Idiopathic retinal arterial occlusive vasculitis in the setting of multiple arterial occlusions

PURPOSE: We present a patient with vaso-occlusive retinal vasculitis to summarize this uncommon entity and review the clinical features and management challenges applicable to patients with retinal vasculitis. OBSERVATIONS: A 76-year-old male presented with sudden-onset severe central vision loss. On examination, vitreous hemorrhage, neovascularization of the optic nerve, peripheral segmental periphlebitis, vessel sclerosis, vascular sheathing, and retinal hemorrhages were observed, and a diagnosis of active vaso-occlusive retinal vasculitis was made. The patient then underwent a complete infectious, inflammatory, and neoplastic workup which returned negative. The patient was treated with locally with a sub-Tenon's injection of 40 mg triamcinolone on presentation and later with oral prednisone. At three-month follow-up, vision improved to 20/300 with regressing neovascularization and clearing of vitreous hemorrhage in the right eye (OD). CONCLUSIONS: Considering novel associations of occlusive retinal vasculitis, it is important to recognize that idiopathic occlusive retinal vasculitis, although uncommon, can occur and represents a prototypical disease form. It is imperative that these patients have a complete infectious, inflammatory, and neoplastic workup owing to the possible overlap of masquerade clinical signs and symptoms.