Multimodal imaging questions etiology of idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome (IRVAN syndrome)

Abstract

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a rare entity of unknown etiology for which many hypotheses have been proposed with inflammation being the most commonly accepted hypothesis. We report cases of a 9 year old girl and a 22 year old male patient with diagnosis of IRVAN syndrome. The conclusions drawn from the clinical examination and multimodal imaging including optical coherence tomography angiography of the patients are discussed. Our conclusions and interpretation point towards IRVAN being a developmental vascular anomaly rather than a consequence of inflammation. It is thus proposed to be renamed as Idiopathic retinal arteriolar aneurysm syndrome (IRAA).