Woman presents with white retinal lesions

A 41-year-old Japanese-Caucasian woman was referred to the New England Eye Center for evaluation of retinal lesions in both eyes. Three weeks prior, she developed blurred vision, vertigo, headache, swollen neck glands, sore throat and malaise. She presented to an outside emergency department where she had a normal MRI of the brain and orbits and a positive throat culture for Streptococcus. She was seen by a consulting ophthalmologist who noted 20/200 vision in each eye with “white round patches surrounding the macula with areas of dot hemorrhages” in both eyes. She was treated with intravenous hydration and oral amoxicillin for presumed streptococcal pharyngitis and discharged with ophthalmology follow-up. She was an otherwise healthy woman without a significant medical, ocular or family history of disease.On presentation to an outside retina specialist, the woman reported constant blurred vision at near and far, fatigue, subjective fevers and night sweats, sore throat, mouth ulcers, swollen cervical lymph nodes, vertigo, diarrhea and bifrontal headache. Best corrected visual acuity was 20/160 in the right eye and 20/80 in the left eye, without improvement on pinhole. IOP, extraocular motility and confrontation visual fields were normal, and her anterior segment examination was unremarkable. Posterior segment examination was notable for perifoveal white retinal infiltrates resembling cotton wool spots with small overlying dot hemorrhages in the macula of both eyes (Figures 1a to 1d). Fluorescein angiography revealed irregular-appearing foveal avascular zones with small vessel occlusions and discontinuity in the periphery of both eyes (Figures 1e to 1j). She was treated with 50 mg of oral prednisone daily for presumed bilateral, multifocal, obliterative small vessel disease with possible macular ischemia bilaterally. One week later, her exam and visual acuities were unchanged, and she was referred to the New England Eye Center for further evaluation.