GLAUCOMA ASSOCIATED WITH LENS SUBLUXATION / DISLOCATION ALONG WITH GLAUCOMA ASSOCIATED WITH CATARACT FORMATION

1. LENS INDUCED
GLAUCOMA
Dr Kumar Siddharth

2. INTRODUCTION
Glaucomas associated with dislocation
of lens
Glaucomas associated with cataract
formation
 Phacolytic glaucoma
 Lens particle glaucoma
 Phacoanaphylaxis
 Phacomorphic glaucoma
Phacotopic glaucoma:
glaucoma due to anterior
lens displacement

3. A. GLAUCOMAS
ASSOCIATED WITH
DISLOCATION OF
LENS
•Incomplete dislocation of
lens from its anatomical
position, in which the lens
is still at least partially
behind the iris but is tilted
or displaced anteriorly/
posteriorly/ perpendicular
to the optical axis
Subluxati
on
•Entire lens is displaced from
its anatomical position,
maybe in the anterior
chamber or may have fallen
posteriorly into the vitreous
cavity
Dislocatio
n
• Hereditary or acquired
lens dislocation, but it is
non specific with regard
to the degree of
displacement.
Ectopia
lentis
Subluxation
Dislocation

4. CONDITIONS ASSOCIATED
WITH ECTOPIA LENTIS
Traumatic dislocation
Pseudoexfoliation syndrome
Simple ectopia lentis
Ectopia lentis et pupillae
Marfan syndrome
Homocystinuria
Weil-Marchesani syndrome
Spontaneous dislocation

5. TRAUMATIC DISLOCATION
Most common cause of displaced lens
Blunt force in anteroposterior direction leads to
equatorial expansion, which disrupts the zonular fibres
and dislocates the lens
In a series of 166 cases of dislocated lens, injury was
reported in 53% of the total group

6. PSEUDOEXFOLIATION
SYNDROME
Pseudoexfoliation
syndrome can be
associated with
spontaneous or
traumatic lens
subluxation or
dislocation
A. Right eye of the patient with complete
dislocation of the lens
B. Gonioscopic view of the right eye
revealing dislocated lens in the inferior
vitreous cavity
C. Left eye of the same patient showing
subluxation of lens

7. SIMPLE ECTOPIA LENTIS
Dislocation of lens can occur
without any ocular or systemic
abnormalities
Can be congenital or occur later
in life
Typically inherited
Autosomal dominant
Usually bilateral and
symmetrical
Lens dislocation generally
upward and outward and
occasionally in the anterior
chamber
Associated problems include
glaucoma and retinal
Ectopia lentis

8. ECTOPIA LENTIS ET
PUPILLAE
Autosomal recessive
Small subluxated lens
Oval or slit shaped pupil that are
displaced, usually in opposite
direction of that of the lens
Bilateral, but asymmetric
Associated with other ocular
abnormalities
Myopia
Cataract
Retinal detachment
Large cornea
Iris transillumination defects.
Ectopia lentis et pupillae

9. PATHOGENESIS OF ECTOPIA
LENTIS ET PUPILLAE
•Maldevelopment of ciliary processes
•Abnormality of secondary vitreous
•Neuroectodermal defect causing
hypoplasia of the iris, and
mesodermal defect causing
persistence of anterior and lateral
elements of tunica vasculosa lentis
Pathogenesis
of the
disease is
unknown.
Theories
proposed are

10. MARFAN SYNDROME
Autosomal dominant
Marfanoid habitus
Tall
Thin stature
Disproportionately long
limbs (arm span>height)
Long fingers and toes
(arachnodactyly)
Narrow high arched
“gothic” palate
Kyphoscoliosis
Sternal abnormalities
Joint laxity
Muscle under development
Cardiovascular lesion
Dilatation of aortic root
Mitral valve prolapse
Aortic aneurysm formation
Longer arm span in Marfan syndrome

11. Finger length in Marfan
Defect in fibrillin coding
gene, needed to produce
proteins required for
elasticity and strength of
connective tissue

