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PSEUDOEXFOLIATION
SYNDROME
Presenter: SHAYRI PILLAI
Liberia Eye Centre
JFK Memorial Medical Centre
L V Prasad Eye Institute
14/APRIL/21
Introduction
» Pseudoexfoliative material is a grey-white fibrillary
substance
» Age related multifactorial, progressive disorder
» Material is deposited on various ocular structures
includes-
- Lens capsule
- Zonular fibres
- Iris
- Trabeculum
- Conjunctiva
Deposits in blood vessels,skin, gall bladder,
kidneys,lungs,heart and meninges

Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Aus
tralia
Skuta,G.L. et.Al. American Academy of Ophthalmology Glaucoma 2020 Edition.USA
» Pseudoexfoliation syndrome (PXS)
- Important ocular manifestation of a systemic disorder
- Commonest cause of secondary open-angle glaucoma
- Rare before the age of 50 years
- Prevalence increases rapidly after the sixth decade of
life, with a prevalence of 5% at age 75–85 years
- More common in women than men
- Incidence of glaucoma (PXG) at diagnosis of PXS is
15–30%
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Au
stralia
Pathogenesis
Multifactorial Pathogenesis
- Imbalance between MMPs and endogenous inhibitors
- Increased oxidative stress
- Altered cellular stress
Gene-environment interactions
- Single nucleotide polymorphism in lysyl oxidase like
(LOXL1) gene
- CACNA1A
Allingham, R. et al. Shields TextBook of Glaucoma, 6th Ed., 2011
Usually due to elevated IOP secondary to trabecular
obstruction by pseudo exfoliative material and liberated iris
pigment leading to secondary degenerative outflow
dysfunction
Pseudo exfoliative material in the apparently unaffected
eye, glaucoma remains confined to one eye in about two-
thirds of patients
Clinical features
Diagnosis is usually incidental, but can follow vision loss
from advanced glaucoma
Cornea
- Pseudoexfoliative material may be deposited on the
endothelium and scattered pigment deposits are
common
- Vertical (Krukenberg) spindle
- Endothelial cell abnormality, such as low density, is
more common than average
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018.
Australia
Anterior chamber
- Pseudoexfoliative material particles are sometimes seen
- Mild aqueous flare from an impaired blood– aqueous barrier
common
Iris
Granular pseudoexfoliative material deposits, pupillary ruff loss
and patchy transillumination defects at the pupillary margin
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Aust
ralia
Anterior chamber angle
- Patchy trabecular and Schwalbe line hyperpigmentation is
common, especially inferiorly
- Sampaolesi line, which is an irregular band of pigment running
on
or anterior to the Schwalbe line, is commonly seen
- Dandruff-like pseudoexfoliative material deposits may be seen
- Increased risk of angle closure probably because of zonular
laxity
IOP
Majority of patients have a chronic open-angle glaucoma that is
usually unilateral at first
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Austra
Sequelae of PXF
» Loss of zonular integrity leads to
- Narrow angles
- Subluxation/Dislocation
- Increased complications during cataract surgery
- Decentration of IOL and capsular bag
- Capsular Contraction
- PCO
Prognosis
- Worse than POAG as the IOP is usually higher and may
exhibit marked fluctuation
- Patients are at significant risk of visual loss and blindness
- Monitor patients closely
- Review patients with PXS at intervals of no more than 6
months
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Austr
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Au
Treatment
- Medical treatment is similar to that of POAG, but failure
is more common
- Laser trabeculoplasty
- Filtration surgery
- Trabecular aspiration
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Au
Challenges in cataract surgery
» Corneal Endotheliopathy
» Poor mydriasis
» Zonular instability and lens subluxation
» Increase risk of vitreous loss
» Increased IOP and inflammation
» Late IOL decentration and dislocation
Thank you!
Excellence Equity Efficiency
L V Prasad Eye Institute

