Background: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome is a very rare primary retinal vasculitis. It seems to evolve in stages, where there is initially a florid vasculitis associated with aneurysm formation. Neuroretinitis and macula edema are common features. Subsequently, retinal ischemia ensues, leading to neovascularization. If untreated, further sight-threatening complications occur, including traction retinal detachment and secondary glaucoma.
Methods and results: Here, we describe a patient with early idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome, who was treated with a combination of systemic immunosuppression and localized photocoagulation. There was substantial regression of the aneurysms and improvement of the macular edema. Treatment of the disorder should be based on the clinical stage and complications of the disorder.
Conclusions: As shown here, a multidisciplinary approach can be very helpful in managing patients with this severe sight-threatening disorder.