Is There a True Neuroretinitis in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome ?

ABSTRACT

Purpose

To clarify whether a true neuroretinitis is part of  idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome.

Methods

Critical literature review.

Results

Neuroretinitis has been considered as a prominent feature of IRVAN syndrome. It has been described as late diffuse optic  disc staining on fluorescein angiography. However, patients with IRVAN syndrome usually do not present with visual function alterations consistent with optic neuropathy or neuroretinitis. The typical stellate exudative maculopathy seen in IRVAN syndrome is primarily caused by leakage from retinal arteriolar aneurysms at or near the optic disc, and it could be misinterpreted as a feature of neuroretinitis.

Conclusion

There is no clinical evidence of true neuroretinitis at any of the disease stages in patients with IRVAN syndrome. The acronym « IRVARE » (Idiopathic Retinal Vasculitis, Aneurysms, and Retinal Exudates) is proposed to better characterize this syndrome.

KEYWORDS:

• Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome
• neuroretinitis
• arterial aneurysms
• retinal exudates
• fluorescein angiography

Acknowledgments

This work has been supported by the Ministry of Higher Education and Research of Tunisia.

Declaration of interest

The authors have no conflicts of interest to declare.