DR MOHAMMED IRFAN SHEIKH over 4 years ago

Marfan’s Syndrome with Ectopia Lentis

A 41-year-old man presented with progressive loss of vision in both eyes that was worse in the right eye than in the left. There was no history of trauma. His uncorrected visual acuity was 6/200 in the right eye and 20/100 in the left eye. Slit-lamp examination revealed ectopia lentis in both eyes, including near-complete inferior dislocation of the lens in the right eye (shown above) in addition to visible stretched zonules (the suspensory strands of the lens) and inferotemporal subluxation of the lens in the left eye. The patient’s mother has Marfan’s syndrome, and the patient has the elongated digits and pectus excavatum that are characteristic of the disease. He underwent pars plana vitrectomy and implantation of a scleral-sutured intraocular lens in the right eye. At final follow-up, the best corrected visual acuity in the right eye was 20/20, with correction of myopia. Marfan’s syndrome is associated with ectopia lentis in both eyes, a characteristic that may be subclinical. Indications for surgery include correction of an otherwise unmanageable difference in refractive power between the two eyes (anisometropia), excessive induced astigmatism, and complications of lens subluxation, including glaucoma, uveitis, and retinal detachment.