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case
• 20 years old saudi female come to outpatient
clinic in KFH & file # 36034723 complaining of
progressive gradual loss of vision in the both
eyes for two years.. she said that ( the glasses
didn't fix the problem and I've changed it
many times ) .. she denied any history of
trauma .. there were no history of diabetes
nor HTN ..
Examination :
on examination :-
VA 6/9 OD ( right ) .
6/18 OS ( left ).
IOP was 17 mmHg in both eyes .
External sign ( Munson's sign ) .
SLE : 1- stromal thinning
2- Vogt's striae
Ophthalmoscope shows oil droplet sign .
Funduscopy appeared normal.
differential diagnosis
• Astigmatism
• Myopia
•
management
• Contact lens because he still on stage 2
Anatomy of cornea:
• The cornea is the transparent, dome-shaped
or Prolate which is flatter in the periphery
and steeper centrally & its window covering
the front of the eye. It is a powerful refracting
surface, providing 2/3 of the eye's focusing
power.
• Average size:
11mm vertically
12mm horizontally
histology
Definition
“Keratoconus” is a medical term used to
describe a condition in which the cornea
assumes a conical shape because of thinning
and protrusion.
Keratoconus
• Keratoconus (KC) is a progressive,
noninflammatory, bilateral (but usually
asymmetrical) ectatic corneal disease,
characterized by paraxial stromal thinning and
weakening that leads to corneal surface
distortion(forming conical shape) induces
irregular astigmatism, myopia and protrusion
leading to mild to moderate impairment in the
quality of vision.
Histopathology
• Inherited ectatic disorder leading to reduction of
corneal thickness
Characterized by:
- Extreme corneal thinning.
- Focal defects in epithelial basement
membrane and Bowmans’ layer.
( Pathognomonic)
Clinical Presentation
• The earliest Symptoms
blurring of vision ( 10Y – 30Y)
Changing eye glass prescription
Uncorrectable vision to 20/20
with glasses
Evaluation and diagnosis of keratoconus
History and family history
Follow up evaluation
Slit -lamp exams
Keratoscopy- Keratometry
Corneal thickness - pachymetry
Topography - Orbscan –Pentacam
Signs / Symptoms
• KC often results in irregular astigmatism which can
severely limit distance and night vision in a way that
can not always be corrected using glasses
• Ghosting/ monocular diplopia
• Glare at night
• Haloes around lights
• Blurred/ distorted vision
Signs / Symptoms
• KC often results in irregular astigmatism which can
severely limit distance and night vision in a way that
can not always be corrected using glasses
Signs / Symptoms
• Frequently changing spectacle Rx and axis of astigmatism
• Poor repeatability of subjective refraction
• Ghosting/ monocular diplopia
• Glare at night
• Haloes around lights
• Blurred/ distorted vision
• Scissors reflex: (swirling retinoscopy reflex)
• Distorted/ irregular keratometer mires with steep readings
• Prominent corneal nerves
Detection of Keratoconus
1. Retinoscpy
2. Slit lamp
3. Keratometry
4. Keratoscpy
Vogt’s striae
fleisher’ ring
SIGNS OF KCN
• External signs :
– Munson” sign
– Rizzuti phenomenon
• Slit-lamp findings:
– Stromal thining
– Posterior stress lines(vogt,s striae)
– Iron ring ( fleischer ring )
– Scarring - epithelial or subepithelial
• Retroillumination signs:
– Scissoring on retinoscopy
– Oil droplet sign (charleaux”)
Signs of KC
• High irregular astigmatism
• External signs :
– Munson’s sign
– Rizzuti phenomenon
Sharply focused beam of light near the nasal
limbus ,produced by lateral illumination of the
cornea in patients with advanced keratoconus
Signs of KC Slit-lamp findings
– Stromal thinning
A hallmark, occurs at the apex of the cone
(the point of maximal protrusion)
:
Posterior stress lines
(Vogt,s striae)
• Fine vertical lines in the deep stroma, just
anterior to descemet,s membrane that parallel
the axis of the cone and disappear transiently
on gentle digital pressure
Corneal Scarring
epithelial or subepithelial
• Superficial linear scars at the corneal apex
• These results from ruptures in bowman,s layer
Iron ring ( fleischer ring )
• The ring is a partial or complete annular line
commonly seen at the base of the cone
• The ring is formed from hemosiderin pigment
deposited in the basal epithelium
Retroillumination and
retinoscopy signs
• Scissoring on retinoscopy
• Oil droplet sign (charleaux”)
Corneal hydrops
•Acute descemet membrane rupture and corneal
hydrops it will led to the deranges of Aq. Humor
inside the cornea
Risk Factors
• Uncontrolled ocular surface allergic disease
• Chronic rubbing of the eyes
• ocular associations
– vernal keratoconjunctivitis
– retinitis pigmentosa
• systemic associations
– connective tissue disorders
(eg, Ehlers-Danlos and Marfan syndromes)
– mitral valve prolapse
– Atopic dermatitis
– Down syndrome.
