- More commonly unilateral although in about one third of cases, it presents bilaterally.
- Affects elderly patients with coexisting cataract.
- Symptoms: usually asymptomatic
- 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.
- 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