Active systemic steroid therapy employed in a case of bilateral frosted branch angiitis with acute chorioretinal circulatory insufficiency

BACKGROUND: Steroid treatment is believed to be effective for frosted branch angiitis, but frosted branch angiitis with retinal circulatory insufficiency does not have a good prognosis by steroid treatment alone. Here, we present a case of a patient that had a good outcome when treated with long-term active systemic betamethasone and vasodilation therapy for bilateral frosted branch angiitis with acute chorioretinal circulatory insufficiency. CASE: A 69-year-old male presented with sudden visual loss in his left eye. The visual acuity was 1.0 in the right eye and 0.1 in the left eye. In his left eye, only mild inflammation occurred in the anterior chamber, but extensive inflammation such as sheathing of retinal vessels, retinal hemorrhage, and edema of the optic disc was present. Fluorescein angiography showed a delay of the arm-retinal artery circulation time and severe dye leakage from the retinal vessels. The following day, the visual acuity worsened to 0.1 in the right eye and hand motion in the left eye. Moreover, extensive inflammation was now present in both the anterior and posterior segments, and the sheathing of the retinal vessels developed to frosted branch-like angiitis. Doppler examination showed flow in the bilateral ophthalmic artery but did not show flow in the central retinal artery or posterior ciliary artery. These findings were compatible with the diagnosis of frosted branch angiitis with chorioretinal circulatory insufficiency. We initiated active systemic steroid therapy for 7 months and vasodilation therapy for 3 months. Two years later, the visual acuity improved to 0.5 in both eyes. CONCLUSION: Long-term active systemic steroid therapy for frosted branch angiitis with severe circulatory insufficiency in the retina and choroid may improve visual function.