Exfoliative glaucoma is a type of open-angle glaucoma caused by the buildup of exfoliative material in the eye. It develops as a complication of exfoliative syndrome, which is characterized by the progressive production and deposition of abnormal fibrillar material in ocular tissues. Exfoliative glaucoma presents with higher intraocular pressures that are difficult to control and a worse prognosis compared to primary open-angle glaucoma. Treatment involves medications, laser trabeculoplasty, and early surgical intervention such as trabeculectomy, as exfoliative glaucoma often responds poorly to medical and laser therapies.

1. PSEUDOEXFOLIATIVE
GLAUCOMA
DR MITHUN KISHORE

2. EXFOLIATIVE SYNDROME AND EXFOLIATIVE GLAUCOMA
• Vogt introduced the term ‘exfoliation superficialis capsulae anteriores’
assuming that the flocculent material arose as small flecks peeling off
from the lens capsule.
• Dvorak Theobald called it pseudoexfoliation to distinguish it from
true exfoliation.
• Studies have shown that lens capsule as one of the sources of
exfoliative material and thus termed it as ‘exfoliation syndrome’ or
pseudoexfoliative syndrome and ‘Exfoliative glaucoma’ when
associated with glaucoma.

3. Exfoliative syndrome (XFS) is an age
related,generalized disorder of the extracellular matrix,
an elastic microfibrillopathy characterized by
progressive production and deposition of abnormal
fibrillar material in ocular and extraocular tissues.

4. EPIDEMIOLOGY
- AGE
Prevalence of XFS increases with age
- GENDER
• Females have increased predisposition for XFS without glaucoma
• XFG develops earlier ,more frequently and in increased severity
among males
PREVALENCE AGE GROUP
0.6% <65 YRS
2.6% 65-74 YRS
5 % 75-85 YRS

5. PREVALENCE OF OHT AND GLAUCOMA IN XFS
• 25% of patients with XFS have elevated IOP
• Patients with XFS and OHT are twice likely to convert to glaucoma.
PREVALENCE OF EXFOLIATION GLAUCOMA IN OPEN ANGLE GLAUCOMA
• 20-40% of patients with open angle glaucoma have XFG .
PREVALANCE OF EXFOLIATIVE GLAUCOMA IN ANGLE CLOSURE GLAUCOMA
• Glaucomatous optic neuropathy was found in 28.3% of XFS cases with
primary angle closure glaucoma or occludable angle.

6. RISK FACTORS FOR CONVERSION FROM XFS TO XFG
• Increased Intraocular pressure
• Presence of XFM in the angle
• Presence of XFM in the other eye
• Decreased pupillary dilation
• Increased trabecular meshwork pigmentation.

7. • ETIOLOGY AND PATHOGENESIS
• GEOGRAPHICAL FACTORS
• High prevalence rate in Nordic countries suggests that a northern
latitude,cold air contribute to development of XFS
• GENETIC FACTORS
• Thorleifsson et al identified single nucleotide polymorphism (SNP)
associated with XFS.
• Three single nucleotide polymorphism in the coding region of LOX L1
gene located on chromosome 15q24 were strongly associated with XFS
and XFG.

8. MICROSCOPIC STRUCTURE OF XFM
• It consisits of a tangle of fibrils and filaments embedded in an
amorphous matrix.
• Fibrils are composed of microfibrillar subunits surrounded by an
amorphous matrix.

9. CHEMICAL STRUCTURE
• It has a protein core surrounded by glycoconjugates
(glycosaminoglycans) which form the amorphous ground substance.
• Protein component includes non collagenous basement membrane
components and epitopes of elastic fibre system.
• Fibrillin -1 – Main component of elastic microfibrils has been
demonstrated in exfoliation fibres and their microfibrillar subunits.

10. • The latent tumor growth factor beta binding proteins (LTBP-1 andLTBP-2)
 Associated with all exfoliative deposits and co-localized with latent TGF
on exfoliation fibers.
 These LTBPs may have a dual role
a) structural component of exfoliation fibers
b) matrix anchorage of latent TGF β1 to XFM.
• The HNK-1 epitope - involved in the adhesiveness of exfoliation material
deposits on intraocular surfaces acting like a glue and binding the various
components together and to the ocular structures.

11. •BIOCHEMICAL CHANGES IN AQUEOUS HUMOUR
IN XFS
• -Increased TGF beta1-responsible for overproduction of extracellular
matrix.
• -Increased level of matrix metalloproteinases(MMP)
• -Elevated level of Homocysteine from disturbed methionine metabolism
associated with disruption of elastic fibre component of extracellular
matrix.

