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Keratoconus, Lenticonus, Lentiglobus ,
Microspherophakia & Lens Coloboma
Dr. Sharad
SCEH, Lahan,
O.A 2nd year student class
keratoconus
• Keratoconus (conical cornea) is a non-inflammatory bilateral (85%) ectatic
condition of cornea in its axial part. It usually starts at puberty and progresses
slowly .
Etiopathogenesis.
• developmental condition & degenerative condition
• hereditary dystrophy and endocrine anomaly.
• Essential pathological changes are progressive thinning and ectasia which occur
as a result of defective synthesis of mucopolysaccharide and collagen tissue .
Clinical features & Symptoms.
• Patient presents with a defective vision due to progressive myopia and
irregular astigmatism .
• Sign : 1. Window reflex is distorted.
• 2. Placidodisc examination shows irregularity of the circles .
• 3. Slit-lamp examination may show thinning and ectasia of central cornea
• 4. Fleischer’s ring at the base of cone, folds in Descemet’s and Bowman’s
membranes .
Signs of Keratoconus :
• 5. On retinoscopy a yawning reflex (scissor reflex ) & irregular astigmatism .
• 6. Ondistant direct ophthalmoscopy : oil droplet reflex
• 7. Munson’s sign, i.e., localised bulging of lower lid when patient looks down is
positive .
• 8. Keratometry. Normal average keratometric values are 45 D. In keratoconus
keratometric values are increased. keratoconus is graded as: mild (< 48 D), moderate
(48–54 D), and severe (>54 D).
morphological classification
• Nipple cone has a small size (<5 mm) and steep curvature.
• • Oval cone is larger (5–6 mm) and ellipsoid in shape.
• • Globuscone is very large (>6 mm) and globe like
Complications
• Keratoconus may be complicated by development of acute hydrops
due to rupture of Descemet’s membrane .
• Lacrimation
• Photophobia
• Blepharospasm
Association Ocular conditions like
• Ectopia lentis, congenital cataract, aniridia
• Retinitis pigmentosa & vernal keratoconjunctivitis (VKC)
• Marfan’s syndrome
• Down’s syndrome
• Ehlers-Danlos syndrome,
Treatment :
• 1. Spectacle correction may improve vision in early cases.
• 2. Contact lenses (rigid gas permeable /RGP lens ) usually improve the
vision in early cases. Scleral contact lens (Rose-K) may be useful.
• 3. INTACS the intracorneal ring segments, are reported to be useful .
• 4. Corneal collagen cross linking with riboflavin (CXL or C3R) and UV-A rays
may slow the progression .
• 5. . Keratoplasty may be required in later stages. Deep anterior lamellar
keratoplasty (DALK) or penetrating keratoplasty (PK )
Keratoconus :
KERATOGLOBUS : Keratoglobus is a rare, noninflammatory corneal
ectasia characterized by diffuse protrusion and thinning of the cornea.
• It is nonprogressive and inherited as an autosomal recessive trait.
• It must be differentiated from congenital buphthalmos, where increased corneal
size is associated with raised intraocular pressure, angle anomaly and/or cupping
of optic disc.
How keratoconus is differentiated from keratoglobus
• Keratometry readings can reach 50-60 D. Fleischer rings, Vogt striae, and anterior
stromal scarring are not typical, but Descemet membrane thickening and folds
are common.
Management of keratoglobus
• No medical therapy has been discovered to treat the underlying pathology.
Instead, attention is focused on complications such as acute hydrops.
• Hypertonic saline, cycloplegia, and bandage soft contact lenses are the
mainstay of treatment.
• Aqueous suppressants have also been proposed to decrease stromal uptake of
fluid .
• SURGERY : penetrating keratoplasty (PK) was one of the first surgical procedures
attempted . A second surgical option involves the use of DSAEK donor tissue to
patch the host endothelium.
Lenticonus :
• It is a rare congenital anomaly of the eye characterized by a conical protrusion on
the crystalline lens capsule and the underlying cortex.
• It can reach a diameter of 2 to 7 mm.
