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

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.…”

“…However, our patient was presented with unilateral floaters which were not reported before. We found in our review that the most common symptoms were blurred vision [ 3 , 12 , 16 ] and vision loss [ 13 ]. There are six criteria to diagnose IRVAN syndrome, three major criteria (numerous aneurysmal dilatations, neuroretinitis, and retinal vasculitis in arterial bifurcation), and three minor criteria (retinal neovascularization, macular exudation, and peripheral capillary perfusion defect) [ 1 ].…”

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

“… 17 – 19 While the aneurysms tend to regress or resolve, there may develop newer aneurysms adjacent to the site of previous aneurysms. 20 , 21 Different mechanisms behind the development of macroaneurysms in IRVAN syndrome have been discussed. Most authors believe segmental retinal vascular inflammation may cause vessel wall weakening and subsequent ectatic changes at the site of weakened vessels.…”