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Ectopia Lentis in Marfan's Syndrome

Ectopia Lentis in Marfan's Syndrome

A 27-year-old woman with a diagnosis of Marfan's syndrome presented with worsening vision and glare in each eye for 1 month. On examination, visual acuity was 20/30 in each eye. Superotemporal partial subluxation of the lens was noted in each eye. On close inspection of the right eye, a decrease in the overall number of zonules was observed as widened spaces between zonules (arrows). Intraocular pressure was normal. Ectopia lentis is an ocular finding in Marfan's syndrome and typically develops between birth and 30 years of age. The lens subluxation is typically bilateral, symmetric, and in the superotemporal direction, although it may occur in other directions. Zonular fibers of the lens are few and are often attenuated and broken. Surgical intervention may be required if the refractive error cannot be managed conservatively or if uncontrolled glaucoma develops. Our patient was treated conservatively, and visual acuity improved with new prescription eyeglasses. The patient's condition has remained stable over a 2-year follow-up period.

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