12. OCULAR FEATURES
IN MARFAN
SYNDROME
Ectopia lentis is linked to gene
fibrillin on chromosome 15
Most common ocular finding in a
case series of 160 patients
Enlargement of the globe because
of scleral stretching and rupture
of zonules
Lens dislocated in 193 eyes
Most commonly superotemporal
subluxation (80%)of the lens, in 4th
to 5th decade
Other features include
Lattice degeneration of retina
Retinal detachment
Hypoplasia of dilator pupillae
strabismus
Superotemporal lens
subluxation in Marfan
syndrome

13. GLAUCOMA IN MARFAN
SYNDROME
Glaucoma may result from lens dislocation
Glaucoma can result from surgical aphakia
Glaucoma can result from anomaly of the
anterior chamber angle
In a review of 573 Marfan patients 29 patients
had glaucoma
Most common mechanism were chronic open
angle and glaucoma following lens extraction
or scleral buckling procedure

14. HOMOCYSTINURIA
Autosomal recessive
Marfanoid habitus
Other systemic features include
Coarse blond hair
Blue iris
Malar flush
Associated with mental retardation
Risk of thromboembolic episodes
Homocystinuria results from deficiency of enzyme cystathione
beta synthase required for amino acid methionine metabolism
Diagnosed by presence of homocysteine in urine
Autosomal recessive
Marfanoid habitus
Other systemic features include
Coarse blond hair
Blue iris
Malar flush
Associated with mental retardation
Risk of thromboembolic episodes

15. GLAUCOMA IN
HOMOCYSTINURIAAppropriate dietary modifications and vitamins supplementations
if started in childhood significantly reduce risk of ocular
complications (pyridoxine, B12, folic acid)
Lens subluxation occurs in younger age and more often
inferonasal
Universal by the age of 25 years in untreated cases
Zonule contains high level of cysteine normally, which is
deficient in homocystinuria, which causes zonular disintegration
Loss of accommodation
Other ocular findings include
Iris atrophy
Optic atrophy
Cataract
Myopia
Retinal detachment
Glaucoma is mostly due to lens dislocation

16. WEILL-MARCHESANI
SYNDROMEInheritance is autosomal dominant
(FBN1 gene, LTBP2 gene) or recessive
(ADAMTS10 gene)
Short and stocky habitus, learning
difficulties
Small round lenses (Microspherophakia)
Glaucoma associated with lens
dislocation is commoner than Marfan
syndrome and Homocystinuria
Shallow anterior chamber
Microspherophakia

17. MICROSPHEROPHAKIA
Small, round lens
Increased anteroposterior diameter
Decreased equatorial diameter
Investigators have hypothesized
that small ciliary body represents
elongated zonules, exerting less
force on the lens giving rise to the
spherical shape of the lens
1. Weill-Marchesani syndrome
2. Marfan syndrome
3. Homocystinuria
4. Klinefelter’s syndrome(XXY)
5. Mandibulofacial dystosis
6. Alport’s syndrome Microspherophakia
DIAMETER NORMAL MICROSPHEROPHAKI
A
VERTICAL 8.8-
9.2mm
6.75-7mm
HORIZONT
AL
3.4-
4.5mm
>5mm

18. GLAUCOMA IN
WEILL-
MARCHESANI
SYNDROMEHighly mobile lens rub against
iris and there is degeneration and
necrosis of epithelial cells and
destruction of the cortical fibres
Glaucoma maybe related to lens
dislocation or forward
subluxation of the lens causing
pupillary block glaucoma
(aggravated by miotic therapy)
Bilateral angle closure glaucoma
have also been reported in
patients without lens subluxation
after mid dilatation with
cyclopentolate
Subluxation of lens
Inverse glaucoma
Miotics
Contraction of ciliary musc
Loosening of zonules
Forward movement of the
Mydriatics
Relaxation of ciliary muscl
Tightening of the zonules
Posterior movement

19. SPONTANEOUS DISLOCATION
Middle aged or older individuals,
spontaneous dislocation of lens with
cataract formation
Spontaneous dislocation because of
mechanical stretching or inflammatory
disintegration /degeneration of zonules
has been reported in
High myopia
Uveitis
Bupthalmos
Hypermature cataract