12. • THEORIES OF PATHOGENESIS OF XFS
AMYLOID THEORY
• XFM constituted of crude anti-amyloid serum suggesting amyloid
deposists.
• This theory could not be supported by further tests.
BASEMENT MEMBRANE THEORY
• Exfoliative material have been associated with basement membranes
of cell types.
• Immunohistochemical studies reveal presence of basement
membrane epitopes like laminin, heparin sulphate in XFM aggregates.

13. • ELASTIC MICRIFIBRIL THEORY
• This theory was based on structural association of XFM associated
with components of elastin system like zonules.
• Immunohistochemical studies demonstrates epitopes of elastin
supports this theory.
• INFECTIOUS ORIGIN THEORY
• XFM after intraocular surgery or ocular trauma suggested the
possibility of this theory
• XFS after penetrating keratoplasty with grafts from elderly supports
this theory.
• No conclusive evidence to support this theory.

14. STRESSED INDUCED ELASTOSIS THEORY
• XFS is a type of stress induced elastosis
• Associated with excessive production of elastic microfibrills by
elastogenic cells which aggregate to form exfoliative fibrils.
• PROTEIN SINK MODEL THEORY
• Soluble proteins found in biological fluids form insoluble structures
that accumulate to form intra and extracellular aggregates which
form fine dusting on surfaces like lens capsule and angle of anterior
chamber.

15. • SOURCES OF EXFOLIATIVE MATERIAL
INTRAOCULAR EXTRAOCULAR
Pre-equatorial lens
epithelium
Fibroblasts
Non pigmented ciliary
epithelium
Smooth and striated
muscle cells
Trabecular endothelium Cardiac muscle cells
Vascular endothelium

16. • EXTRAOCULAR AND SYSTEMIC SITES OF XFM DEPOSITS
EXTRAOCULAR
SITES
SYSTEMIC
SITES
Conjunctiva skin
Orbital tissues Visceral organs
Blood vessels wall
Meninges

17. • PATHOGENESIS OF GLAUCOMA IN EXFOLIATIVE SYNDROME
In open angle glaucoma
Exfoliation material produced locally in the trabecular
meshwork and passive accumulation via aqueous
disorganization and degeneration of juxtacanalicular and schlemm’s canal
Focal collapse of the schlemm’s canal
Decreased aqueous outflow
Increased intraocular pressure

18. • In angle closure glaucoma
Shallow anterior chamber with zonular weakness
forward movement of lens
pupillary block
increase in intraocular pressure

19. CLINICAL FEATURES
EARLY SIGNS
 PUPILLARY MARGIN
XFM deposited on or behind
the pupillary margin with
loss of pupillary ruff.

20. LENS
Uniform ,homogenous/ground glass
appearance of the anterior lens
surface.
PUPIL AND IRIS
• Atrophy of the iris pigment
epithelium
• Loss of pupillary ruff
• Poor mydriasis
• Pigment dispertion after pupillary
dilatation.

21.  CILIARY PROCESSES
Deposition of exfoliative material in the ciliary processes is one of
the earliest feature seen on indirect ophthalmoscopy with
indentation.
SIGNS IN ESTABLISHED XFS
 LENS
Deposition of XFM on lens shows a classical pattern which
Consists of 3 zones which is visible after dilatation

22.  Central disc
• Corresponds to diameter of pupil.
• Homogenous white sheet with rolled up edges with normal
underlying lens capsule and epithelium.
• Absent in 20 % of cases.

23.  Granular peripheral zone
• Extends equatorially as tongue shaped projections which
merge into normal capsule before reaching the anterior
insertion of zonules.
 Intermediate zone
• 1-2 mm of clear zone devoid of XFM which is formed as a
result of iris scraping the exfoliation material from the
surface of the lens during pupillary movement

24.  Pre equatorial zone
• This zone is clinically hidden by iris.
• It consists of nodular excrescences covering zonules and
their attachments.
Three zones or Bull’s eye pattern seen on the anterior lens capsule on
retroillumination

25.  PHACODONESIS
Due to spontaneous subluxation and dislocation of the lens due to
weakness of zonules secondary to deposition of XFM.
 CATARACT
Increased prevalence of cataract (nuclear and subcapsular cataract)
Cause – ocular ischemia and oxidative damage have been proposed.

