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JAMA Ophthalmology Clinical Challenge
September 26, 2019

An 18-Month-Old With a White Optic Nerve Head Mass

Author Affiliations
  • 1Department of Ophthalmology and Visual Sciences, Vanderbilt University Medical Center, Nashville, Tennessee
  • 2Vanderbilt University School of Medicine, Vanderbilt University, Nashville, Tennessee
  • 3Vanderbilt-Ingram Cancer Center, Vanderbilt University Medical Center, Nashville, Tennessee
JAMA Ophthalmol. Published online September 26, 2019. doi:10.1001/jamaophthalmol.2019.3798

An 18-month-old girl with an unremarkable medical history was referred to ocular oncology from pediatric ophthalmology for an intraocular mass near the optic nerve. Her parents noticed the left eye turning out for the past year. On examination, she fixed and followed with her right eye but not with the left, with 5.0-diopter exophoria at near in the left eye. Penlight examination results showed left leukocoria. Intraocular pressure and motility were normal bilaterally. On subsequent examination under anesthesia, the left optic nerve was obscured by a whitish mass. While the optic nerve itself was obscured, a pigmented crescent could be seen surrounding it (Figure, A). Ultrasonography results demonstrated an elevated retinal mass overlying an excavated optic nerve head. A small focus of hyperechogenicity was noted at the optic nerve head, consistent with calcium (Figure, B). Fluorescein angiography results demonstrated staining of the central whitish mass, which was obscuring the underlying retinal vessels. On fluorescein angiography results, no vessels were seen within the central whitish mass itself.

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