20. OTHER CONDITIONS
ASSOCIATED WITH ECTOPIA
LENTIS
Ehlers-Danlos syndrome
Hyperlysinaemia
Sulfite oxidase deficiency
Aniridia
Scleroderma
Retinitis pigmentosa
Persistent pupillary
membrane
Axenfeld-Rieger syndrome
Dominantly inherited
blepharoptosis and high
myopia
Marfan like syndrome with
hyaloretinal degeneration
Sturge-Weber syndrome
Syphilis
Crouzon disease
Refsum syndrome

21. EXAMINATIONS AND
INVESTIGATIONS
Slit lamp
examination
Lenticular subluxation
Phacodonesis
Iridodonesis
Anterior chamber depth
In anterior lens
dislocation, clear lens
looks like oil droplet in
aqueous
Ultrasound
biomicroscopy
Zonular loss
Zonular stretching
Zonular stretching Zonular loss

22. MECHANISM OF GLAUCOMA
ASSOCIATED WITH
SUBLUXATED OR
DISLOCATED LENS
1. Angle
closure
2. Phacolytic
glaucoma
3. Concomitant
trauma

23. 1. ANGLE CLOSURE
MECHANISM
1. The anteriorly subluxated lens can push the iris
forward and obliterate the angle
2. Increased iridolenticular contact causes potential
pupillary block and iris bombe formation

24. ANGLE CLOSURE BY
PUPILLARY BLOCK
A. The lens may block the
aqueous flow through pupil if
dislocated in the pupil or
anterior chamber or tilted
against the iris
Common in microspherphakia,
because of loose zonules
If the pupillary block is because of
relaxed zonular support, it is
worsened by miotic therapy which
causes further relaxation of the
zonules
Cycloplegics may help by pulling the
lens posteriorly
B. Pupillary block maybe
associated with dislocated lens
due to herniation of vitreous in
the pupil Pupillary block

25. 2. PHACOLYTIC GLAUCOMA
In some cases lens may
dislocate completely into the
vitreous cavity and undergo
degenerative changes with
release of materials that
obstructs the aqueous flow
Subacute course of
glaucoma, hyperaemia and
inflammation Posteriorly dislocated lens

26. 3. CONCOMITANT TRAUMA
In traumatic dislocation of lens, concomitant
trauma to the iridocorneal angle maybe the
cause of the associated glaucoma
A transient pressure elevation of uncertain
origin may persist for weeks after traumatic
dislocation of lens

27. MANAGEMENT OF GLAUCOMA
ASSOCIATED WITH LENS
DISLOCATION
1. Dislocated or subluxated lens should only be removed when
 Inability to reposit it back in posterior chamber
 Complete dislocation of lens in anterior chamber
 Intractable glaucoma
 Reduction in visual acuity/severe diplopia
 Phacolytic glaucoma
 Phacoanaphylaxis

28. OTHER MODALITIES OF
MANAGEMENT
2. Laser iridotomy
Should be done peripherally to avoid subsequent obstruction
by the lens
Prophylactic iridotomy in microspherophakia has also been
advocated to avoid pupillary block
3. Hyperosmotic agents, carbonic anhydrase inhibitors and
topical hypotensive agents may be useful in breaking the
attacks
4. Laser peripheral iridoplasty maybe helpful in angle closure
glaucoma without significant pupillary block component

29. MANAGEMENT IN DIFFERENT
CASES
Lens displaced partially in anterior
chamber/pupil
Dilate the pupil allowing the lens
to reposit back in the posterior
chamber followed by miotic,
followed by iridotomy
Microspherophakia with anterior
subluxation of lens and pupillary
block
Patient should lie supine,
Hyperosmotics and ocular
hypotensive agents are given
Avoid both miotics and
cycloplegics
Lens is completely dislocated in
the anterior chamber
Constrict the pupil and surgically
remove the lens
Lens dislocated in vitreous cavity Lens removal is avoided unless it is
needed to relieve glaucoma/uveitis
or improve vision

