The
n e w e ng l a n d j o u r na l
of
m e dic i n e
images in clinical medicine Lindsey R. Baden, M.D., Editor
Ectopia Lentis
A
31-year-old woman with Marfan’s syndrome presented with Deniz Çebi Olgun, M.D. amblyopia and a history of gradual bilateral vision loss over the previous 3 to Fatih Kantarci, M.D. 4 months. She also had a recent history of mild sensorineural hearing loss Istanbul University within the previous 2 weeks. Ophthalmologic examination revealed blue sclera and Istanbul, Turkey a substantial loss of vision, with best-corrected visual acuity of 20/80 in the right [email protected] eye and 20/400 in the left eye. Slit-lamp examination of dilated pupils showed dislocation of the lens into the vitreous in both eyes. There was retinal degeneration but no evidence of retinal tear or detachment. The intraocular pressure was normal. Magnetic resonance imaging of the cranium was performed owing to the unexplained hearing loss, and image sections obtained through the orbit with the use of T2-weighted scans in the axial plane showed posterior dislocation of the lens in both eyes. Marfan’s syndrome is an autosomal dominant disorder that affects connective tissues. Ocular involvement, including lens dislocation, is common. The patient declined orbital surgery and was treated conservatively with corrective eyeglasses. The hearing loss was assumed to have been caused by a viral infection. The patient was treated with glucocorticoids, and the symptoms of hearing loss resolved completely. At a 2-year follow-up visit, the patient’s visual loss was stable, with no worsening in the intervening period.
DOI: 10.1056/NEJMicm1308550 Copyright © 2015 Massachusetts Medical Society.
n engl j med 372;9
nejm.org
february 26, 2015
The New England Journal of Medicine Downloaded from nejm.org on November 3, 2015. For personal use only. No other uses without permission. Copyright © 2015 Massachusetts Medical Society. All rights reserved.
e13
n e w e ng l a n d j o u r na l
of
m e dic i n e
images in clinical medicine Lindsey R. Baden, M.D., Editor
Ectopia Lentis
A
31-year-old woman with Marfan’s syndrome presented with Deniz Çebi Olgun, M.D. amblyopia and a history of gradual bilateral vision loss over the previous 3 to Fatih Kantarci, M.D. 4 months. She also had a recent history of mild sensorineural hearing loss Istanbul University within the previous 2 weeks. Ophthalmologic examination revealed blue sclera and Istanbul, Turkey a substantial loss of vision, with best-corrected visual acuity of 20/80 in the right [email protected] eye and 20/400 in the left eye. Slit-lamp examination of dilated pupils showed dislocation of the lens into the vitreous in both eyes. There was retinal degeneration but no evidence of retinal tear or detachment. The intraocular pressure was normal. Magnetic resonance imaging of the cranium was performed owing to the unexplained hearing loss, and image sections obtained through the orbit with the use of T2-weighted scans in the axial plane showed posterior dislocation of the lens in both eyes. Marfan’s syndrome is an autosomal dominant disorder that affects connective tissues. Ocular involvement, including lens dislocation, is common. The patient declined orbital surgery and was treated conservatively with corrective eyeglasses. The hearing loss was assumed to have been caused by a viral infection. The patient was treated with glucocorticoids, and the symptoms of hearing loss resolved completely. At a 2-year follow-up visit, the patient’s visual loss was stable, with no worsening in the intervening period.
DOI: 10.1056/NEJMicm1308550 Copyright © 2015 Massachusetts Medical Society.
n engl j med 372;9
nejm.org
february 26, 2015
The New England Journal of Medicine Downloaded from nejm.org on November 3, 2015. For personal use only. No other uses without permission. Copyright © 2015 Massachusetts Medical Society. All rights reserved.
e13
