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 denizcebiolgun@gmail.com
eye and 20/400 in the left eye. Slit-lamp examination of dilated pupils showed dis-
location 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 unex-
plained 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 con-
nective tissues. Ocular involvement, including lens dislocation, is common. The
patient declined orbital surgery and was treated conservatively with corrective eye-
glasses. 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 re-
solved 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 e13

The New England Journal of Medicine
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