30. B. GLAUCOMAS
ASSOCIATED WITH
CATARACT
FORMATION
Phacolytic
glaucoma
Lens
particle
glaucoma
Phaco
anaphylaxi
s
Phacomorphic
glaucoma
Lens protein leak from intact
cataract and obstructs
trabecular meshwork
Lens material liberated by
trauma or surgery obstructs
the outflow channel
Sensitization to lens protein
produces granulomatous
inflammation and occasionally
secondary glaucoma
A swollen lens causes
increased pupillary block and
secondary angle closure

31. 1. PHACOLYTIC (LENS
PROTEIN) GLAUCOMA
Described first by Gifford in 1900 as open angle
glaucoma associated with Hypermature cataract
Term Phacolytic glaucoma was suggested by Flocks and
colleagues after reporting that glaucoma inducing
mechanism was macrophagic response to lens material
Lens protein glaucoma was suggested by Epstein
colleagues

32. CLINICAL FEATURES OF
PHACOLYTIC
GLAUCOMAAcute onset monocular pain and redness
Gradual diminution of vision over months to years
On examination
•High IOP
•Conjunctival hyperaemia
•Diffuse corneal oedema
•Anterior chamber angle open and grossly
normal
•Heavy flare in the anterior chamber with
iridescent and hyper refringent particles
(calcium oxalate or cholesterol crystals)
•Chunks of white material in aqueous/ant
lens capsule/corneal endothelium which
histologically are macrophages
•No true KPs or hypopyon
Rare opacities in the vitreous
Cataract is typically mature/Hypermature and opaque
Corneal edema with
conjunctival injection
Conjunctival injection with deep
anterior chamber and prominent
anterior chamber reaction
Clumps of lens protein in
anterior chamber
Clumps of lens protein in
anterior chamberPseudo hypopyon
Lens protein deposition in
the angle and endothelium
Hypermature cataractCalcium oxalate crystal is
birefringent in polarized
light
Calcium oxalate crystal in len

33. Calcium oxalate crystal
Calcium oxalate crystal is
birefringent in polarized
light
Clumps of lens protein in
anterior chamber
Clumps of lens protein in
anterior chamber
Conjunctival injection with
deep anterior chamber
and prominent anterior
chamber reaction
Hypermature cataract Pseudo hypopyon
Corneal oedema with
conjunctival injection
Lens protein deposition in the
angle and endothelium

34. THEORIES OF MECHANISM
With age the lens composition is
altered to components with heavier
molecular weight
Release of lens protein into the
aqueous through microscopic defects
in the anterior lens capsule
Various theories try to explain how
this protein lead to raised IOP
Macrophages laden with
phagocytosed lens material block the
trabecular meshwork
High molecular weight soluble
protein which increases in
cataractous lens directly obstruct the
outflow of the aqueous
Macrophages in trabecular
meshwork

35. DIFFERENTIAL DIAGNOSIS OF
PHACOLYTIC GLAUCOMA
1. Primary acute angle closure glaucoma, ruled out by
gonioscopy
2. Open angle glaucoma associated with uveitis
 Difficult to differentiate clinically, paracentesis and
microscopic evaluation might be needed.
 A therapeutic trial of steroids will only provide temporary
remission if phacolysis is the underlying cause
3. Neovascular glaucoma
4. Trauma
5. Occult posterior segment tumour

36. MANAGEMENT OF
PHACOLYTIC GLAUCOMA
SHOULD BE HANDLED AS AN EMERGENCY, ULTIMATELY BY
REMOVAL OF THE LENS
It is desirable to first bring the IOP under control
Hyperosmotics
Carbonic anhydrase inhibitors
beta blockers
Alpha 2 agonists
Minimize associated inflammation with topical steroid
and cycloplegic therapy
If pressure cant be lowered medically, it can be done
at the time of surgery by gradual release of aqueous
through paracentesis

37. CATARACT SURGERY IN
PHACOLYTIC GLAUCOMA
Capsulorrhexis, lens delivery and residual cortical
aspiration has to be performed in an unusually
delicate manner to minimize zonular and capsular
stress
AC wash should be done thoroughly to remove all
the lens matter
Glaucoma usually clear and there is often return of
good vision with PCIOL despite a significant
preoperative reduction
A study on patients with Phacolytic glaucoma with
cataract surgery found IOP was less than 21mmHg
in all patients without use of antiglaucoma
medication

