Please make a gift donation today The major ocular abnormality in Marfan syndrome is ectopia lentis (lens subluxation or dislocation. While relatively little is known about the exact mechanism of this ocular pathology in Marfan syndrome, a number of theories have been suggested. Wheatley et. al (1995) found that while fibrillin is localized to the superficial capsule and ciliary epithelial surface at the attachment of the zonules in normal eyes, Marfan patients lack such localization and exhibit abnormal ciliary processes with absent or severely disorganized zonules. This pathology was found to be positively correlated with lens subluxation. Clinically, ectopia lentis is bilateral in 60-87% of Marfan patients and is stable from childhood. Symptoms include fluctuating blurred vision, monocular diplopia, and pain. On exam, patients demonstrate refractive instability with myopia and astigmatism, iridodonesis, phacodinesis, and a recessed angle. The most common direction of dislocation on exam is superotemporal. In addition, there may be secondary complications from lens movement such as phacolytic uveitis from posterior subluxation of the lens to the vitreous.
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