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OPTOM FASLU MUHAMMED
a. Anterior lenticonus
b. Posterior lenticonus
c. Microspherophakia
d. Coloboma of the lens
 The surface capsule in the pupillary zone with
the underlying cortical fibres exhibit spherical
or conoid transformation
 Predominantly affect boys often bilaterally
 There may be associated systemic anomalies
- alport's syndrome
 Seen in girls as solitary unilateral condition
 It may be seen associated with Lowe's
syndrom
 The oil globule reflex in the pupillary area is
the main clinical feature.
 The site of the cone shows a thin basement
membrane (capsule)
 Decrease in nucleated cells in the anterior
form
 Posterior cone is composed of abnormally
nucleated cells (which are generally absent)
 In this condition, the lens is spherical in shape
(instead of normal biconvex) and small in
size.
 Microspherophakia may occur as an isolated
familial condition or as a feature of other
syndromes e.g.,Weil-Marchesani or Marfan’s
syndrome.
 It is seen as a notch in the lower quadrant of
the equator .
 It is usually unilateral and often hereditary
a. Flecks
b. Vacuoles
c. Opacification (cataract)
- Primary
- secondary
 Focal lenticular opacification occurs due to
adhesion of pupillary membrane anteriorly
and hyaloid vessel posteriorly.
 The are sub capsular or superficial cortical.
 Vacuoles of different sizes may appear in sub
capsular or cortical zones.
 They represents hydropic degenerator.
 A number of agents, metabolic chemical may
produce vacuolar change.
 Morphological classification
1. Capsular cataract. It involves the capsule and
may be:
i. Anterior capsular cataract
ii. Posterior capsular cataract
2. Sub capsular cataract. It involves the
superficial part of the cortex (just below the
capsule) and includes:
i. Anterior sub capsular cataract
ii. Posterior sub capsular cataract
3. Cortical cataract. It involves the major part of
the cortex.
4. Supranuclear cataract. It involves only the
deeper parts of cortex (just outside the
nucleus).
5. Nuclear cataract. It involves the nucleus of
the crystalline lens
6. Polar cataract. It involves the capsule and
superficial part of the cortex in the polar
region only and may be:
i. Anterior polar cataract
ii. Posterior polar cataract
 The cataracts are often stationary
 Attenuation or loss of epithelial cells followed
by fibrous metaplasia of sub capsular region.
 New epithelial cell may be laid down
underneath the fibrous plaque.
 The cataract developing elderly is called
senile cataract.
 They are idiopathic in nature
 These are account for 55% of curable
blindness in india
 Clinical recognisable forms of senile cataract
1. Cortical (soft cataract)
2. Nuclear (hard cataract)
 There is fibre fragmentation , loss of
epithelial cells and disturbance in the
epithelial configuration noticed in the bow
region.
 Hypermature cataract show vacuolation.
 Associated nuclear alteration such as
pigmentation and hardening.
[A] Maturation of the cortical type of senile
Cataract
1. Stage of lamellar separation.
 The earliest senile change is demarcation of
cortical fibres owing to their separation by
fluid.
 This phenomenon of lamellar separation can
be demonstrated by slit-lamp examination
only.
 These changes are reversible.
2. Stage of incipient cataract.
 In this stage early detectable opacities with
clear areas between them are seen.
 Two distinct types of senile cortical cataracts
can be recognised at this stage:
(a) Cuneiform senile cortical cataract
 It is characterised by wedge-shaped opacities
with clear areas in between.
 These extend from equator towards centre and
in early stages can only be demonstrated after
dilatation of the pupil.
 They are first seen in the lower nasal quadrant.
 These opacities are present both in anterior and
posterior cortex and their apices slowly progress
towards the pupil.
 On oblique illumination these present a
typical radial spoke-like pattern of greyish
white opacities .
 On distant direct ophthalmoscopy, these
opacities appear as dark lines against the red
fundal glow.
 Since the cuneiform cataract starts at
periphery and extends centrally, the visual
disturbances are noted at a comparatively
late stage.
(b) Cupuliform senile cortical cataract
 a saucershaped opacity develops just below the
capsule usually in the central part of posterior
cortex (posterior subcapsular cataract), which
gradually extends outwards.
 There is usually a definite demarcation between
the cataract and the surrounding clear cortex.
 Cupuliform cataract lies right in the pathway of
the axial rays and thus causes an early loss of
visual acuity.
