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JAMA Ophthalmology Clinical Challenge
August 2018

Severe Progressive Vision Loss in a Teenager

Author Affiliations
  • 1Department of Ophthalmology and Visual Science, Yale School of Medicine, New Haven, Connecticut
  • 2Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, Connecticut
  • 3Eye Surgery Associates, LLC, New Haven, Connecticut
JAMA Ophthalmol. 2018;136(8):950-951. doi:10.1001/jamaophthalmol.2017.6792

A 14-year-old girl was seen with progressive vision loss in both eyes. Two years before our examination, her visual acuity had been 20/200 OD and 20/60 OS. At our initial evaluation, her visual acuity was 20/300 OD and 20/250 OS. Optic nerve atrophy was noted, with no optociliary collaterals. Imaging of the retinal nerve fiber layer showed thinning in both eyes. Automated perimetry demonstrated general constriction of visual fields and bilateral central scotomas. Ocular motility was full, without pain, and orthotropic. Two years prior, initial magnetic resonance imaging (MRI) of the brain and orbits with and without contrast was read as normal. Retrospectively, those images were reviewed and noted to be limited due to artifact from dental braces and a lack of fat suppression. Two years prior, lumbar puncture revealed normal opening pressure.

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