Computerized Corneal Topography
• Corneal topography is
the method by which
the corneal shape,
curvature, power, and
irregularities are
measured
Computerized Corneal Topography
• Corneal topography provides:
 The Axial Curvature Map.
 Describe the Shape and Power
including the central 1-2 mm
 The Elevation Maps.
 The Pachymetric Map.
 Quantitative Indices
Keratoconus
• Localised inferiorly displaced steepness
• Keratoconus suspect
– K > 47.2
– I-S > 1.4
• Clinical keratoconus
– K > 48.7
– I-S > 1.9
How to reade am mape
• be sure you are reading for same Pt & be sure
File # & age & sex & this map for whice eye
• green colure is near to normal Yellow is
boredr line and red in like patches its is hot
spote
• Focus on avery map alone
• Put one point & strata reading
Anterior Map
Posterior Map
Keratometric Map
Pachemetry Map
Keratoconus pattern
non-Keratoconus cornea
general symmetry overall
with no exsessive steepening
Keratocunus cornea
extreme asymmetrical
and inferior steepening
Abnormal Topography
Topographic patterns of keratoconus
 Inferior steepening without bowtie pattern
specially more prominent temporally
 Asymmetrical bow tie (AB)
& inferior/superior steepening(IS-SS)
 Central steepening +/- superimposed with
asymmetrical bowtie pattern
 symmetric bow tie (AB) &
skewed radial axes (SRAX)
Topographic patterns of keratoconus
Topographic patterns of keratoconus
Topographic patterns of keratoconus
 Inferior steepening without bowtie pattern
specially more prominent temporally
 Asymmetrical bow tie (AB)
& inferior/superior steepening (IS-SS)
 Central steepening +/- superimposed with
asymmetrical bowtie pattern
 Asymmetic /symmetric bow tie (AB) &
skewed radial axes (SRAX)
Keratoconus pattern:
Inferior steepening without bowtie pattern
specially more prominent temporally
Keratoconus pattern:
asymmetrical bow tie (AB)
& inferior steepening(IS)
Keratoconus pattern:
asymmetrical bow tie (AB)
& superior steepening(SS)
Keratoconus pattern:
Central keratoconus
central unusual steepening without bow tie
Keratoconus pattern:
Central steepening, superimposed with asymmetrical bowtie-
slightly irregular astigmatism asymmetrical central hourglass
Globus topographic type of KCN
Keratoconus pattern:
Symmetic(SB) /asymmetric bow tie (AB) &
(SRAX)
Keratoconus pattern:
asymmetrical bow tie &
skewed radial axes (AB/SRAX)
Keratoconus pattern:
symmetrical bow tie &
skewed radial axes (AB/SRAX)
Grading of keratoconus according to
amsler krumeich classification:
Stage I
• Eccentric steeping
• Myopia and astigmatism < 5.00 D
• Mean central K readings < 48.00 D
Stage II
• Myopia and astigmatism from 5.00 to 8.00 D
• Mean central K readings < 53.00 D
• Absence of scarring
• Minimum corneal thickness >400 μm.
Stage III
• Myopia and astigmatism from 8.00 to 10.00 D
• Mean central K readings >53.00 D
• Absence of scarring
• Minimum corneal thickness 300 to 400 μm.
Stage IV
• Refraction not measurable
• Mean central K readings >55.00 D
• Central corneal scarring
• Minimum corneal thickness 200 μm
treatment
• Spectacles—in stage 1
• Contact lens in stage 1;2 and 3(hard lenses)
• Photothrapeutic keratotomy.(in advanced kc)
• Intrastromal corneal rings.