26. • IRIS
• Deposition of exfoliative material at the pupillary margin.
• Pigment loss from iris and its deposition on anterior segment structures.
• Transillumination defects at pupillary margin secondary to pigment loss from
iris.
• Diffuse pigment deposition on iris surface.
IRIS TRANSILLUMINATION DEFECT

27. PUPIL
Eyes with XFS dilate poorly and constrict poorly with pilocarpine due
to fibrotic and degenerative changes in the iris sphincter and dilator
pupillae.
EYES AFTER INSTALLATION OF MYDRIATIC

28. PEX MATERIAL DEPOSITION ON CORNEAL
ENDOTHELIUM
CORNEAL SIGNS
• Diffuse non specific pigmentation of
central endothelium in the pattern of
krukenberg spindle.
• Altered endothelial cell count and
morphology.
• Keratopathy characterised by
 Diffuse ,irregular thickening of
descemet’s membrane.
 Focal accumulation of exfoliation
material on/ within descemets
membrane.

29. ANTERIOR CHAMBER ANGLE
• Increased trabecular
pigmentation is a early sign on
gonioscopy.
• Pigment deposited on
Schwalbe’s line and anterior to
it (Sampaolesi’s line).

30. OPTIC NERVE HEAD AND RNFL LAYER
• The mean optic disc area has been reported to be smaller in eyes with
XFS and exfoliative glaucoma.
• Diffuse cupping compared to POAG.
• The percentage area of optic disc pallor in XFS patients greater than
controls.
• The RNFL has been found to be significantly thinner in XFS eyes

31. Clinical Presentations of XFG
• Secondary open angle glaucoma
• Normal tension glaucoma
• Acute open angle glaucoma
• Angle closure glaucoma
• Neovascular glaucoma

32. • Secondary Open Angle Glaucoma
• Exfoliation glaucoma (XFG) can present as open angle glaucoma
occurring due to the deposition of exfoliative material in the
trabecular meshwork.
• Early failure of medical therapy and late failure of laser
trabeculoplasty is common and these cases may require an early
surgical intervention.
• It mimics primary open angle glaucoma.

33. Differentiating features of XFG from POAG
• More aggressive
• Worse prognosis
• Higher mean IOP
• More diurnal fluctuations with marked pressure spikes
• Marked optic nerve damage
• Rapid visual field loss

34. • Poor response to medications
• Frequent need for surgical intervention
• Asymmetric presentation
• Postmydriasis IOP spikes
• Pigmentation in anterior chamber
• Greater rate of conversion from ocular hypertension

35. • ‘Normal Tension’ Exfoliation Glaucoma
• Higher maximum IOP level and a greater IOP fluctuation within
normal range could be significant risk factors for development of
glaucomatous ONH changes in eyes with exfoliation in the presence
of a normal pressure.
• Non-IOP dependant factors include.
 impaired ocular and retrobulbar perfusion
Abnormalities of elastic tissue of the lamina cribrosa.

36. •Acute Open Angle Glaucoma
• Dispersion of pigment granules and XFM in the anterior
chamber after diagnostic pupillary dilation lead to marked
rise in IOP causing diffuse corneal edema producing a
deceptive clinical picture of acute primary angle closure
glaucoma.
• Presents with IOP rise of more then 50 mm Hg in spite of an
open angle.

37. • Angle Closure Glaucoma
Mechanisms proposed are
Narrow anterior chamber angle, smaller anterior chamber volume,
along with a minimal subluxation of lens which predisposes to
pupillary block.
Formation of posterior synechiae due to a deranged blood aqueous
barrier leading to pupillary block.

38. • Neovascular Glaucoma
• CRVO is more common in patients with exfoliation.
• Patients with CRVO can present as neovascular glaucoma.

39. • DIFFERENTIAL DIAGNOSIS
• Pigment dispersion syndrome and pigmentary glaucoma
• Disorders associated with melanin dispersion in the anterior segment
 Uveitis
 Trauma
 Surgery(ab interno surgeries)
 Diabetes
• True exfoliation of the lens

40. MANAGEMENT OF XFG
Medical Management
AIM – To decrease mean IOP and its fluctuations.
Features
• Poor response to medical therapy
• IOP often cannot be controlled with monotherapy, thus combination
therapy is preferred

41.  Aqueous Suppressants
• Beta blockers are effective in controlling initial IOP but significant
diurnal fluctuations have been noted in XFG in contrast to POAG.
• Carbonic anhydrase inhibitors(Dorzolamide) is also effective as timolol
and also has an additive action in combination therapy but should be
avoided in patients with compromised corneal endothelium.
• Alpha adrenergic agonists like brimonidine may also be used in
combination therapy.

42.  Prostaglandin Analogs
• Prostaglandin analogs increases the aqueous outflow via the
uveoscleral pathway have been found to be effective in lowering IOP
in exfoliation glaucoma.
• Latanoprost provide a better control of diurnal fluctuation as
compared to timolol.
• Both latanoprost and travoprost have been found to reduce IOP at
each time point in a 24 hour study.

