Multimodal imaging and OCTA in IRVAN syndrome

Author: Hugo Tapia Quijada (Spain)

Co-authors: Nisamar Betancor Caro, Paula Fernandez Alvarez

Purpose

The syndrome of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) is a rare clinical entity of unknown etiology. The diagnosis of IRVAN is one of exclusion and is based on a constellation of clinical characteristics. We present a case of IRVAN syndrome and its study with multimodal imaging that includes OCTA.

Setting/Venue

Section Retina. Hospital Universitario de Canarias. Tenerife-Spain

Methods

Case Report

Results

A 42-year-old woman, with no significant history, presented with pain and poor vision in her left eye (LE) secondary to neovascular glaucoma. Visual acuity was 1.0 in RE and hand movements in LE. The fundoscopy showed macroaneurysms in RE located in arteriolar arches and also in optic nerve, the periphery showed hyalinized ischemic vessels, and in LE more severe ischemic signs with macular involvement and papillary neovascularization were observed. Fluorescein angiography (FA) revealed bilateral occlusive vasculitis with greater involvement in LE. OCTA in RE showed flow in the macroaneurysms and in LE ischemia in the superficial plexus in the macula and optic nerve. Systemic studies were carried out for vasculitis, all of them being negative. Our patient was diagnosed with IRVAN syndrome. Oral steroids associated with panretinal laser photocoagulation in the LE caused the disappearance of papillary neovascularization without recurrence at 4 months.

Conlusions

OCTA demarcates non-perfused areas well but cannot replace FA but should be considered alternatively for non-invasive follow-up of IRVAN syndrome.

Financial Disclosure

NO FINANCIAL DISCLOSURE