Pseudoexfoliation syndrome

Common cause of secondary glaucoma: 25% of PXF patients develop glaucoma

More severe and resistant course than POAG

Systemic condition

• Whitish dandruff-like material is deposited on

  • Eyes
  • Skin
  • Heart
  • Lungs
  • Kidneys
  • Meninges

• Abnormal elastic microfibrils, basement membrane material, glycosaminoglycans

• Possibly associated with raised homocysteine

• 20% of population in Scandinavia

• Linked to LOXL1 gene on chromosome 15

Risk factors

• Age >40
• Female
• Ethnicity: north european/northern latitudes, mediterranean
• Higher altitudes

Clinical features

• Dandruff-material on

  • Pupillary border
  • Lens capsule
  • Stains with Masson Trichrome

• Peripupillary transillumination defects (cp. Pigment dispersion)

• Poor mydriasis

• Iridodonesis

• Phacodonesis: with risk of zonulodialysis during cataract surgery

• Pigment in the AC

• Pigment and pseudoexfoliative material on corneal endothelium

• Irregular pigment and PXF material deposition in the trabeculum and anterior to Schwalbe’s line (Sampaolesi’s line) on gonioscopy (also seen in PDS, trauma, poorly controlled diabetes and iris melanoma)

  • Open but narrow angle

• Glaucoma

  • Open angle: PXF deposition in TM
  • Tends to present unilaterally with progression to both eyes in 40%
  • Narrow angle (rare): weak zonules causing anterior lens-iris movement
  • PXF glaucoma tends to be more volatile with faster progression compared to POAG

Management

• As for POAG
• More likely to need laser and surgery