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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Peters KS, Daniels AB. An 18-Month-Old With a White Optic Nerve Head Mass. JAMA Ophthalmol. Published online September 26, 2019. doi:10.1001/jamaophthalmol.2019.3798
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