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

Abstract

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.

IRVAN syndrome: A case report and a literature review

“…The epidemiology of the syndrome assured that the majority of patients were young females [ 1 , 5 ]. Our literature review included publications about cases of 10 females and 6 males, and the average age at the time of diagnosis was 28.8 years (range, 9–60 years) [ [2] , [3] , [4] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] ]. Whereas, Samuel et al study determined the average age of diagnosis at 31.5 years (range, 9–60 years) in their study which involved 22 patients.…”

“…According to a study, there was no association between IRVAN and any systemic disorder [ 6 ]. The majority of reviewed cases had a clear medical history and negative serum tests [ [2] , [3] , [4] , [10] , [11] , [12] , [13] , [14] , [15] , 17 ], in comparison to positive human leukocyte antigen B51 (HLA-B51) and antinuclear antibodies (ANA) at 1/50 titer in one case [ 9 ]. AM Abu El-Asrar et al reported a case of association between Allergic fungal sinusitis (AFS) and IRVAN syndrome.…”

“…Our patient was diagnosed at stage-3 and stage-2 for the LE and RE, respectively. Several symptoms were recorded in the literature such as progressive painless diminution of vision [ 10 ], bilateral blurred vision [ 12 ], sudden vision loss [ 13 ], exotropia, and central scotoma [ 4 ]. However, our patient was presented with unilateral floaters which were not reported before.…”

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

“…Authors have used OCTA to evaluate disk, posterior pole macroaneurysms, and in some cases, CNP. 6 , 27 , 34 , 48 It can clearly demonstrate vascular changes, including hypoperfusion, 27 aneurysmal changes, retinal neovascularization, and vascular remodeling which can be sometimes obscured by leakage and edema on FA. 6 , 27 , 34 , 48 It is particularly helpful in cases with disk aneurysms which are hard to discern on FA due to leakage and overlying neuroretinitis.…”

“…OCTA can delineate CNP areas with sensitivity equal to or better than FA; however, a smaller field of view limits its ability to detect peripheral CNP. 48 It can be compensated by obtaining scans from multiple areas and montaging multiple views. Nonetheless, some newer devices with updated software can provide up to 200° field of view and can be used for peripheral retina as well.…”