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July 3, 2019

Association of Nonuveitic Retinal Vasculitis and Tractional Retinoschisis With Cystoid Macular Edema With Fabry Disease

Author Affiliations
  • 1Duke Eye Center, Duke University, Department of Ophthalmology, Durham, North Carolina
  • 2Byers Eye Institute, Department of Ophthalmology, Stanford University, Palo Alto, California
  • 3Retina Consultants, PC, Hartford, Connecticut
JAMA Ophthalmol. 2019;137(9):1086-1088. doi:10.1001/jamaophthalmol.2019.2170

A 62-year-old white woman presented with several years of poor vision in the right eye. Her ocular history included recent cataract surgery in both eyes. Her medical history was significant for hypertrophic cardiomyopathy (HCM) and renal artery stenosis. She also reported intermittent pain in her hands and feet and chronic diarrhea.

Her best-corrected visual acuity was 20/150 OD and 20/20 OS. An anterior segment examination showed vortex keratopathy (cornea verticillata) in both eyes. Fundus examination results of the right eye revealed perivascular sheathing (Figure 1A) and segmental perivenular leakage on fluorescein angiography (Figure 1B). Optical coherence tomography results demonstrated the presence of an epiretinal membrane, tractional retinoschisis, and cystoid macular edema (CME) (Figure 2A). An examination of the left eye revealed mild retinal vascular tortuosity.