Extensive Dynamics of Aneurysms and Long-term Prognosis in IRVAN Syndrome: A Case Series

Ocul Immunol Inflamm. 2022 Apr 3;30(3):623-627. doi: 10.1080/09273948.2020.1821902. Epub 2020 Sep 29.

Abstract

Objectives: To report the extensive dynamic clinical condition and long-term therapeutic prognosis in three cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome.

Methods: Retrospective, interventional case series.

Results: Data of the three IRVAN syndrome patients were retrospectively reviewed at study enrollment and during the follow-up period. The mean follow-up time was 60 months. All three patients manifested the typical characteristics of IRVAN syndrome with bilateral eye involvement. The good long-term visual acuity has been maintained after treatment. The aneurysms showed extensive dynamic changes and regressed mostly.

Conclusion: IRVAN syndrome is a rare condition that may progress rapidly and cause severe vision loss without treatment. The photocoagulation is beneficial and should be performed in the early phase. The aneurysms presented extensive dynamic changes through multimodal imaging which suggested a migratory inflammatory process involving retinal arterioles.

Keywords: IRVAN syndrome; extensive dynamics of aneurysms; therapeutic prognosis.

MeSH terms

  • Aneurysm* / diagnosis
  • Aneurysm* / surgery
  • Dementia
  • Fluorescein Angiography / methods
  • Hearing Loss, Central
  • Humans
  • Optic Atrophy
  • Prognosis
  • Retinal Vasculitis* / drug therapy
  • Retinitis* / drug therapy
  • Retrospective Studies

Supplementary concepts

  • Jensen syndrome