Frosted branch angiitis as ocular manifestation of Behçet's disease: unusual case report and literature review

Abstract

We report an unusual case of unilateral frosted branch angiitis associated with Behçet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behçet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behçet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.

Keywords: Behçet's disease; Frosted branch angiitis; Occlusive retinal vasculitis.

Fig. 1

(A) Initial fundus photograph of the left eye showed thick, white, confluent sheathing surrounding the retinal veins and arteries from the posterior pole to the periphery with extensive retinal hemorrhages, optic disc swelling, and macular edema. (B) Initial B-scan showed diffuse, thickened chorioretinal layer with retinal detachment of the posterior pole. (C) The next day after treatment, hypopyon in the anterior chamber appeared. (D) During the acute phase of inflammation, spectral-domain optical coherence tomography (OCT) showed a severely edematous and detached retina in the macular area. (E) Eight months after treatment, OCT image revealed an atrophied retina and disruption of the photoreceptor segment in the macular area. (F) Four months later, fundus photograph of the left eye showed that perivascular sheathing and macular edema disappeared, but neovascularization of the optic disc developed. (G) Initial angiography of the left eye demonstrated a prolonged arteriovenous transit time of 25 seconds, background blockage due to extensive retinal hemorrhage, widespread areas of capillary non-perfusion, and extensive staining of sheathing vessels. (H) Two months after treatment, angiography demonstrated a widespread non-perfusion area involving three quadrants of the retina, fluorescein blockage by retinal hemorrhage and dye leakage from neovascularization of the optic disc.