Abstract
Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) is a rare retinal vascular inflammatory disorder affecting young adults. The hallmark of this condition is the presence of aneurysmal dilatations of the retinal arterioles at or near the optic disc giving them a “knotted” appearance allowing the diagnosis to be made clinically in most cases. The exact etiology of the condition is unknown as yet. The clinical course of IRVAN can vary from spontaneous resolution to progressive ishcaemia, neovascularization, neovascular glaucoma and even blindness.
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Abu El-Asrar AM, Jestaneiah S, Al-Serhani AM. Regression of aneurysmal dilatations in a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) associated with allergic fungal sinusitis. Eye (Lond). 2004;18:197–9.
Akesbi J, Brousseaud FX, Adam R, Rodallec T, Nordmann JP. Intravitreal bevacizumab (Avastin) in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. Acta Ophthalmol. 2010;88:e40–1.
Basha M, Brown GC, Palombaro G, Shields CL, Shields JA. Management of IRVAN syndrome with observation. Ophthalmic Surg Lasers Imaging Retina. 2014;45:e18–22.
Chang TS, Aylward GW, Davis JL, Mieler WF, Oliver GL, Maberly AL, et al. Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis: the Retinal Vasculitis Study Group. Ophthalmology. 1995;102:1089–97.
Cheema RA, Al-Askar E, Cheema HR. Infliximab therapy for idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome. J Ocul Pharmacol Ther. 2011;27:407–10.
Empeslidis T, Banerjee S, Vardarinos A, Konstas AG. Dexamethasone intravitreal implant for idiopathic retinal vasculitis, aneurysms, and neuroretinitis. Eur J Ophthalmol. 2013;23:757–60.
Gharbiya M, Pantaleoni FB, Accorinti M, et al. Indocyanine green angiography in a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis. Eye (Lond). 2000;14:655–9.
Hammond MD, Ward TP, Katz B, Subramanian PS. Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome. J Neuroophthalmol. 2004;24:221–4.
Karagiannis D, Soumplis V, Georgalas I, Kandarakis A. Ranibizumab for idiopathic retinal vasculitis, aneurysms, and neuroretinitis: favorable results. Eur J Ophthalmol. 2010;20:792–4.
Karel I, Peleska M, Divisova G. Fluorescence angiography in retinal vasculitis in children’s uveitis. Ophthalmologica. 1973;166:251–64.
Kincaid J, Schatz H. Bilateral retinal arteritis with multiple aneurysmal dilatations. Retina. 1983;3:171–8.
Malan P, Ciurana AJ, Boudet C. Arterite retinienne bilaterale avec dilatations aneurysm ales multiples. J Fr Ophtalmol. 1986;9:23–8.
Owens SL, Gregor ZJ. Vanishing retinal arterial aneurysms: a case report. Br J Ophthalmol. 1992;76:637–8.
Pichi F, Ciardella AP. Imaging in the diagnosis and management of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN). Int Ophthalmol Clin. 2012;52:275–82.
Rouvas A, Nikita E, Markomichelakis N, Theodossiadis P, Pharmakakis N. Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment. J Ophthalmic Inflamm Infect. 2013;3:21. doi:10.1186/1869-5760-3-21.
Russell SR, Folk JC. Branch retinal artery occlusion after dye yellow photocoagulation of an arterial macroaneurysm. Am J Ophthalmol. 1987;104:186–7.
Saatci AO, Ayhan Z, Takes O, Yaman A, Bajin FM. Single bilateral dexamethasone implant in addition to panretinal photocoagulation and oral azathioprine treatment in IRVAN syndrome. Case Rep Ophthalmol. 2015;6:56–62.
Samuel MA, Equi RA, Chang TS, Mieler W, Jampol LM, Hay D, et al. Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system. Ophthalmology. 2007;114:1526–9.
Sashihara H, Hayashi H, Oshima K. Regression of retinal arterial aneurysms in a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis. Retina. 1999;19:250–1.
Sawhney GK, Payne JF, Ray R, Mehta S, Bergstrom CS, Yeh S. Combination anti-VEGF and corticosteroid therapy for idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. Ophthalmic Surg Lasers Imaging Retina. 2013;44:599–602.
Soheilian M, Nourinia R, Tavallali A, Peyman GA. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (irvan) syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody (P-ANCA). Retin Cases Brief Rep. 2010;4:198–201.
Yeshurun I, Recillas-Gispert C, Navarro-Lopez P, Arellanes-Garcia L, Cervantes-Coste G. Extensive dynamics in location, shape, and size of aneurysms in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis. Am J Ophthalmol. 2003;135:118–20.
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Dogra, M.R., Katoch, D. (2016). Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis (IRVAN). In: Gupta, V., Nguyen, Q., LeHoang, P., Herbort Jr., C. (eds) The Uveitis Atlas. Springer, New Delhi. https://doi.org/10.1007/978-81-322-2506-5_131-1
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DOI: https://doi.org/10.1007/978-81-322-2506-5_131-1
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