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

Graefes Arch Clin Exp Ophthalmol. 2019 May;257(5):1013-1018. doi: 10.1007/s00417-019-04291-5. Epub 2019 Mar 15.

Abstract

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

Method: Case series PATIENTS: Six eyes from three patients were included.

Results: 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.

Conclusion: 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.

Keywords: Medical retina; Multimodal imaging; Uveitis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aneurysm / diagnosis*
  • Female
  • Fluorescein Angiography / methods*
  • Follow-Up Studies
  • Fundus Oculi
  • Humans
  • Laser Coagulation
  • Male
  • Multimodal Imaging*
  • Retinal Artery*
  • Retinal Vasculitis / diagnosis*
  • Retinal Vasculitis / surgery
  • Retinitis / diagnosis*
  • Retinitis / surgery
  • Tomography, Optical Coherence / methods*
  • Young Adult