IRVAN Syndrome – June 2017

June 2017

Case Presentation

A 65 year-old female was referred to Illinois Retina Associates for evaluation of decreased vision and macular changes in both eyes. She had a past ocular history of laser treatment in both eyes but had not seen a retinal specialist in a few years.

She was known for coronary artery disease, heart failure and atrial fibrillation and taking the following medication: hydralazine, coumadin, carvedilol, furosemide, pravastatin, aspirin. She was not diabetic.


Her uncorrected visual acuity was 20/400 in the right eye, and 20/60 in the left eye, not improved with pinhole. Anterior segment examination was unremarkable except for mild nuclear sclerosis in both eyes. On posterior segment examination of both eyes, there were scattered laser scars in both eyes, multiple sclerotic vessels, occasional retinal hemorrhages, and scattered fibrotic vascular dilations consistent with macroaneurysms (Figures 1 & 2).

Ocular Exam

Vision was 20/25 and 20/20, right and left eyes respectively. Intraocular pressures, pupils, nerves and anterior segments were unremarkable. Fundus examination of the right eye revealed subretinal fluid under fovea and inferior macula with no associated hemorrhage or exudates (Figure 1). The left eye was unremarkable (Figure 2).

Figure 1
Figure 2

Differential Diagnosis

  • Diabetic Retinopathy
  • Sickle Cell Retinopathy
  • Idiopathic multiple bilateral macroaneurysms
  • Macroaneurysms secondary to poorly controlled hypertension
  • Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome

Diagnostic Testing

On fluorescein angiography of both eyes, there were extensive areas of peripheral ischemia, focal retinal vasculitis, macroaneurysms, telangiectatic vessels, and evidence of prior laser (Figures 3 & 4).

Figure 3
Figure 4

Optical coherence tomography of the right eye showed subfoveal outer retinal atrophy and scarring (Figure 5), while the left eye had mild retinal striae (Figure 6).

Figure 5
Figure 6


The examination was consistent with “idiopathic retinal vasculitis-aneurysms-neuroretinitis” (IRVAN) syndrome.


The patient was followed closely for the development of vision-threatening complications. Given the absence of macular edema and neovascularisation, no treatment was needed at this time.


The patient was followed closely for the development of vision-threatening complications. Given the absence of macular edema and neovascularisation, no treatment was needed at this time.

A number of treatments have been described in the literature, including laser photocoagulation, cryotherapy, corticosteroids, anti-VEGF injection, immunosuppressive medication, and surgical intervention (vitrectomy), with mixed results. (3-5)

The visual outcomes are variable, depending on the aggressiveness of the clinical course and timely intervention. Close follow-up is essential to prevent visual loss. (1-5)


Samuel MA, Equi RA, Chang TS, et al. Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system. Ophthalmology. 2007;114:1526–1529Karagiannis D, Soumplis V, Georgalas I,

Pichi F, Ciardella AP. Imaging in the Diagnosis and Management of Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN). Int Ophthalmol Clin. 2012 Fall;52(4):275-82.

Kandarakis A. Ranibizumab for idiopathic retinal vasculitis, aneurysms, and neuroretinitis: favorable results. Eur J Ophthalmol 2010; 20(4): 792 – 794

Cheema RA, Al-Askar E, Cheema HR. Infliximab Therapy for Idiopathic Retinal Vasculitis, Aneurysm, and Neuroretinitis Syndrome. J Ocul Pharmacol Ther. 2011 Aug;27(4):407-10

Basha M, Brown GC, Palombaro G, Shields CL, Shields JA.Management of IRVAN syndrome with observation.Ophthalmic Surg Lasers Imaging Retina. 2014 May 1;45 Online:e18-22.