3. Immature senile cataract (ISC).
 In this stage, opacification progresses further.
 The cuneiform or cupuliform patterns can be recognised till
the advanced stage of ISC when opacification becomes
more diffuse and irregular.
 The lens appears greyish white but clear cortex is still
present and so iris shadow is visible.
 In some patients, at this stage, lens may become swollen
due to continued hydration.This condition is called
‘intumescent cataract'.
 Intumescence may persist even in the next stage of
maturation.
 Due to swollen lens anterior chamber becomes shallow.
4. Mature senile cataract (MSC).
 In this stage ,opacification becomes
complete, i.e., whole of the cortex is involved.
 Lens becomes pearly white in colour.
 Such a cataract is also labelled as ‘ripe
cataract’
5. Hypermature senile cataract (HMSC).
 When the mature cataract is left in situ, the
stage of hypermaturity sets in.
 The hypermature cataract may occur in any
of the two forms:
(a) Morgagnian hypermature cataract:
 In some patients, after maturity the whole cortex
liquefies and the lens is converted into a bag of
milky fluid.
 The small brownish nucleus settles at the
bottom, altering its position with change in the
position of the head. Such a cataract is called
Morgagnian cataract.
 Sometimes in this stage, calcium deposits may
also be seen on the lens capsule.
(b) Sclerotic type hypermature cataract:
 Sometimes after the stage of maturity, the
cortex becomes disintegrated and the lens
becomes shrunken due to leakage of water.
 The anterior capsule is wrinkled and thickened
due to proliferation of anterior cells and a dense
white capsular cataract may be formed in the
pupillary area.
 Due to shrinkage of lens, anterior chamber
becomes deep and iris becomes tremulous
(iridodonesis).
[B] Maturation of nuclear senile cataract
 In it, the sclerotic process renders the lens
inelastic and hard, decreases its ability to
accommodate and obstructs the light rays.
 These changes begin centrally and slowly
spread peripherally almost up to the capsule
when it becomes mature;
 however, a very thin layer of clear cortex may
remain unaffected.
 The nucleus may become diffusely cloudy
(greyish) or tinted (yellow to black) due to
deposition of pigments.
 In practice, the commonly observed
pigmented nuclear cataracts are either
amber, brown (cataracta brunescens) or black
(cataracta nigra) and rarely reddish (cataracta
rubra) in colour.
 Lenticular opacification following an
intraocular inflammation or neoplasm.
 The anatomical site of lens damage is based
on the location of primary disease process.
 Anterior segment disorder such as uveitis
produce anterior cataract
 Vitreo retinitis or melanoma affects posterior
lens.
1. Phacoanaphylactic uveitis.
 A hypermature cataract may leak lens
proteins into anterior chamber.
 These proteins may act as antigens and
induce antigen antibody reaction leading to
uveitis.
2. Lens-induced glaucoma.
 It may occur by different mechanisms e.g.,
due to intumescent lens (phacomorphic
glaucoma) and
 leakage of proteins into the anterior chamber
from a hypermature cataract (phacolytic
glaucoma).
3. Subluxation or dislocation of lens.
 It may occur due to degeneration of zonules
in hypermature stage.
 Lenticular capsule is thin basement membrane
of underlying epithelial cells.
 It is the thickest one in the body, elastic in
character and resist entry bacteria or any
particulate matter.
1. Thinning
2. Thickening
3. Exfoliation
- true
- pseudo
 It may occur in mature cataracts
 It may occur focally or diffusely.
 Diffuse basement membrane thickening, as
an ageing process, may accompany senile
cataract.
 Focal thickening is noticed in lowe’s
syndrome (oculo cerebral renal syndrome).
 They are of two types
1. True exfoliation
 It is rare and occurs due to laminar
separation of capsule , which scrolls over
anterior surafce.
 The disease may be related to infrared
exposure
2.Pseudo exfoliation
 It is a disease characterised by deposition of
white fluffy dandruff-like material over
anterior surface of lens, iris, zonules, etc.
 Biochemically , it is glycoprotein with sialo
and oligosaccharide side chains.
 The disease occurs in 50-60 yr old individuals
with involvement of both eyes in over 50%
cases.
 The material in 5-10% cases is known to
affect outflow channels leading to ocular
hypertension.
 Ultrastructurally , it is filamentous in nature
similar to basement membrane.
