The Retina Reference

Marfan Syndrome with Subluxated Crystalline Lens

In Marfan Syndrome, the zonules that hold the crystalline lens in position tend to break, leading to decentration of the lens. Initially this can be managed with refractive correction (glass change or contact lenses), but eventually the crystalline lens needs to be surgically removed and replaced with a well-centered manmade intraocular lens. The operation is called vitrectomy, lensectomy and insertion of an intraocular lens implant. In this slit lamp photograph, the edge of the decentered crystalline lens is indicated by yellow arrows. This 29 year old woman had a similar finding in the fellow eye, and this was the problem that led to the diagnosis of Marfan Syndrome in her case. Genetic testing revealed that she had the fibrillin gene mutation of chromosome 15.