• The conus may occur anteriorly or posteriorly.
anterior & posterior lenticonus
Association
Keratoconus, lenticonus, lentiglobus , microspherophakia &amp;
Keratoconus, lenticonus, lentiglobus , microspherophakia &amp;

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Keratoconus, lenticonus, lentiglobus , microspherophakia &amp;

  • 1. Keratoconus, Lenticonus, Lentiglobus , Microspherophakia & Lens Coloboma Dr. Sharad SCEH, Lahan, O.A 2nd year student class
  • 3. • Keratoconus (conical cornea) is a non-inflammatory bilateral (85%) ectatic condition of cornea in its axial part. It usually starts at puberty and progresses slowly .
  • 4. Etiopathogenesis. • developmental condition & degenerative condition • hereditary dystrophy and endocrine anomaly. • Essential pathological changes are progressive thinning and ectasia which occur as a result of defective synthesis of mucopolysaccharide and collagen tissue .
  • 5. Clinical features & Symptoms. • Patient presents with a defective vision due to progressive myopia and irregular astigmatism . • Sign : 1. Window reflex is distorted. • 2. Placidodisc examination shows irregularity of the circles . • 3. Slit-lamp examination may show thinning and ectasia of central cornea • 4. Fleischer’s ring at the base of cone, folds in Descemet’s and Bowman’s membranes .
  • 6. Signs of Keratoconus : • 5. On retinoscopy a yawning reflex (scissor reflex ) & irregular astigmatism . • 6. Ondistant direct ophthalmoscopy : oil droplet reflex • 7. Munson’s sign, i.e., localised bulging of lower lid when patient looks down is positive . • 8. Keratometry. Normal average keratometric values are 45 D. In keratoconus keratometric values are increased. keratoconus is graded as: mild (< 48 D), moderate (48–54 D), and severe (>54 D).
  • 7. morphological classification • Nipple cone has a small size (<5 mm) and steep curvature. • • Oval cone is larger (5–6 mm) and ellipsoid in shape. • • Globuscone is very large (>6 mm) and globe like
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  • 11. Complications • Keratoconus may be complicated by development of acute hydrops due to rupture of Descemet’s membrane . • Lacrimation • Photophobia • Blepharospasm
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  • 13. Association Ocular conditions like • Ectopia lentis, congenital cataract, aniridia • Retinitis pigmentosa & vernal keratoconjunctivitis (VKC) • Marfan’s syndrome • Down’s syndrome • Ehlers-Danlos syndrome,
  • 14. Treatment : • 1. Spectacle correction may improve vision in early cases. • 2. Contact lenses (rigid gas permeable /RGP lens ) usually improve the vision in early cases. Scleral contact lens (Rose-K) may be useful. • 3. INTACS the intracorneal ring segments, are reported to be useful . • 4. Corneal collagen cross linking with riboflavin (CXL or C3R) and UV-A rays may slow the progression . • 5. . Keratoplasty may be required in later stages. Deep anterior lamellar keratoplasty (DALK) or penetrating keratoplasty (PK )
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  • 18. KERATOGLOBUS : Keratoglobus is a rare, noninflammatory corneal ectasia characterized by diffuse protrusion and thinning of the cornea.
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  • 20. • It is nonprogressive and inherited as an autosomal recessive trait. • It must be differentiated from congenital buphthalmos, where increased corneal size is associated with raised intraocular pressure, angle anomaly and/or cupping of optic disc.
  • 21. How keratoconus is differentiated from keratoglobus • Keratometry readings can reach 50-60 D. Fleischer rings, Vogt striae, and anterior stromal scarring are not typical, but Descemet membrane thickening and folds are common.
  • 22. Management of keratoglobus • No medical therapy has been discovered to treat the underlying pathology. Instead, attention is focused on complications such as acute hydrops. • Hypertonic saline, cycloplegia, and bandage soft contact lenses are the mainstay of treatment. • Aqueous suppressants have also been proposed to decrease stromal uptake of fluid . • SURGERY : penetrating keratoplasty (PK) was one of the first surgical procedures attempted . A second surgical option involves the use of DSAEK donor tissue to patch the host endothelium.
  • 23. Lenticonus : • It is a rare congenital anomaly of the eye characterized by a conical protrusion on the crystalline lens capsule and the underlying cortex. • It can reach a diameter of 2 to 7 mm. • The conus may occur anteriorly or posteriorly.
  • 24. anterior & posterior lenticonus
  • 25.