit
• “Piggyback” lenses : Gas permeable rigid lenses are worn over soft
lenses
Especially designed contact lense
32. • The Rose K lenses : Unique keratoconus lens design with nor complex
computer generated peripheral curves based on precollected data
• Scleral lenses : Sometimes prescribed for cases of advanced or very irregular
keratoconus
• Refractive surgeries : LASIK or Photorefractive keratectomy is contraindicated
-Phototherapeutic keratectomy can be done in selected cases to reduce
steepness of cone in patients who have become Contact lens intolerant
- The resultant flattening of the cone makes contact lens fitting easier
33. • A recent surgical alternative to corneal transplant is the insertion of
intrastromal ring segments (ICRS)
• These implants are designed to be placed at a depth approximately
2/3rd the corneal thickness*
• They act by shortening the corneal arc length and have a net effect of
flattening the central cornea*
• ICRS have be indicated for contact lens intolerant patients with early
keratoconus who have minimal central stromal scarring
Intrastromal Corneal Ring Segments
36. • This treatment have been shown to slow down or arrest the progression
of keratoconus and in some cases reverse it
• The need to keratoplasty thus might be significantly reduced
• Clinical trials are continuing and the technique is definitely showing
promise in treating early cases
Corneal Collagen Cross linking with Riboflavin
37. • Corneal stroma soaked in riboflavin 0.1% eyedrops in 20% dextran and
activated by approximately 30mins illumination with UV-A light
Treatment procedure
38.
39. • Approximately 10% of patients will progress to a point
- where visual correction is not possible
- thinning becomes excessive
- scarring as a result of contact lens wear
Corneal Transplant
no one theory fully explains clinical findings
No direct evidence but the condition often first develops at the time of puberty and occasionally during pregnancy.
due to loss of cohesion between collagen fibrils and non collagenous matrix.
due to loss of cohesion between collagen fibrils and non collagenous matrix.
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fine stress lines in deep stroma. It is often the earliest slit lamp finding.
deposition of iron in basal epithelial cells in ring shape at base of conical protrusion. Faint and broad in early and thin and discrete as condition advances.
due to change in density.
acute rupture in descemet’s membrane results in development of sudden onset redness and pain due to imbibition of aqueous into corneal stroma.
reflex appears to swirl or spin around point corresponding to apex of cone.
on distant direct ophthalmoscopy
Corneal Topography
usually present inferior or inferotemporally
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small near centre less than 5mm in diameter
the most common type, apex of cone is displaced well below midline.
a large cone affecting nearly three quarter of corneal surface, more than 6mm in diameter
(based on keratometry)
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Often considered a variant of keratoconus with corneal thinning. Differentiated by videokeratography which shows against the rule corneal astigmatism
An inflammatory disease that affects the superior limbus & shows corneal thinning often with vascularisation and lipid deposits.
corneal thinning primarily at the margins resulting spherical enlarged eye.
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The Orbscan IIz is unique among corneal topographers in its ability to image the posterior surface of the cornea. Early signs of certain corneal diseases or abnormalities are seen first on the posterior corneal surface.
Top two images: Shape maps of the surface elevation of the front and back of the cornea.
Lower left image: A map of the surface power of the cornea.
Lower right image: A representation of the cornea's thickness.
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Since each individual cone is different above is a trial and error method.
greater risk for scarring and excessive thinning leading to possible post LASIK corneal ectasia.
are surgically inserted through a a small radial incision into a track created within the central cornea stroma
amount of flattening is determined by the inserts thickness
collagen cross linking within the corneal stroma and so recovers some of its mechanical strength
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-gold standard surgery for keratoconus patients with success rates of more than 90%
-keratoconic cornea is replaced by removing the central area of cornea and a full thickness donor corneal button is sutured in its place
-corneal trephines between 8-8.5mm are used
-second eye is not grafted until the first eye is successfully rehabilitated usually keeping an interval of 12 months.
a partial thickness corneal transplant
-The host cornea is removed upto the depth of posterior stroma and a lamellar donor corneal button is sutured in place
technique requires less recovery time, and poses less chance for corneal graft rejection or failure.
-This technique is difficult and visual acuity is inferior to that of penetrating keratoplasty
In DALK the patients endothelium is retained giving additional structural integrity to post graft cornea. This technique requires less recovery time