Serial optical coherence tomography angiography reveals progressive retinal reperfusion and structural recovery following immunosuppressive therapy in pediatric bilateral occlusive retinal vasculitis

Abstract

Abstract We report a case of bilateral occlusive retinal vasculitis in a previously healthy 6-year-old girl who developed severe visual loss 7 days after streptococcal infection. At presentation, best-corrected visual acuity was 0.125 in the right eye and 0.08 in the left eye. Fundus examination demonstrated extensive bilateral retinal vascular occlusion with retinal edema. Fluorescein angiography revealed widespread posterior pole nonperfusion, while optical coherence tomography angiography (OCTA) demonstrated extensive retinal capillary dropout corresponding to the ischemic areas. Visual field testing showed bilateral central absolute scotomas.Systemic investigations, including immunological testing, and polymerase chain reaction analyses of intraocular fluid and cerebrospinal fluid were unremarkable, excluding infectious etiologies. The patient was treated with intravenous methylprednisolone followed by oral prednisolone, together with colchicine and a tumor necrosis factor-α inhibitor for presumed noninfectious retinal vasculitis. Following treatment, visual function gradually improved. At 8 months, best-corrected visual acuity improved to 1.0 in the right eye and 0.5 in the left eye, accompanied by restoration of the ellipsoid zone on optical coherence tomography and improvement of the central scotomas. Serial OCTA demonstrated progressive reperfusion of the initially extensive retinal nonperfusion areas, indicating vascular remodeling after immunosuppressive therapy. Conclusion: This case demonstrates that retinal nonperfusion in severe pediatric occlusive retinal vasculitis may be at least partially reversible. Serial OCTA revealed progressive reperfusion of previously nonperfused retinal capillaries, which paralleled recovery of visual acuity and visual field. These findings highlight the value of OCTA for longitudinal assessment and suggest that prompt, appropriate immunosuppressive therapy can restore retinal perfusion and visual function even in severe pediatric occlusive vasculitis.