Frosted branch angiitis complicated by retinal vein occlusion: clinical course and long-term visual outcome

Abstract

Purpose: Frosted branch angiitis (FBA) is a rare entity characterized by acute panuveitis in the form of a florid translucent retinal perivascular sheathing of both arterioles and venules, with variable uveitis, retinal edema and visual loss. Primary and secondary cases have been described in association with infectious, inflammatory and malignant etiologies. We aim to describe the clinical course and long-term visual outcome of three patients who developed retinal vein occlusion (RVO) and macular edema as a complication of FBA.

Methods: Descriptive case series.

Results: Three young healthy patients aged 22, 37 and 45 years presented with sudden visual disturbance secondary to unilateral primary FBA, which improved significantly following high-dose steroid therapy. Several weeks later, RVO developed, with marked cystoid macular edema. Visual improvement was achieved and maintained with anti-VEGF therapy over a follow-up period ranging from 14 to 44 months.

Conclusion: FBA may be considered a risk factor for the development of secondary RVO because of the severe retinal vasculitis that eventually leads to activation of the coagulation system and retinal thrombosis. It remains to be determined whether antiplatelet therapy needs to be administered prophylactically in such a scenario in order to reduce the risk or prevent the development of RVO.

Keywords: Bevacizumab; Branch retinal vein occlusion; Central retinal vein occlusion; Frosted branch angiitis; Macular edema; Ranibizumab.