A 23-year-old monocular woman with neurofibromatosis type 1 and Moyamoya syndrome developed gradual, asymptomatic intraocular pressure (IOP) increase in her seeing eye to 53 mmHg, which was refractory to maximum medical therapy. Vision was 20/20, anterior segment examination was only remarkable for Lisch nodules, and angle was open 360 degrees with no blood in Schlemm’s canal. Disc and retinal examinations were normal (Fig A), and retinal nerve fiber layer OCT and Humphrey Visual Field 24-2 were normal. Other secondary causes of IOP elevation were ruled out. An Ahmed FP7 was implanted, and the anterior chamber was 50% filled with Healon Pro to prevent sudden hypotony-associated complications. On postoperative day 1, visual acuity was 20/20, IOP was 10 mmHg, and fundus examination demonstrated disc edema and multifocal intraretinal hemorrhages in all layers extending from the arcades to the periphery consistent with decompression retinopathy (Fig B). By postoperative week 4, the disc edema resolved, and the hemorrhages were markedly improved (Fig C). By postoperative week 10, the hemorrhages were completely resolved (Fig D), vision remained 20/20, and IOP was controlled (Magnified version of Fig A-D is available online at www.aaojournal.org).Article InfoIdentification

DOI: https://doi.org/10.1016/j.ophtha.2022.01.006

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