38. TRABECULECTOMY IN
MANAGEMENT OF
PHACOLYTIC GLAUCOMAIn a retrospective study eyes with Phacolytic glaucoma,
trabeculectomy was added along with standard cataract
surgery if
Symptoms endured for more than 7 days
Pre operative control of IOP with maximal medical treatment
was inadequate
At 6 months IOP and visual acuity did not vary in groups with
only cataract surgery compared to cataract surgery with
trabeculectomy
Trabeculectomy can be considered if the symptoms have
persisted for more than a week preoperatively to
Reduce the post operative rise In IOP
Decrease the need of systemic hypotensive medication

39. 2. LENS PARTICLE GLAUCOMA
Liberation of lens particle and debris after
disruption of lens capsule by trauma or
surgery, blocking the trabecular meshwork

40. CLINICAL FEATURES OF LENS
PARTICLE GLAUCOMA
Disruption of lens capsule by
surgery/penetrating injury
The onset of IOP elevation is right after the
primary incident
Significant pain, redness, diminution of vision
On examination corneal oedema, heavy cells
and flare, hypopyon and fluffy cortical matter in
anterior chamber
Lens particle glaucoma tend to have greater
inflammatory component
Posterior and anterior synechiae
Inflammatory pupillary membrane
Sometimes the onset of glaucoma may be
delayed by many years after capsular

41. MECHANISM OF LENS
PARTICLE GLAUCOMA
1. Perfusion studies in enucleated human eyes have
demonstrated that small amount of free particulate
lens material significantly reduce outflow
2. Associated inflammation, whether in response to
surgery/trauma/retained lens matter may contribute
to glaucoma in this condition
The severity of IOP elevation depends on
Quantity of lens material released
Degree of inflammation
Ability of trabecular meshwork to clear the cortical
matter
Functional status of ciliary body

42. DIFFERENTIAL DIAGNOSIS OF
LENS PARTICLE GLAUCOMA
In it typical form, lens particle glaucoma is easy to
diagnose on the basis of history and physical findings
In atypical forms like in cases of delayed onset and
spontaneous capsular rupture, differentiating it from
Phacolytic glaucoma and phacoanaphylaxis may be
challenging
When in doubt microscopic examination of aqueous from
anterior chamber may be helpful, demonstrating
leukocyte and macrophage along with lens cortical
matter.

43. MANAGEMENT OF LENS
PARTICLE GLAUCOMA
1. In some cases IOP can be controlled medically, drugs
reducing aqueous production should be used
2. Since inflammation is present, cycloplegic and steroids
can be given
3. lens matter should be surgically removed.
Intracameral alpha chymotrypsin
Steroids should be used in moderate amount and with
caution as it may delay absorption of cortical matter
IOP returns to normal only after lens material has been
absorbed/removed

44. 3. PHACOANAPHYLAXIS
Verhoff and Lemoine in 1922 reported that few
individuals were hypersensitive to lens protein.
Rupture of lens capsule in these cases led to an
intraocular inflammation, which they called
Endophthalmitis phacoanaphylactica.
Also known as phacoantigenic glaucoma/Phacogenic
uveitis

45. CLINICAL FEATURES OF
PHACOANAPHYLAXIS
Preceding disruption of lens capsule by
surgery or penetrating injury
Distinguishing feature is the latent
period, during which sensitization to the
lens protein occurs
Typical physical finding is chronic,
granulomatous type inflammation around
lens material in the involved eye or in the
fellow eye after it has undergone
extracapsular cataract surgery
glaucoma is rarely a feature of
phacoanaphylaxis
Commonly seen when lens material,
particularly nucleus, retained in vitreous
Sympathetic ophthalmia may occur
Exposed lens
material producing
granulomatous
inflammation

