Angle closure

Question 1

Causes of ectopia lentis

Answer 1

Pseudoexfoliation syndrome
Trauma
Marfan syndrome
Homocystinuria
Microspherophakia
Weill-Marchesani syndrome
Ehlers-Danlos syndrome
Sulfite oxidase deficiency

Question 2

What is microspherophakia, what syndromes can it be associated with, and how do you treat subsequent angle closure?

Answer 2

1. Lens has spherical or globular shape –> ectopia lentis –> pupillary block
2. Weill-Marchesani, Marfan
3. Treat with cycloplegia –> tightens the zonules, flattens the lens, pulls it posteriorly to relieve pupillary block

Avoid miotics –> may worsen pupillary block and can loosen the zonules making the lens more globular