Pseudoexfoliation (PXF) Syndrome
Systemic condition with manifestations in the eye
Grey-white fibrillary extracellular material composed of protein core surrounded by glycosamingoglycans → produced by abnormal ageing epithelial cells in trabeculum, equatorial lens capsule, iris, ciliary body
Inherited microfibrillopathy, Genetic link to LOX1 gene
Deposited on
Anterior capsule of lens
Bull’s eye appearance with three distinct zones
Translucent, central disc with occasional curled edges
Clear zone
Peripheral granular zone which may have radial striations
Corneal endothelium - pigment deposition, lower cell density
Zonules - proteolytic enzymes cause zonular disintegration → lens subluxation and phacodonesis
Ciliary body and processes
Iris - White flecks on pupillary margin of iris with loss of pigment at the pupillary ruff, mid-peripheral transillumination defects
Trabecular meshwork - patchy hyperpigmentation
Sampolaesi line → scalloped band of pigment running anterior to Schwalbe’s line
Anterior vitreous face
Conjunctiva Extrabulbar sites - EOM, orbital septa, posterior ciliary arteries, vortex veins, central retinal vessels
Cataract surgery in PXF patients poses many challenges (more on this later)
Most common cause of Secondary open angle glaucoma → risk → 5% at 5 years, 15% at 10 years
Image from Rajan Eye Care Hospital