 Displacement of the lens from its normal
position (in patellar fossa) results from partial
or complete rupture of the lens zonules.
 Topographically, displacements of the lens
may be classified as subluxation and luxation
or dislocation.
I. Subluxation
 It is partial displacement in which lens is
moved sideways (up, down, medially or
laterally), but remains behind the pupil.
 It results from partial rupture or unequal
stretching of the zonules.
II. Dislocation or luxation of the lens
 In it all the zonules are severed from the lens.
 A dislocated lens may be incarcerated into
the pupil or present in the anterior chamber ,
the vitreous (where it may be floating – lens
nutans; or fixed to retina – lens fixata), sub-
retinal space, subscleral space or extruded
out of the globe, partially or completely.
(a) Simple ectopia lentis
 In this condition displacement is bilaterally
symmetrical and usually upwards.
 It is transmitted by autosomal dominant
inheritance.
(b) Ectopia lentis et pupillae
 It is characterised by displacement of the
lens associated with slit-shaped pupil which is
displaced in the opposite direction.
 Other associations may be cataract,
glaucoma and retinal detachment.
(c) Ectopia lentis with systemic anomalies
1. Marfan’s syndrome
2. Homocystinuria
3. Weil-Marchesani syndrome
 Mesodermal dysplasia with several ocular
cardiovascular anomalies characterises marfan’s
syndrome.
 The disorder is autosomal dominant with
metabolic abnormality related to collagen
synthesis.
 The ocular lesions include iris hypo -
pigmentation,subluxation of lens.
 In this condition lens is displaced upwards and
temporally (bilaterally symmetrical).
 It is an autosomal recessive,inborn error of
metabolism.
 Absence or deficiency cystathionine synthetase
which results in increase in homocystine.
 The abnormality associated with formation of
short segment of zonules and PAS positive
material accumulation over ciliary epithelium.
 In it the lens is usually subluxated downwards
and nasally.
 It is condition of autosomal recessive
mesodermal dysplasia.
 Ocular features are spherophakia, and forward
subluxation of lens which may cause pupil
block glaucoma.
 The eye may also be myopic.

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Lenticular abnormalities

  • 2.
  • 3. a. Anterior lenticonus b. Posterior lenticonus c. Microspherophakia d. Coloboma of the lens
  • 4.  The surface capsule in the pupillary zone with the underlying cortical fibres exhibit spherical or conoid transformation
  • 5.  Predominantly affect boys often bilaterally  There may be associated systemic anomalies - alport's syndrome
  • 6.
  • 7.
  • 8.  Seen in girls as solitary unilateral condition  It may be seen associated with Lowe's syndrom
  • 9.
  • 10.
  • 11.  The oil globule reflex in the pupillary area is the main clinical feature.  The site of the cone shows a thin basement membrane (capsule)  Decrease in nucleated cells in the anterior form  Posterior cone is composed of abnormally nucleated cells (which are generally absent)
  • 12.  In this condition, the lens is spherical in shape (instead of normal biconvex) and small in size.  Microspherophakia may occur as an isolated familial condition or as a feature of other syndromes e.g.,Weil-Marchesani or Marfan’s syndrome.
  • 13.
  • 14.  It is seen as a notch in the lower quadrant of the equator .  It is usually unilateral and often hereditary
  • 15.
  • 16.
  • 17. a. Flecks b. Vacuoles c. Opacification (cataract) - Primary - secondary
  • 18.  Focal lenticular opacification occurs due to adhesion of pupillary membrane anteriorly and hyaloid vessel posteriorly.  The are sub capsular or superficial cortical.
  • 19.
  • 20.  Vacuoles of different sizes may appear in sub capsular or cortical zones.  They represents hydropic degenerator.  A number of agents, metabolic chemical may produce vacuolar change.
  • 21.
  • 22.  Morphological classification 1. Capsular cataract. It involves the capsule and may be: i. Anterior capsular cataract ii. Posterior capsular cataract 2. Sub capsular cataract. It involves the superficial part of the cortex (just below the capsule) and includes: i. Anterior sub capsular cataract ii. Posterior sub capsular cataract
  • 23. 3. Cortical cataract. It involves the major part of the cortex. 4. Supranuclear cataract. It involves only the deeper parts of cortex (just outside the nucleus). 5. Nuclear cataract. It involves the nucleus of the crystalline lens
  • 24. 6. Polar cataract. It involves the capsule and superficial part of the cortex in the polar region only and may be: i. Anterior polar cataract ii. Posterior polar cataract
  • 25.
