Pseudoexfoliation syndrome demonstrating the three identifiable zones on the anterior lens capsule. Note in the second picture (taken from a different patient) the white flaky material deposits on the pupillary margin.

More commonly unilateral although in about one third of cases, it presents bilaterally. Affects elderly patients with coexisting cataract. Clinical features: Symptoms: usually asymptomatic Signs: Gray-white fibrillar material deposited on the surface of structures in the anterior chamber of the eye The deposits produce three identifiable zones on the anterior lens capsule and are best seen when the pupil is dilated: The translucent material deposit in the pupillary area which is quite faint and easily missed, outlined by dandruff-like deposits More dense deposits of granular material at the peripheral zone Clear intermediate zone, which is attributed to the posterior iris rubbing against the anterior lens capsule The PXE material deposits on the zonules and ciliary processes may be associated with zonular weakness and lead to lens subluxation or zonular dialysis during cataract surgery Increased pigment deposition at Schwalbe's line (i.e. Sampaolesi's line) Deposits of flaky PXE material in the angle may compromise aqueous outflow and result in secondary glaucoma. Open angle glaucoma is more common in patients with PXE (than in normal population) and may be more resistant to medical therapy. Management: Regular follow-up of patients (including those with normal intraocular pressures and optic discs) Topical and/or systemic anti-glaucoma medications are initially used to treat patients with increased intraocular pressure Surgical treatment (including laser trabeculoplasty and filtration surgery) may be indicated for glaucoma resistant to medical therapy

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