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    Pseudoexfoliation Syndrome

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    Pseudoexfoliation Syndrome

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    Faculty of Medicine

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    Pseudoexfoliation syndrome

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    Faculty of Medicine

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    10 views22 pages

    Pseudoexfoliation Syndrome

    Uploaded by

    Version 1
    Faculty of Medicine

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 22

    Outlines (01-06)

    01 02 03

    Definition
    Case
    Approach & Signs

    04 05 06

    Symptoms
    Causes / Risk factors Treatment / Management
    Case
    Objective I
    Case 1
    A 68-year-old male patient presents to the ophthalmology clinic with complaints of gradually
    worsening vision in both eyes over the past year. He has noticed increased difficulty with glare,
    especially when driving at night, and mentions that bright lights cause significant discomfort. The
    patient reports no significant pain, redness, or discharge from the eyes.

    Medical History:
    - Hypertension, managed with medication.
    - No known history of diabetes.
    - Not using any eye drops
    - No prior ocular surgeries or significant trauma.
    - Family history is notable for glaucoma in his mother.

    Examination:
    - VA cc 20/40 in the right eye, 20/50 in the left eye.
    - Intraocular Pressure (IOP): with I-care, Right eye 24 mmHg, left eye 26 mmHg.
    - EOM: full and Pupil RRR with no RAPD
    Case 1
    - Slit-Lamp Examination:
    - LL: WNL
    - Cornea: Clear, no signs of corneal edema.
    - Conj/sclera: WNL
    - Anterior Chamber: Deep and quiet.
    - Iris:Presents with a patchy atrophy pattern and transillumination defects.
    - Lens: shown later
    - Pupil: Mildly sluggish reaction to light.
    - Fundus Examination Optic nerve head shows cupping with a cup-to-disc ratio of
    0.7 in both eyes. Flat retina, No signs of retinal detachment or hemorrhage.
    Question

    What is the diagnosis and what is the


    expected findings in the gonioscopy?
    Pseudoexfoliation Syndrome
    Presented by:
    Eyad Fatani
    Omar Khawaji

    Supervised by: Dr Hanan Jamjoum


    Case 1
    Gonioscopy Findings
    - Open angles with moderate pigmentation of the trabecular meshwork.
    - Presence of the same grayish-white material in the angle structures.
    - may also note weak zonules during dynamic gonio

    Special Tests
    - Visual Field Testing: Shows arcuate scotomas consistent with glaucomatous
    damage.
    - Optical Coherence Tomography (OCT): Thinning of RNFL in both eyes.

    ---
    Pseudoexfoliation

    Definition
    Pseudoexfoliation syndrome is a systemic condition characterized by the accumulation of a distinctive
    fibrillar material on ocular tissues, particularly the lens capsule, iris, and trabecular meshwork. It is a
    leading cause of secondary open-angle glaucoma.

    Epidemiology
    Prevalence: Common in individuals over 60 years old, with prevalence increasing with age.
    More prevalent in Scandinavian countries, Lower prevalence in African and Asian populations.
    Slightly more common in females than males.

    Etiology (mostly unknown)


    Genetic Factors: Linked to polymorphisms in the LOXL1 gene.
    Links to Environmental Factors?: Possible association with ultraviolet light exposure.
    Age-Related Changes: Degeneration of extracellular matrix components in ocular tissues.
    Approach and Signs
    Objective III
    Pseudoexfoliation syndrome

    Approach

    ● Patient History: Assess for symptoms like decreased vision, glare, and family history
    of glaucoma. Also inquire about any systemic conditions and medication history.

    ● Eye Examination:
    Slit-Lamp Examination: we will look for characteristic fibrillar material on the
    anterior lens capsule and pupillary margin.
    Intraocular Pressure (IOP) Measurement: Elevated IOP is common.
    Gonioscopy: Check for pigment dispersion and pseudoexfoliative material in the
    angle.
    Pseudoexfoliation syndrome

    Approach
    ● Visual Field Testing: To assess for glaucomatous damage.
    OCT: Evaluate retinal nerve fiber layer (RNFL) thickness.

    ● Monitoring and Follow-Up: regular monitoring of IOP and optic nerve head.
    Assess progression of glaucoma and lens changes.

    ● Characteristic Ocular Signs:


    ● Lens Capsule: Presence of grayish-white, flaky material on the anterior lens capsule in a bull’s eye
    pattern.
    Pupillary Border: Irregular, flaky deposits on the pupillary margin.

    Anterior Segment Changes: Iris Atrophy and Patchy iris transillumination defects.
    Zonular Weakness: May lead to lens subluxation.
    PEX material detected earliest in
    ciliary processes and zonules

    When it involves zonules it could


    cause lens subluxation and
    phacodonesis

    It aggregates in the origin and


    anchorage of zonules and invades
    zonular lamella creating weakness
    Pseudoexfoliation syndrome

    Approach
    Angle and Trabecular Meshwork:

    ● Pigment Dispersion: Increased pigmentation of the trabecular


    meshwork.
    ● Pseudoexfoliative Material: Observed in the angle structures.
    ● Image shows trabecular hyperpigmentation (most marked- inferior)
    patchy distribution

    Optic Nerve Changes

    ● Cupping: Increased cup-to-disc ratio indicating glaucomatous


    damage.
    ● Visual Field Defects: Arcuate scotomas typical of glaucoma.
    Risks: Causes :

    •Age over 50 - Genetic :


    •Genetic ( family ) - Glaucoma :
    angle-closure glaucoma more series than open
    •North European : high prevalence in Scandinavia
    A: Abnormal appears on the anterior lens capsule like iron filings on the edge of a magnet

    B: The iris pigment epithelium “saw-toothed”

    Pathophysiology ●Single-nucleotide polymorphisms (SNPs) in the coding region of the lysyl-


    oxidase-like 1 (LOXL1) gene, which is responsible for cross-linking of elastin,
    have been identified as strong genetic risk factors for PEX syndrome and PEX
    glaucoma
    Symptoms Signs:

    ●Not specific. - Increased IOP

    ●Sometimes patients may complain of : - damage to optic nerve

    Lessened visual acuity or changes in their - Poor dilation with peripupillary transillumination
    perceived visual field, and such changes defect
    may be secondary with cataracts or
    glaucoma - Hyperpigmentation of the trabecular meshwork

    - Fibrillar white flaky deposits on the anterior lens


    capsule (Hoarfrost Ring) & pupillary border
    Complications :

    •Glaucoma or optic nerve cupping

    •Progressive open angle glaucoma

    •Corneal decompensation

    •Cataract surgery such as,


    • Drop nucleus or lens fragment
    • Zonular dialysis
    • Anterior chamber fibrinoid syndrome after cataract extraction
    • Anterior capsular contraction syndrome: Anterior capsule fibrosis and phimosis
    Management

    •Routine regular eye exam annually

    •Glaucoma : eye drops

    •Antioxidants

    •Lower Homocysteine if the level is high

    •Laser surgery: drainage angle helps it drain more fluid from the eye.
    Differential diagnosis

    •Lens capsule deposits: true exfoliation syndrome

    •Trabecular meshwork hyperpigmentation/glaucoma:


    Pigment dispersion syndrome

    •Iris transillumination defects:


    Pigment dispersion syndrome, trauma, prior herpes infection

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