Grand Rounds

A young patient presented with chronic decreased vision, with best-corrected visual acuity (BCVA) of 20/30 in the right eye (OD) and 20/80 in the left eye (OS). She had no history of ocular trauma or disease. Slit-lamp examination revealed faint cortical lens opacities without signs of vitritis. Fundoscopic examination showed mottling of the macula bilaterally. Systemic evaluation for inflammatory and infectious causes was negative. Fluorescein angiography (FA) showed no signs of vasculitis or vascular abnormalities. Optical coherence tomography (OCT) revealed bilateral ERM with distinctive flame-shaped features. Given the unusual presentation, genetic testing was performed, yielding a positive result for Neurofibromatosis type 2 (NF2). Further investigation with brain MRI confirmed bilateral vestibular schwannomas, consistent with NF2. Due to progressive vision loss in the left eye (BCVA decline to 20/100), the patient underwent pars plana vitrectomy (PPV) with membrane peeling. One year postoperatively, her vision improved to 20/70, with stable retinal architecture and no recurrence of ERM. This case highlights the importance of considering NF2 in young patients with bilateral ERM and distinctive OCT findings. Early diagnosis and intervention can help manage ocular and systemic manifestations, improving long-term outcomes.

Presentation Date: 04/03/2025
Issue Date: 04/10/2026