Dr Suresh SubramaniamOphthalmology Department

Seoul National University Hospital20 may 2016

Pseudoexfoliative Glaucoma

Estimated, the global prevalence of glaucoma to be 3.54%◦ The number of people with glaucoma worldwide

(aged 40-80 years) will increase from 64.3 million in 2013 to 111.8 million in 2040

1st leading cause of irreversible blindness◦ 4.5 million people open-angle glaucoma◦ 3.9 million people angle-closure glaucoma

These numbers are set to rise to 5.9 and 5.3 million, respectively, by 2020

Glaucoma

Global Prevalence of Glaucoma and Projections of Glaucoma Burden through 2040Tham, Yih-Chung et al. ;Ophthalmology , Volume 121 , Issue 11 , 2081 - 2090

True◦ True exfoliation or lamellar delamination of the

lens capsule Pseudo

◦ Deposition of a protein-like material within the anterior segment of the eye Whitish-gray protein

Lens Iris Ciliary epithelium Corneal endothelium Trabecular meshwork

Pseudoexfoliation

Pseudoexfoliation syndrome Pseudoexfoliative glaucoma

• Presence of typical pseudoexfoliation material on the anterior lens capsule in one or both eyes

• A normal optic disk

appearance and visual field testing (Humphrey 30-2 SITA standard test)

• IOP < 21 mm Hg

• Pseudoexfoliative material on the anterior lens capsule

• Typical glaucomatous cupping and visual field loss in one or both eyes

• IOP > 21 mm Hg

Serpil Yazgan et al; (2015) Corneal Biomechanical Comparison of Pseudoexfoliation Syndrome, Pseudoexfoliative Glaucoma and Healthy Subjects, Current Eye Research, 40:5, 470-475

Definition : PXG Pseudoexfoliation syndrome (PXS) is a systemic condition with

eye manifestations.

◦ Deposits on various structures of the anterior segment.

The nature of this material is mostly fibrillar with fibers made up of microfibrils and coated with amorphous material.

Fibrils is diverse and includes basement membrane components enzymes involved in extracellular matrix maintenance.

Pseudoexfoliation is the most common cause of secondary open-angle glaucoma worldwide.

Pseudoexfoliative Glaucoma, PXG

Late onset condition with prevalence that increases markedly with age

Generally more prevalent in women than in men

In Japan the incidence of PXS increased with age from 0.7% in ages 50 to 60 years to 7.3% in ages over 80 years.

Demographics

Ritch R: Exfoliation syndrome: clinical findings and occurrence in patients with occludable angles. Trans Am Ophthalmol Soc. 1994, 92: 845-944

Evidence suggests a strong genetic component to this condition.

PXS has been strongly associated with single nucleotide polymorphisms (SNPs) of the lysyl oxidase-like 1 (LOXL1) gene on chromosome 15q24.1

Potential Genetic Disease

Elhawy E et. al.;Pseudoexfoliation syndrome, a systemic disorder with ocular manifestations. Human Genomics 2012 6:22

Non-genetic factors correlated with exfoliation syndrome◦ exposure to ultraviolet radiation◦ immunological or virus involvement ◦ transmission through keratoplasty◦ nutrition – selenium, caffeine etc

Nongenetic factors

PXM fibrils contain components of the elastic fiber and basement membrane system

It is likely that PXM accumulation is a result of a combination of both excessive synthesis and insufficient degradation.

Pathology and Nature

Elhawy E et. al.;Pseudoexfoliation syndrome, a systemic disorder with ocular manifestations. Human Genomics 2012 6:22

Current Concept Of The Pathogenesis Of Exfoliation Syndrome

Increased out flow resistance at TM◦ Blockage by PXM

The side of accumulation and pathological alteration occurs at juxtacanalicular tissue beneath inner wall of Schlemm`s canal Site of greatest resistance

Thickened through gradual deposition of PXM Gradual build up of PXM at juxtacanalicular

tissue correlates with IOP level◦ Development of Glaucomatous Optic Nerve

damage

Mechanisms of Glaucoma

The age of the patient Asymmetry and possible unilateral

Diagnosing the Disease

Deposits on the anterior capsule, in a manner resembling a target

Small white fibrillar deposits on the anterior capsule or on the pupil margin

Pigmentation beyond the expected level Peripupillary atrophy Asymmetry in the signs Poorly dilating pupils

Cataract Ocular ischemia

◦ Iris ischemia Retinal Vein Occlusion

Ocular Association

PXM was identified in the wall of greater vessels

Found in the heart, lung, liver, kidney, gall bladder and cerebral meninges

Association with specific diseases◦ TIA◦ HPT◦ MCI◦ Stroke◦ Alzheimer`s◦ Hearing loss

Systemic Association

IOP reduction ◦ Same as POAG treatment

LASER◦ Laser trabeculoplasty delay the use of medical

therapy Surgical Approach

◦ Trabeculectomy has comparable to those in POAG Surgical complication more common Pre op high IOP predispose

Choroidal hemorrhage or effusion Weakened zonules-> lens movement / subluxation Hyphaema

Management

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