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Bull's eye!

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

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