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

Chong, Yu Jeat; Logeswaran, Abison; Sarmad, Ambreen; Damato, Erika M.

doi:10.1007/s00417-019-04291-5

Cited by 8 publications

(18 citation statements)

References 9 publications

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“… 14 , 32 Eyes that require long-term immunosuppression are maintained on DMARDs for stable visual outcome in the long term. 12 , 41 , 47 , 52 Stage II and III eyes are usually treated with laser photocoagulation alone or in combination with systemic steroids to avoid permanent vision loss from progressive ischemia and its sequelae. Some authors demonstrated combination therapy with laser photocoagulation and intravitreal anti-VEGF injections.…”

Section: Discussionmentioning

confidence: 99%

Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series

Khan

Shahzad

et al. 2022

Ophthalmol Eye Dis

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Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare spectrum of retinal vasculitis, aneurysms, and neuroretinitis affecting young individuals in their third decade. Most of our current knowledge is based on case reports, case series, and a handful of collaborative studies. There is much diversity in treatment approaches and outcomes in the reported literature. We have aggregated published case reports and case series into quantitative and narrative synthesis to draw evidence-based conclusions toward clinical features, atypical and rare findings, systemic associations, disease course, and treatment outcomes. The analysis suggested the disease mostly affects young individuals with a female predilection. Anterior chamber and vitreous inflammation are common than previously believed. The most prevalent pattern of retinal vasculitis in IRVAN eyes is mixed vasculitis, followed by arteritis and phlebitis. Most eyes at the time of presentation have capillary nonperfusion and require treatment. Most eyes retain good visual acuity; however, treatment is required to maintain visual function. Intravitreal antivascular endothelial growth factors administered as an adjunct to retinal laser photocoagulation are more likely to improve visual outcomes. Besides, we have discussed the different hypotheses on the etiopathogenesis of the disease and stronger evidence suggests an inflammatory origin of the disease.

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Section: Discussionmentioning

confidence: 99%

Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series

Khan

Shahzad

et al. 2022

Ophthalmol Eye Dis

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“…This syndrome affects young healthy adults, with a female preponderance. 1,2 Its diagnosis is based on three major and three minor criteria, as established by Chang et al in 1995. 1 The major criteria, which are retinal vasculitis, aneurysmal dilatation, and neuroretinitis, conferred to this entity its denomination as IRVAN syndrome (Idiopathic Retinal Vasculitis, Aneurysms, Neuroretinitis).…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Its diagnosis is based on three major and three minor criteria, as established by Chang et al in 1995. 1 The major criteria, which are retinal vasculitis, aneurysmal dilatation, and neuroretinitis, conferred to this entity its denomination as IRVAN syndrome (Idiopathic Retinal Vasculitis, Aneurysms, Neuroretinitis). Recently, a study conducted by Chawla et al has put into question the etiopathogenesis of this syndrome as they found no evidence of a true disc edema.…”

Section: Discussionmentioning

confidence: 99%

“…Indocyanine green angiography may be useful to illustrate the pattern of papillary macroaneurysms. 1,3…”

Section: Discussionmentioning

confidence: 99%

“…Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) is a rare clinical entity that was first described by Kincaid and Shatz in 1983 and then better illustrated with the series of Chang et al in 1995. 1,2 Its pathophysiology is supposed to be related to intraocular inflammation, resulting in vasculitis and optic disc edema. 3 Multimodal imaging, including Optical Coherence Tomography Angiography (OCT-A), is nowadays helping to better elucidate the limited understanding of its mechanisms.…”

Section: Introductionmentioning

confidence: 99%

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Optical Coherence Tomography Angiography in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome: A case report

Ouederni

Sassi

Chelly

et al. 2020

European Journal of Ophthalmology

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Purpose: Since its first description by Chang et al. in 1995, the diagnosis of Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome has been based on the findings of Fundus Fluorescein Angiography (FFA). Our purpose was to describe the utility of optical coherence tomography angiography (OCT-A) in its diagnosis and management. Case description: A 40-year-old female presented with bilateral blurred vision. Her best corrected visual acuity was at 8/10. Fundus examination revealed blurred disc margins, perivascular exudates, arterial sheaths and retinal hemorrhages bilaterally. FFA showed staining of the optic disc with dye leakage in the right eye, a punctuate hyperfluorescence of the temporosuperior artery in the left eye, bilateral vascular sheathing and capillary dropout. OCT-A showed simultaneous presence of papillary aneurysm and neovascularization of the optic nerve head in the right eye, a papillary aneurysm in the left eye and bilateral capillary non-perfusion. Our patient was diagnosed with IRVAN syndrome. Oral steroids associated with panretinal laser photocoagulation and intravitreal injection of bevacizumab in the right eye resulted in vanishing of the papillary neovascularization with no recurrence on OCT-A at 10-month follow-up. Conclusions: OCT-A is an additional tool to FFA for visualization of arterial macroaneurysms and retinal neovascularization without the interference of dye leakage. It well demarcates nonperfused areas and ensures follow-up of retinal neovascularization. Its limitations are the limited field of view and the low sensitivity in detecting arteriolar dilations. Thus, OCT-A is unable to outplace FFA but should be considered alternately with it for non-invasive follow-up of IRVAN syndrome.

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Treatment Strategies for Neuroretinitis: Current Options and Emerging Therapies

Fairbanks

Starr

Chen

et al. 2019

Curr Treat Options Neurol

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

Cited by 8 publications

References 9 publications

Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series

Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series

Optical Coherence Tomography Angiography in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome: A case report

Treatment Strategies for Neuroretinitis: Current Options and Emerging Therapies

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