Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): case series of three patients with multimodal imaging

  • Yu Jeat ChongEmail author
  • Abison Logeswaran
  • Ambreen Sarmad
  • Erika Marie Damato
Inflammatory Disorders



The purpose of this study was to report the clinical presentation, disease progression, treatment and complications of IRVAN.


Case series


Six eyes from three patients were included.


All eyes were treated with pan-retinal photocoagulation (PRP). One eye received Ozurdex (dexamethasome implant) for persistent macular exudates and oedema. One eye received Avastin injections for retinal neovascularization. Oral steroids were given to all patients at some point during the disease process. One patient had additional immunosuppression with mycophenolate mofetil. Despite aggressive PRP, the visual outcomes varied widely. One patient maintained 6/6 vision bilaterally at 84 months follow-up. The second patient had progressive visual loss secondary to macular exudates and oedema, from 6/9 right eye, 6/6 left eye to 6/18 right eye, 6/60 left eye within 12 months despite Ozurdex injection. The third patient’s vision at presentation was 6/5 right eye, and 6/4 left eye. Despite further interventions including Avastin and mycophenolate mofetil, he continued to have progressive neovascularization and recurrent vitreous haemorrhage. At 72 months, his vision had deteriorated to 6/60 right eye, 6/18 left eye.


The progression of IRVAN can vary greatly, in spite of aggressive treatment with PRP, oral and intravitreal steroids, immunosuppressant medication and anti-VEGF agents. The variation in disease progression occurs both within the same individual as well as between individuals. An individualised approach to therapy is advocated.


Medical retina Multimodal imaging Uveitis 


Compliance with ethical standards

Conflict of interest

Dr. Erika Marie Damato has received speaker fees from AbbVie and Novartis in the past 12 months. All the other authors declare that they have no conflict of interest

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Consent not required due to retrospective, anonymous and observational nature of this series. The study was performed at the Birmingham Midland Eye Centre. This research was presented at the European Association for Vision and Eye Research conference in NICE (2017). There are no proprietary interests to declare.


  1. 1.
    Chang TS, Aylward GW, Davis JL et al (1995) Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Retinal Vasculitis Study. Ophthalmology 102(7):1089–1097CrossRefPubMedGoogle Scholar
  2. 2.
    Samuel MA, Equi RA, Chang TS et al (2007) Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system. Ophthalmology 114(8):1526–9.e1CrossRefPubMedGoogle Scholar
  3. 3.
    Sigler EJ, Grosso A (2016) Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. Retina Phila Pa 36(3):e16–e17CrossRefGoogle Scholar
  4. 4.
    Banaee T, Hosseini SM (2015) Optical coherence tomography features in idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome. J Ophthalmic Vis Res 10(2):193–196CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Marín-Lambíes C, Gallego-Pinazo R, Salom D et al (2012) Rapid regression of exudative maculopathy in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome after intravitreal ranibizumab. Case Rep Ophthalmol 3(2):251–257CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Saatci AO, Ayhan Z, Take_ Ö et al (2015) Single bilateral dexamethasone implant in addition to panretinal photocoagulation and oral azathioprine treatment in IRVAN syndrome. Case Rep Ophthalmol 6(1):56–62CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Basha M, Brown GC, Palombaro G et al (2014) Management of IRVAN syndrome with observation. Ophthalmic Surg Lasers Imaging Retina 45 Online:e18–e22PubMedGoogle Scholar
  8. 8.
    Ferris F (1996) Early photocoagulation in patients with either type I or type II diabetes. Trans Am Ophthalmol Soc 94:505Google Scholar
  9. 9.
    Akesbi J, Brousseaud FX, Adam R et al (2010) J.P. Intravitreal bevacizumab (Avastin®) in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. Acta Ophthalmol 88(2):e40–1Google Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Yu Jeat Chong
    • 1
    Email author
  • Abison Logeswaran
    • 2
  • Ambreen Sarmad
    • 1
  • Erika Marie Damato
    • 3
  1. 1.Birmingham Midland Eye CentreBirminghamUK
  2. 2.Moorfields Eye HospitalLondonUK
  3. 3.Addenbrookes HospitalCambridge University Hospitals NHS TrustCambridgeUK

Personalised recommendations