Pseudoexfoliation syndrome and glaucoma
Flaky white material builds up in the front of the eye with age, clogging the drain and weakening the lens supports. It causes a severe glaucoma.
What it is
An age-related disorder in which a fibrillar, flaky white material is produced and deposited on the lens capsule, pupil margin, drainage angle and other tissues. It is the most common identifiable cause of secondary open-angle glaucoma, which tends to have higher and more variable pressures than primary glaucoma. It also weakens the zonules, making cataract surgery riskier.
See it in 3D
Who gets it
Increases with age, usually after 60; frequency varies widely between populations and is high in some (for example Nordic countries); often one eye first.
Causes
- Genetic predisposition (variants of the LOXL1 gene)
- Ageing
- Environmental factors (latitude, ultraviolet exposure)
Risk factors
- Age over 60
- Family history
- Some ethnic and geographic backgrounds
Pathophysiology
Abnormal extracellular matrix material accumulates; it is rubbed off the lens by the moving iris, releasing pigment and debris that clog the trabecular meshwork and raise pressure. The same material weakens the zonules and the iris dilates poorly.
Symptoms and signs
- None until glaucoma or cataract develops
- Gradual peripheral field loss in advanced glaucoma
Diagnosis
- Clinical
- Dilated slit lamp examination shows a target-like pattern of white material on the anterior lens capsule; poor dilation and transillumination defects at the pupil margin.
- Tests
- Tonometry, gonioscopy (heavy angle pigmentation), optic disc and field assessment.
- Imaging
- OCT of the nerve fibre layer to follow glaucoma.
Differential diagnosis
- Pigment dispersion syndrome
- True exfoliation (heat-related capsular delamination)
- Primary open-angle glaucoma
Goal of treatment
Control the eye pressure and plan cataract surgery safely.
Treatment
- Medical
- Pressure-lowering drops.
- Procedure
- Laser trabeculoplasty, often effective in this condition.
- Surgical
- Glaucoma surgery when pressure is not controlled; cataract surgery with measures to support weak zonules.
Complications
- Glaucoma with rapid visual field loss
- Lens subluxation or dislocation
- Complications of cataract surgery
- Central retinal vein occlusion
Prognosis
Glaucoma is more aggressive than primary open-angle glaucoma but controllable with timely treatment.
Test yourself
Think of the answer, then tap the question to see it.
Where is pseudoexfoliation material best seen?
On the anterior lens capsule after dilating the pupil.
Why is cataract surgery riskier in pseudoexfoliation?
Because the zonules are weak and the pupil dilates poorly.
Which gene is linked to pseudoexfoliation?
LOXL1.
In ophthalmology
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Pseudoexfoliation Syndrome and Glaucoma (StatPearls Publishing (NCBI Bookshelf), 2023).
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Representative model, for educational purposes only.