Pseudoexfoliation Syndrome

Question 1

What is PXF syndrome?

Answer 1

Systemic disease in which grayish-white material is deposited on anterior segment structures and systemically. (Skin, heart, lungs)

Question 2

Cause of PXF syndrome

Answer 2

Defect in LOXL1 gene. Codes for an enzyme essential for the formation of elastin fibers.

Question 3

Signs (3)

Answer 3

1. Grayish-white flaky deposition all over:

Anterior lens capsule- central zone of deposition (often with rolled up edges, middle clear zone, peripheral cloudy zone)

Pupillary margin, endothelium, TM, Anterior hyaloid, ciliary body, zonules.

2. Iris sphincter atrophy with TIDs at the pupillary margin.
3. Pigmentation of the TM, Schwalbe’s line (Sampaolesi) and K endothelium.

Question 4

Complications (3)

Answer 4

Secondary open angle glaucoma. Fibrillar material and pigment may obstruct the TM.

Secondary narrow angle or angle closure glaucoma. Unstable zonules can lead to anterior lens dislocation and narrow angles.

Lens dislocation if zonules are unstable.

Question 5

How to monitor?

Answer 5

See pt every 6-12 months for PXF glaucoma.

• Do SL exam with retroillumination (to help see iris TIDs and lens opacities, also helps grade severity of fibrillar deposition/pigment on anterior segment structures)
• IOP
• Gonio to view fibrillar/pigment deposition.
• ON photos
• ON OCT
• VF

Question 6

PXF is the most common cause of ____ glaucoma

Answer 6

Secondary open angle glaucoma.

60% develop OHTN or glaucoma.