43.  Pilocarpine
• Lower the IOP by increasing the aqueous outflow.
• Pilocarpine limits the pupillary movement, thus prevents iridolenticular
friction and pigment release .
• Hazards of pilocarpine use
- aggravation of the blood aqueous barrier dysfunction
- development of posterior synechiae by restricting pupillary
movement
- aggravation of lens opacities and ciliary block glaucoma in eyes with
marked zonular instability.

44. • Inter-national Collaborative Exfoliation Syndrome Treatment (ICEST)
study examined treatment with latanoprost and pilocarpine vs timolol
vs fixed-combination timolol and dorzolamide in eyes with exfoliation
syndrome and elevated IOP found that the latanoprost and
pilocarpine lowers IOP and improved aqueous outflow.

45. Lasers
• XFG patients respond well to laser trabeculoplasty due to the high baseline
IOP as well as the heavily pigmented trabecular meshwork
• Patients with higher baseline IOP, the drop in IOP after ALT is greater in
these eyes than in non-XFG eyes.
• The success rate varies from 70 to 90 % with IOP reduction upto 30%.
Inflammatory reaction and IOP spikes are frequently seen.
• The patients require a IOP lowering and anti-inflammatory medications in
the early post laser period.
• Late failure of laser treatment presenting with high IOP, unexpected disc
damage and visual field progression.

46. • Selective laser trabeculoplasty
- selectively targets the intracellular melanin granules in the
trabecular meshwork cells.
- IOP reduction is more pronounced if prelaser IOP is high.
- can be done after failed ALT.
• Peripheral laser iridotomy is performed when angle closure is present
along with exfoliation

47. •SURGICAL MANAGEMENT
• Required earlier and more frequently as compared to
other forms of glaucoma due to the early failure of
medical and laser treatment.
• Surgical options available are
- Traeculectomy
- Non penetrating glaucoma filtering surgery(NPGS)
- cataract surgery
- combined cataract and glaucoma surgery

48. TRABECULECTOMY
• Trabeculectomy with antimetabolites is the surgery of choice.
• PEXG treated with trabeculectomy associated with increases
perioperative and early postoperative complications like
- Increased risk of choroidal haemorrhage/effusion
. - Increase risk of haemorrhage due to iris vasculopathy at the site of
peripheral iridectomy.
- vitreous obliteration of trabeculectomy opening in eyes with loose
zonules and anterior movement of the lens.
- Increased risk of fibrinous reaction post op period.

49. NON PENETRATING GLAUCOMA
SURGERY
• Avoids unfavourable bleb related complications and
decreases incidence of post operative inflammation.
• Commonly done Non penetrating glaucoma surgeries are
- Trabeculectomy ab externo
- Deep sclerectomy

50. COMBINED CATARACT AND GLAUCOMA SURGERY
• Indicated in patients with coexistant cataract and XFG.
• It is associated with
- Greater incidence of spontaneous lens displacement and capsular
rupture.
- Increased incidence of zonular dehiscence and vitreous loss.
- Increased incidence of post capsular opacification.

51. CATARACT SURGERY
Cataract surgery alone is sufficient in controlling IOP in eyes with little or no
glaucoma damage or in eyes with well controlled glaucoma on minimal
medications.
PREOPERATIVE ASSESSMENT
 Pupillary dilation
 Phacodonesis
 Control of glaucoma
 Zonular status

52. • INTRAOPERATIVE CONSIDERATIONS
 SMALL PUPIL
• Preoperative NSAIDS given to prevent intraoperative miosis.
• Posterior synechiae if present should be lysed with
capsularhexis forceps.
• Pupil can be expanded using
- Cohesive ophthalmic viscosurgical device.
- Pupil expansion device.

53.  ZONULAR WEAKNESS
Dependind on severity of zonular dialysis following
can be used
 Capsular tension ring(CTR)
 Cionni modified CTR(m CTR)
 Capsular tension segment(CTS)
 HYDRODISSECTION AND VISCODISSECTION
 Hydrodissection performed with minimal stress on zonules.
 OVD injected between the lens capsule and cortex to prevents
trapping of cortex during CTR insertion and also provides greater
corticocapsular cleavage than hydrodissection alone.

54.  IOL INSERTION
One piece acrylic IOL of choice- this IOL design allows
surgeon to dial the IOL gently into capsular bag and
place felxible haptic in desired orientation with
minimal capsular and zonular stress.

55. THANK YOU