Frosted Branch Angiitis as Ocular Manifestation of Behçet's Disease: Unusual Case Report and Literature Review
A 39-year-old male presented with decreased vision in his left eye for two days. Visual acuity was 20 / 20 in the right eye and hand movement in the left eye. On slit lamp examination of the left eye, severe inflammation was seen in the anterior chamber (cells +4) and the vitreous (cells +4). Fundus examination of the left eye demonstrated 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 (). The right eye was normal. B-scan ultrasonography and spectral-domain optical coherence tomography of the left eye showed a severely edematous and detached retina in posterior pole (). Fluorescein angiography demonstrated a prolonged arteriovenous transit time, blockage of fluorescein corresponding to extensive retinal hemorrhage, widespread area of capillary non-perfusion, and extensive staining of sheathing veessels (). A diagnosis of occlusive retinal vasculitis resembling acute FBA was made and systemic evaluation for retinal vasculitis was performed. The patient had a history of recurrent genital and oral ulcers over two years, but no other systemic manifestations of Behçet's disease on presentation. The results of laboratory examinations, including serology for herpes virus, varicella zoster virus, and human immunodeficiency virus were normal or negative. Other examinations, including brain magnetic resonance imaging and polymerase chain reaction assay for varicella zoster virus and cytomegalovirus in the aqueous humor, were negative or normal. Human leukocyte antigen typing resulted in a positive HLA-B51.\nThe patient was started on intravenous methylprednisolone. Vitreous opacity progressed and angle hypopyon appeared until 24-hour after treatment initiation (). However, during two weeks after treatment, the vascular sheathing and vitreous opacity gradually disappeared. Systemic steroids were slowly tapered during the next two months and cyclosporine 150 mg/day was added. Two months later, follow-up angiographic findings revealed a widespread area of non-perfusion in three retinal quadrants with neovascularization of the disc (). Although panretinal photocoagulation for non-perfusion areas was performed, neovascularization progressed by four months (). However, iris rubeosis and neovascular glaucoma had not developed by 10 months. Six months after his presentation, he developed two ulcers on oral mucosa and pseudofolliculitis on his face, along the hairline. A diagnosis of Behçet's disease was made using the international criteria following a rheumatology consultation. Eight months later, follow-up optical coherence tomography imaging revealed atrophy and disruption of the photoreceptor layer in the macular area (). The final visual acuity was hand movement in the left eye. He is currently on maintenance cyclosporine therapy.