  • 26.  The cataracts are often stationary  Attenuation or loss of epithelial cells followed by fibrous metaplasia of sub capsular region.  New epithelial cell may be laid down underneath the fibrous plaque.
  • 27.  The cataract developing elderly is called senile cataract.  They are idiopathic in nature  These are account for 55% of curable blindness in india
  • 28.  Clinical recognisable forms of senile cataract 1. Cortical (soft cataract) 2. Nuclear (hard cataract)
  • 29.  There is fibre fragmentation , loss of epithelial cells and disturbance in the epithelial configuration noticed in the bow region.  Hypermature cataract show vacuolation.  Associated nuclear alteration such as pigmentation and hardening.
  • 30. [A] Maturation of the cortical type of senile Cataract 1. Stage of lamellar separation.  The earliest senile change is demarcation of cortical fibres owing to their separation by fluid.  This phenomenon of lamellar separation can be demonstrated by slit-lamp examination only.  These changes are reversible.
  • 31. 2. Stage of incipient cataract.  In this stage early detectable opacities with clear areas between them are seen.  Two distinct types of senile cortical cataracts can be recognised at this stage:
  • 32. (a) Cuneiform senile cortical cataract  It is characterised by wedge-shaped opacities with clear areas in between.  These extend from equator towards centre and in early stages can only be demonstrated after dilatation of the pupil.  They are first seen in the lower nasal quadrant.  These opacities are present both in anterior and posterior cortex and their apices slowly progress towards the pupil.
  • 33.
  • 34.  On oblique illumination these present a typical radial spoke-like pattern of greyish white opacities .  On distant direct ophthalmoscopy, these opacities appear as dark lines against the red fundal glow.  Since the cuneiform cataract starts at periphery and extends centrally, the visual disturbances are noted at a comparatively late stage.
  • 35. (b) Cupuliform senile cortical cataract  a saucershaped opacity develops just below the capsule usually in the central part of posterior cortex (posterior subcapsular cataract), which gradually extends outwards.  There is usually a definite demarcation between the cataract and the surrounding clear cortex.  Cupuliform cataract lies right in the pathway of the axial rays and thus causes an early loss of visual acuity.
  • 36. 3. Immature senile cataract (ISC).  In this stage, opacification progresses further.  The cuneiform or cupuliform patterns can be recognised till the advanced stage of ISC when opacification becomes more diffuse and irregular.  The lens appears greyish white but clear cortex is still present and so iris shadow is visible.  In some patients, at this stage, lens may become swollen due to continued hydration.This condition is called ‘intumescent cataract'.  Intumescence may persist even in the next stage of maturation.  Due to swollen lens anterior chamber becomes shallow.
  • 37. 4. Mature senile cataract (MSC).  In this stage ,opacification becomes complete, i.e., whole of the cortex is involved.  Lens becomes pearly white in colour.  Such a cataract is also labelled as ‘ripe cataract’
  • 38. 5. Hypermature senile cataract (HMSC).  When the mature cataract is left in situ, the stage of hypermaturity sets in.  The hypermature cataract may occur in any of the two forms:
  • 39. (a) Morgagnian hypermature cataract:  In some patients, after maturity the whole cortex liquefies and the lens is converted into a bag of milky fluid.  The small brownish nucleus settles at the bottom, altering its position with change in the position of the head. Such a cataract is called Morgagnian cataract.  Sometimes in this stage, calcium deposits may also be seen on the lens capsule.
  • 40.
  • 41. (b) Sclerotic type hypermature cataract:  Sometimes after the stage of maturity, the cortex becomes disintegrated and the lens becomes shrunken due to leakage of water.  The anterior capsule is wrinkled and thickened due to proliferation of anterior cells and a dense white capsular cataract may be formed in the pupillary area.  Due to shrinkage of lens, anterior chamber becomes deep and iris becomes tremulous (iridodonesis).
  • 42. [B] Maturation of nuclear senile cataract  In it, the sclerotic process renders the lens inelastic and hard, decreases its ability to accommodate and obstructs the light rays.  These changes begin centrally and slowly spread peripherally almost up to the capsule when it becomes mature;  however, a very thin layer of clear cortex may remain unaffected.