46. MECHANISM OF
PHACOANAPHYLAXIS
It has been demonstrated in rabbits that autologous lens
protein is antigenic, and lens capsule was assumed to
isolate the lenticular antigen.
This concept was not supported by human studies, which
failed to demonstrate lens antibodies after injury to the
lens and showed an equal incidence of antibodies in
patients with cataracts and controls
The same study did show a higher prevalence of antibodies
in small group with Hypermature cataracts and more
frequent postoperative uveitis in patients with antibodies in
preoperative blood specimens
Cellular reaction is characterized by polymorphonuclear
leukocytes, lymphoid, epithelioid and giant cells around a
nidus of lens material
Occasional glaucoma in phacoanaphylaxis may be related
to the accumulation of these cells in the trabecular

47. MANAGEMENT OF
PHACOANAPHYLAXIS
1. Steroid and cycloplegic therapy used to control the
uveitis
2. Antiglaucoma medication if needed
3. Surgical removal of lens material

48. 4. INTUMESCENT LENS
PHACOMORPHIC GLAUCOMA
In some eyes with advanced
cataract, lens may become
swollen or intumescent, with
progressive reduction in anterior
chamber angle
The angle closure may be caused
by an enhanced pupillary block
mechanism or by forward
displacement of lens iris
diaphragm
Diagnosed by observing a
mature, intumescent cataract
associated with central anterior
chamber depth, shallower than
the fellow eye
Shallow Anterior chamber with
corneal oedema

49. Shallow anterior chamber
Hypermature cataract
with shallow anterior
chamber
Anterior segment OCT
showing pupillary block

50. TREATMENT OF
PHACOMORPHIC GLAUCOMA
1. Medical reduction of IOP
Hyperosmotics
Carbonic anhydrase inhibitors
Topical beta blockers
Alpha 2 agonists
2. Extraction of the cataract
A study on patients with Phacomorphic glaucoma,
suggested that the acute angle closure attack can be
relieved in all cases with laser iridotomy before proceeding
with cataract surgery, but it is considered unnecessary by
most.
Chronic angle closure with formation of peripheral anterior
synechiae, goniosynechialysis in conjunction with cataract
extraction

51. CLINICAL FEATURES
Phacolytic glaucoma Lens protein glaucoma Phacoanaphylaxis
• Older patients
• Long history of poor
vision
• Sudden onset
monocular pain,
redness
• Markedly elevated IOP
• Microcystic corneal
oedema
• Prominent Anterior
chamber reaction with
cells and flare
• No KPs (no true KPs)
• Mature/Hypermature
cataract
• Cell debris in the angle
• Occurs within weeks
of initial surgery or
trauma usually
• Cortical material in
anterior chamber
• Elevated IOP
• Prominent anterior
chamber reaction
• Microcystic corneal
oedema
• Posterior
synechiae/periphera
l anterior synechiae
in long standing
cases
• Following
injury/surgery after a
latent period
• Moderate anterior
chamber reaction
• KPs
• Low grade vitritis
• Posterior
synechiae/peripheral
anterior synechiae in
long standing cases
• Residual lens material
in anterior chamber
• Glaucomatous
changes rare

52. AQUEOUS HUMOUR
MICROSCOPY
Phacolytic glaucoma Lens protein glaucoma Phacoanaphylaxis
• Lens material laden
macrophages
• Melanin laden
macrophages
• RBCs
• Ghost RBCs
• Macrophages
showing
erythrophagocytosis
• Free cell debris
• Macrophages
• Free lens material
• Polymorphonuclear
leukocytes
• Lymphocytes
• Epithelioid cells
• Giant cells
Method : Phase contrast microscopy or Millipore filtration and staining

53. MANAGEMENT
Pupillary
block in
subluxation /
dislocation
Phacolytic
glaucoma
Lens particle
glaucoma
Phacoanaphylax
is
Phacomorph
ic glaucoma
• Lens
repositioni
ng with
peripheral
laser
iridotomy
• Lens
extraction
• Laser
peripheral
iridoplasty
• IOP lowering
agents
• Steroids
• Cycloplegics
• Lens
extraction
• Trabeculecto
my
• IOP
lowering
agents
• Steroids
• Cycloplegi
cs
• Removal of
cortical
matter
• Intracamer
• Steroids
• Cycloplegics
• IOP lowering
agents
• Removal of
lens matter
• IOP
lowering
agents
• Cataract
extractio
n ±
Periphera
l Laser
iridotomy

54. THANK
YOU