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pxf.pptx

  • 1. PSEUDOEXFOLIATION SYNDROME Presenter: SHAYRI PILLAI Liberia Eye Centre JFK Memorial Medical Centre L V Prasad Eye Institute 14/APRIL/21
  • 2. Introduction » Pseudoexfoliative material is a grey-white fibrillary substance » Age related multifactorial, progressive disorder » Material is deposited on various ocular structures includes- - Lens capsule - Zonular fibres - Iris - Trabeculum - Conjunctiva Deposits in blood vessels,skin, gall bladder, kidneys,lungs,heart and meninges  Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Aus tralia
  • 3. Skuta,G.L. et.Al. American Academy of Ophthalmology Glaucoma 2020 Edition.USA
  • 4. » Pseudoexfoliation syndrome (PXS) - Important ocular manifestation of a systemic disorder - Commonest cause of secondary open-angle glaucoma - Rare before the age of 50 years - Prevalence increases rapidly after the sixth decade of life, with a prevalence of 5% at age 75–85 years - More common in women than men - Incidence of glaucoma (PXG) at diagnosis of PXS is 15–30% Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Au stralia
  • 5. Pathogenesis Multifactorial Pathogenesis - Imbalance between MMPs and endogenous inhibitors - Increased oxidative stress - Altered cellular stress Gene-environment interactions - Single nucleotide polymorphism in lysyl oxidase like (LOXL1) gene - CACNA1A Allingham, R. et al. Shields TextBook of Glaucoma, 6th Ed., 2011
  • 6. Usually due to elevated IOP secondary to trabecular obstruction by pseudo exfoliative material and liberated iris pigment leading to secondary degenerative outflow dysfunction Pseudo exfoliative material in the apparently unaffected eye, glaucoma remains confined to one eye in about two- thirds of patients
  • 7. Clinical features Diagnosis is usually incidental, but can follow vision loss from advanced glaucoma Cornea - Pseudoexfoliative material may be deposited on the endothelium and scattered pigment deposits are common - Vertical (Krukenberg) spindle - Endothelial cell abnormality, such as low density, is more common than average Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Australia
  • 8. Anterior chamber - Pseudoexfoliative material particles are sometimes seen - Mild aqueous flare from an impaired blood– aqueous barrier common Iris Granular pseudoexfoliative material deposits, pupillary ruff loss and patchy transillumination defects at the pupillary margin Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Aust ralia
  • 9. Anterior chamber angle - Patchy trabecular and Schwalbe line hyperpigmentation is common, especially inferiorly - Sampaolesi line, which is an irregular band of pigment running on or anterior to the Schwalbe line, is commonly seen - Dandruff-like pseudoexfoliative material deposits may be seen - Increased risk of angle closure probably because of zonular laxity IOP Majority of patients have a chronic open-angle glaucoma that is usually unilateral at first Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Austra
  • 10. Sequelae of PXF » Loss of zonular integrity leads to - Narrow angles - Subluxation/Dislocation - Increased complications during cataract surgery - Decentration of IOL and capsular bag - Capsular Contraction - PCO
  • 11. Prognosis - Worse than POAG as the IOP is usually higher and may exhibit marked fluctuation - Patients are at significant risk of visual loss and blindness - Monitor patients closely - Review patients with PXS at intervals of no more than 6 months Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Austr
  • 12. Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Au
  • 13. Treatment - Medical treatment is similar to that of POAG, but failure is more common - Laser trabeculoplasty - Filtration surgery - Trabecular aspiration Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2018. Au
  • 14. Challenges in cataract surgery » Corneal Endotheliopathy » Poor mydriasis » Zonular instability and lens subluxation » Increase risk of vitreous loss » Increased IOP and inflammation » Late IOL decentration and dislocation
  • 15. Thank you! Excellence Equity Efficiency L V Prasad Eye Institute

Editor's Notes

  1. Pseudoexfoliative material is a grey-white fibrillary substance deriving from abnormal extracellular matrix metabolism in ocular and other tissues. Also call glaucoma capsulare LOXL1 mutations are related to pseudoexfoliation syndrome but it likely involves the reduced or abnormal synthesis of elastin fibers. Single nucleotide polymorphisms Pseudoexfoliative material has been found in skin and visceral organs, leading to the concept that PXS is the ocular manifestation of a systemic disorder PXS is associated with an increased prevalence of high-tone hearing loss and cardiovascular disorders
  2. Pseudoexfoliative material has been found in skin and visceral organs, leading to the concept that PXS is the ocular manifestation of a systemic disorder. PXS is associated with an increased prevalence of high-tone hearing loss and cardiovascular disorders.
  3. Several hypothesis. Matrix metalloproteinases endogenous inhibitors are protein inhibits formatn of blood vessels.Altered cellular stress leads to degeneration of tissues. Also CACNA 1A is cause of exfoliation in humans(familyof genes provide instructions for making calcium channels
  4. Pseudoexfoliative material is a grey-white fibrillary substance deriving from abnormal extracellular matrix metabolism in ocular and other tissues. Also call glaucoma capsulare Pseudoexfoliative material has been found in skin and visceral organs, leading to the concept that PXS is the ocular manifestation of a systemic disorder PXS is associated with an increased prevalence of high-tone hearing loss and cardiovascular disorders
  5. LOXL1 mutations are related to pseudoexfoliation syndrome but it likely involves the reduced or abnormal synthesis of elastin fibers. Single nucleotide polymorphisms
  6. LOXL1 mutations are related to pseudoexfoliation syndrome but it likely involves the reduced or abnormal synthesis of elastin fibers. Single nucleotide polymorphisms
  7. Ocular ischemia-iris ischemia with microneovascularisation is seen Hyphema on table or post operative period.chronic break down of blood aqueous barrier
  8. Argon laser trabeculoplasty
  9. Specular microscopy .Nuclear cataract more common with pxf