Pathophysiology of zonular diseases.

PURPOSE OF REVIEW: The mechanisms implicated in the clinical manifestations of zonular diseases, especially ectopia lentis, are reviewed.

RECENT FINDINGS: The molecular mechanisms involve fibrillin in a large spectrum of heritable diseases characterized by zonular stretching. The usual complications are refractive errors, especially myopia, glaucoma (either primary open angle, secondary angle closure and pupil block by anterior displacement of the lens) and retinal detachment.

SUMMARY: The genetics and molecular understanding provide information for genetic counseling. Treatment of myopia and glaucoma depend on the underlying mechanism, and lens surgery techniques are continuously improved.