Pseudoexfoliation

 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