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Pseudoexfoliation Syndrome

Curious. Pseudoexfoliation syndrome seems to have 3 abbreviations (PXF/PES /PEX)? PES also stands for phantom eye syndrome!

Pseudoexfoliation Syndrome 

PXF tends to occur in older individuals, and is seen in people from Scandinavian countries more frequently than other places.

It is a systemic disease affecting the basement membrane, which results in secretion of amyloid material in the eye, blood vessels and organs. This condition may be associated with oxidative stress and genetic mutation.

Textbooks describe pseudoexfoliation syndrome as ‘dandruff-like’ material /flakes on the surface of the lens and anterior chamber. The iris pushes the flakes into a whitish-grey ring (“Hoarfrost ring”) on the anterior surface of the lens.

The flakes settle at the trabecular meshwork, impeding outflow and raising IOP. The risk of glaucoma in PXF is 1% per year (i.e. 5% in 5 years, 15% in 15 years). 

PXF also causes:

Weakened lens zonules: PXF material triggers enzymatic degradation of lens zonules as they settle there. Weakened zonules means there is a risk of lens subluxation. Further, extra care needs to be taken during cataract surgery - eg during capsulorhexis, breaking up the lens, positioning the PCIOL etc. 

Iris atrophy: occurs because of loss of blood vessels at the iris. This makes dilating the pupil difficult. 

*PXF material that accumulates at the drainage angle can present a physical obstacle to dilation as well. 

Differential

POAG, true exfoliation of the lens, angle closure glaucoma, pigment dispersion syndrome, amyloidosis

-true exfoliation may be due to aging, infrared radiation (heat), trauma, prolonged iridocyclitis

-exfoliative material on the lens is also a sign of a past angle-closure attack.

Management of patient

Monitor IOP, annual follow up to check for glaucoma.