Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives

Abstract

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome typically affects young, healthy individuals. Despite the dramatic fundus appearance seen in this syndrome, these patients are usually asymptomatic. The syndrome includes peculiar vascular abnormalities in the form of multiple aneurysmal dilatations seen along retinal arterioles and optic nerve-head arterioles, which are best appreciated on fluorescein angiography. Neuroretinitis and retinal vasculitis are seen in all patients, and manifested by staining of the optic nerve head and diffuse leakage from vessels, mainly arterioles, on fluorescein angiography. The devastating vision-threatening outcomes of this syndrome include exudative retinopathy and extensive peripheral retinal nonperfusion areas, which can eventually lead to neovascularization. This review summarizes current knowledge on the variable clinical aspects of this disease, highlighting diagnostic and treatment strategies.

Retinal vasculitis – Clinical manifestations and outcome at tertiary eye care centre in south India

“…It is usually bilateral, but a few cases of unilateral presentation have also been reported and a few cases where it was unilateral at presentation can also involve both eyes later. 7 In cases of IRVAN, arterioles are commonly involved, associated with multiple aneurysmal dilatations of the retinal and optic nerve-head arterioles. These peculiar vascular anomalies measure 75-300 µm in diameter, and are present at or near major branching sites on retinal arterioles.…”

“…These peculiar vascular anomalies measure 75-300 µm in diameter, and are present at or near major branching sites on retinal arterioles. 7 Multiple sclerosis (MS) and Susac syndrome cases were referred to neuro physician and managed by the neurologist. These cases are rare but requires proper workup to prevent blindness in young adults.…”

The use of pan-retinal photocoagulation to treat recurrent vitreous haemorrhage with neovascularisation in the context of Epstein syndrome: an MYH9-related disorder

“…There are a plethora of rare diseases that result in aberrant neovascularisation following retinal ischaemia or vaso-occulsive disease such as that seen in this case. Idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome demonstrates a further rare disease with multiple microvascular pathologies coexist that lead to neovascularisation and further accelerated deterioration in vision 18. Similarly, vaso-occlusive disease secondary to systemic lupus erythematosus (SLE) demonstrates neovascularisation and vitreous haemorrhages that can manifest as the initial presentation of SLE 19 20.…”

A Typical Features in Irvan Syndrome Demonstrated on Multimodal Imaging

“…The clinical presentation and course vary remarkably.The disease may have a self-limiting course in some eyes,while many eyes progress to retinal oedema, ischaemia, and neovascularization advancing to neovascular glaucoma. 1,2 The diagnosis of IRVAN syndrome is based on the presence of three major criteriathat are not caused by any other disease: retinal vasculitis, aneurysms, and neuroretinitis. Three minor and optional criteria are macular exudation, capillary nonperfusion (CNP) and retinal neovascularization.1,2…”

“…Nonetheless, the acquired nature, mild intraocular inflammation, resolution of aneurysms over time or in response to anti-inflammatory therapy and immune modulators all support an inflammatory origin. 1,2,4 Neuroretinis in IRVAN syndrome is characterized by hyperaemic and oedematous optic discs and is depicted by FFA as a hyperfluorescent disc in the later phase of angiogram 1. Other ocular features include macular oedema, epiretinalmem branes (ERMs), optic disc aneurysms, retinal capillary nonperfusion (CNP), retinal vascular occlusions, retinal neovascularization, vitreous haemorrhage and iris rubeosis.1,2 Low-grade inflammatory activity is usually seen in the anterior chamber and/or vitreous at some point during the disease course.…”