• Corneal collagene cross linking . (stage 1;2)
• Phakic intraocular lenses.(stage1)
• Keratoplasty. (mainly in stage 4)
END
Prof. M.Barbary

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keratoconus

  • 1.
  • 2. case • 20 years old saudi female come to outpatient clinic in KFH & file # 36034723 complaining of progressive gradual loss of vision in the both eyes for two years.. she said that ( the glasses didn't fix the problem and I've changed it many times ) .. she denied any history of trauma .. there were no history of diabetes nor HTN ..
  • 3. Examination : on examination :- VA 6/9 OD ( right ) . 6/18 OS ( left ). IOP was 17 mmHg in both eyes . External sign ( Munson's sign ) . SLE : 1- stromal thinning 2- Vogt's striae Ophthalmoscope shows oil droplet sign . Funduscopy appeared normal.
  • 5. management • Contact lens because he still on stage 2
  • 6.
  • 7. Anatomy of cornea: • The cornea is the transparent, dome-shaped or Prolate which is flatter in the periphery and steeper centrally & its window covering the front of the eye. It is a powerful refracting surface, providing 2/3 of the eye's focusing power. • Average size: 11mm vertically 12mm horizontally
  • 9. Definition “Keratoconus” is a medical term used to describe a condition in which the cornea assumes a conical shape because of thinning and protrusion.
  • 10. Keratoconus • Keratoconus (KC) is a progressive, noninflammatory, bilateral (but usually asymmetrical) ectatic corneal disease, characterized by paraxial stromal thinning and weakening that leads to corneal surface distortion(forming conical shape) induces irregular astigmatism, myopia and protrusion leading to mild to moderate impairment in the quality of vision.
  • 11. Histopathology • Inherited ectatic disorder leading to reduction of corneal thickness Characterized by: - Extreme corneal thinning. - Focal defects in epithelial basement membrane and Bowmans’ layer. ( Pathognomonic)
  • 12. Clinical Presentation • The earliest Symptoms blurring of vision ( 10Y – 30Y) Changing eye glass prescription Uncorrectable vision to 20/20 with glasses
  • 13. Evaluation and diagnosis of keratoconus History and family history Follow up evaluation Slit -lamp exams Keratoscopy- Keratometry Corneal thickness - pachymetry Topography - Orbscan –Pentacam
  • 14. Signs / Symptoms • KC often results in irregular astigmatism which can severely limit distance and night vision in a way that can not always be corrected using glasses • Ghosting/ monocular diplopia • Glare at night • Haloes around lights • Blurred/ distorted vision
  • 15. Signs / Symptoms • KC often results in irregular astigmatism which can severely limit distance and night vision in a way that can not always be corrected using glasses
  • 16. Signs / Symptoms • Frequently changing spectacle Rx and axis of astigmatism • Poor repeatability of subjective refraction • Ghosting/ monocular diplopia • Glare at night • Haloes around lights • Blurred/ distorted vision • Scissors reflex: (swirling retinoscopy reflex) • Distorted/ irregular keratometer mires with steep readings • Prominent corneal nerves
  • 17. Detection of Keratoconus 1. Retinoscpy 2. Slit lamp 3. Keratometry 4. Keratoscpy Vogt’s striae fleisher’ ring
  • 18. SIGNS OF KCN • External signs : – Munson” sign – Rizzuti phenomenon • Slit-lamp findings: – Stromal thining – Posterior stress lines(vogt,s striae) – Iron ring ( fleischer ring ) – Scarring - epithelial or subepithelial • Retroillumination signs: – Scissoring on retinoscopy – Oil droplet sign (charleaux”)
  • 19. Signs of KC • High irregular astigmatism • External signs : – Munson’s sign – Rizzuti phenomenon Sharply focused beam of light near the nasal limbus ,produced by lateral illumination of the cornea in patients with advanced keratoconus
  • 20. Signs of KC Slit-lamp findings – Stromal thinning A hallmark, occurs at the apex of the cone (the point of maximal protrusion) :
  • 21. Posterior stress lines (Vogt,s striae) • Fine vertical lines in the deep stroma, just anterior to descemet,s membrane that parallel the axis of the cone and disappear transiently on gentle digital pressure
  • 22. Corneal Scarring epithelial or subepithelial • Superficial linear scars at the corneal apex • These results from ruptures in bowman,s layer
  • 23.
