Pathophysiology of zonular diseases.
OBJECTIVE
The mechanisms implicated in the clinical manifestations of zonular diseases, especially ectopia lentis, are reviewed.


RESULTS
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.


CONCLUSIONS
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.
Dureau
Pascal
P
Fondation Ophtalmologique Adolphe de Rothschild, Pediatric Ophthalmology Department, Paris, France. pdureau@fo-rothschild.fr
D004479:Ectopia Lentis / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D005337:Fibrin / Q000378:metabolism*; D005901:Glaucoma / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications / Q000378:metabolism / Q000503:physiopathology; D012029:Refraction, Ocular / Q000502:physiology*; D012030:Refractive Errors / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology
D005337:Fibrin