  • 43.  The nucleus may become diffusely cloudy (greyish) or tinted (yellow to black) due to deposition of pigments.  In practice, the commonly observed pigmented nuclear cataracts are either amber, brown (cataracta brunescens) or black (cataracta nigra) and rarely reddish (cataracta rubra) in colour.
  • 44.
  • 45.  Lenticular opacification following an intraocular inflammation or neoplasm.  The anatomical site of lens damage is based on the location of primary disease process.  Anterior segment disorder such as uveitis produce anterior cataract  Vitreo retinitis or melanoma affects posterior lens.
  • 46. 1. Phacoanaphylactic uveitis.  A hypermature cataract may leak lens proteins into anterior chamber.  These proteins may act as antigens and induce antigen antibody reaction leading to uveitis.
  • 47. 2. Lens-induced glaucoma.  It may occur by different mechanisms e.g., due to intumescent lens (phacomorphic glaucoma) and  leakage of proteins into the anterior chamber from a hypermature cataract (phacolytic glaucoma).
  • 48. 3. Subluxation or dislocation of lens.  It may occur due to degeneration of zonules in hypermature stage.
  • 49.  Lenticular capsule is thin basement membrane of underlying epithelial cells.  It is the thickest one in the body, elastic in character and resist entry bacteria or any particulate matter. 1. Thinning 2. Thickening 3. Exfoliation - true - pseudo
  • 50.  It may occur in mature cataracts
  • 51.  It may occur focally or diffusely.  Diffuse basement membrane thickening, as an ageing process, may accompany senile cataract.  Focal thickening is noticed in lowe’s syndrome (oculo cerebral renal syndrome).
  • 52.  They are of two types 1. True exfoliation  It is rare and occurs due to laminar separation of capsule , which scrolls over anterior surafce.  The disease may be related to infrared exposure
  • 53. 2.Pseudo exfoliation  It is a disease characterised by deposition of white fluffy dandruff-like material over anterior surface of lens, iris, zonules, etc.  Biochemically , it is glycoprotein with sialo and oligosaccharide side chains.  The disease occurs in 50-60 yr old individuals with involvement of both eyes in over 50% cases.
  • 54.  The material in 5-10% cases is known to affect outflow channels leading to ocular hypertension.  Ultrastructurally , it is filamentous in nature similar to basement membrane.
  • 55.  Displacement of the lens from its normal position (in patellar fossa) results from partial or complete rupture of the lens zonules.  Topographically, displacements of the lens may be classified as subluxation and luxation or dislocation.
  • 56. I. Subluxation  It is partial displacement in which lens is moved sideways (up, down, medially or laterally), but remains behind the pupil.  It results from partial rupture or unequal stretching of the zonules.
  • 57.
  • 58. II. Dislocation or luxation of the lens  In it all the zonules are severed from the lens.  A dislocated lens may be incarcerated into the pupil or present in the anterior chamber , the vitreous (where it may be floating – lens nutans; or fixed to retina – lens fixata), sub- retinal space, subscleral space or extruded out of the globe, partially or completely.
  • 59.
  • 60. (a) Simple ectopia lentis  In this condition displacement is bilaterally symmetrical and usually upwards.  It is transmitted by autosomal dominant inheritance.
  • 61. (b) Ectopia lentis et pupillae  It is characterised by displacement of the lens associated with slit-shaped pupil which is displaced in the opposite direction.  Other associations may be cataract, glaucoma and retinal detachment.
  • 62. (c) Ectopia lentis with systemic anomalies 1. Marfan’s syndrome 2. Homocystinuria 3. Weil-Marchesani syndrome
  • 63.  Mesodermal dysplasia with several ocular cardiovascular anomalies characterises marfan’s syndrome.  The disorder is autosomal dominant with metabolic abnormality related to collagen synthesis.  The ocular lesions include iris hypo - pigmentation,subluxation of lens.  In this condition lens is displaced upwards and temporally (bilaterally symmetrical).
  • 64.  It is an autosomal recessive,inborn error of metabolism.  Absence or deficiency cystathionine synthetase which results in increase in homocystine.  The abnormality associated with formation of short segment of zonules and PAS positive material accumulation over ciliary epithelium.  In it the lens is usually subluxated downwards and nasally.
  • 65.  It is condition of autosomal recessive mesodermal dysplasia.  Ocular features are spherophakia, and forward subluxation of lens which may cause pupil block glaucoma.  The eye may also be myopic.