  • 24. Iron ring ( fleischer ring ) • The ring is a partial or complete annular line commonly seen at the base of the cone • The ring is formed from hemosiderin pigment deposited in the basal epithelium
  • 25. Retroillumination and retinoscopy signs • Scissoring on retinoscopy • Oil droplet sign (charleaux”)
  • 26. Corneal hydrops •Acute descemet membrane rupture and corneal hydrops it will led to the deranges of Aq. Humor inside the cornea
  • 27. Risk Factors • Uncontrolled ocular surface allergic disease • Chronic rubbing of the eyes • ocular associations – vernal keratoconjunctivitis – retinitis pigmentosa • systemic associations – connective tissue disorders (eg, Ehlers-Danlos and Marfan syndromes) – mitral valve prolapse – Atopic dermatitis – Down syndrome.
  • 28. Computerized Corneal Topography • Corneal topography is the method by which the corneal shape, curvature, power, and irregularities are measured
  • 29. Computerized Corneal Topography • Corneal topography provides:  The Axial Curvature Map.  Describe the Shape and Power including the central 1-2 mm  The Elevation Maps.  The Pachymetric Map.  Quantitative Indices
  • 30. Keratoconus • Localised inferiorly displaced steepness • Keratoconus suspect – K > 47.2 – I-S > 1.4 • Clinical keratoconus – K > 48.7 – I-S > 1.9
  • 31. How to reade am mape • be sure you are reading for same Pt & be sure File # & age & sex & this map for whice eye • green colure is near to normal Yellow is boredr line and red in like patches its is hot spote • Focus on avery map alone • Put one point & strata reading
  • 36. Keratoconus pattern non-Keratoconus cornea general symmetry overall with no exsessive steepening Keratocunus cornea extreme asymmetrical and inferior steepening
  • 38. Topographic patterns of keratoconus  Inferior steepening without bowtie pattern specially more prominent temporally  Asymmetrical bow tie (AB) & inferior/superior steepening(IS-SS)  Central steepening +/- superimposed with asymmetrical bowtie pattern  symmetric bow tie (AB) & skewed radial axes (SRAX)
  • 39.
  • 40. Topographic patterns of keratoconus
  • 41. Topographic patterns of keratoconus
  • 42. Topographic patterns of keratoconus  Inferior steepening without bowtie pattern specially more prominent temporally  Asymmetrical bow tie (AB) & inferior/superior steepening (IS-SS)  Central steepening +/- superimposed with asymmetrical bowtie pattern  Asymmetic /symmetric bow tie (AB) & skewed radial axes (SRAX)
  • 43. Keratoconus pattern: Inferior steepening without bowtie pattern specially more prominent temporally
  • 44. Keratoconus pattern: asymmetrical bow tie (AB) & inferior steepening(IS)
  • 45. Keratoconus pattern: asymmetrical bow tie (AB) & superior steepening(SS)
  • 46. Keratoconus pattern: Central keratoconus central unusual steepening without bow tie
  • 47. Keratoconus pattern: Central steepening, superimposed with asymmetrical bowtie- slightly irregular astigmatism asymmetrical central hourglass
  • 50. Keratoconus pattern: asymmetrical bow tie & skewed radial axes (AB/SRAX)
  • 51. Keratoconus pattern: symmetrical bow tie & skewed radial axes (AB/SRAX)
  • 52. Grading of keratoconus according to amsler krumeich classification: Stage I • Eccentric steeping • Myopia and astigmatism < 5.00 D • Mean central K readings < 48.00 D Stage II • Myopia and astigmatism from 5.00 to 8.00 D • Mean central K readings < 53.00 D • Absence of scarring • Minimum corneal thickness >400 μm.
  • 53. Stage III • Myopia and astigmatism from 8.00 to 10.00 D • Mean central K readings >53.00 D • Absence of scarring • Minimum corneal thickness 300 to 400 μm. Stage IV • Refraction not measurable • Mean central K readings >55.00 D • Central corneal scarring • Minimum corneal thickness 200 μm
  • 54. treatment • Spectacles—in stage 1 • Contact lens in stage 1;2 and 3(hard lenses) • Photothrapeutic keratotomy.(in advanced kc) • Intrastromal corneal rings. • Corneal collagene cross linking . (stage 1;2) • Phakic intraocular lenses.(stage1) • Keratoplasty. (mainly in stage 4)
  • 55.