Abstract

A 22-year-old woman with tuberous sclerosis (TS) and an incomplete resection of a left-sided astrocytoma presented with a 4-year history of visual deterioration. Neurologic examination revealed a left relative afferent pupillary defect (RAPD) (video) with a pale left optic disc. Visual acuity was 20/100 bilaterally and a left-sided superior visual field loss was seen. Optical coherence tomography showed a left retinal hamartoma (figure 1). Contrast-enhanced brain MRI showed bilateral cortical and subcortical tubers (figure 2). Marcus-Gunn pupil or RAPD is a nonspecific feature denoting optic neuropathy.1 TS ophthalmopathy includes retinal and iris hamartomas, choroid colobomas, and hypopigmented lesions on iris and ciliary body.2

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