Skip to main content

Advertisement

SpringerLink
Log in

Further observations on a bilateral IRVAN syndrome case

  • Case Report
  • Published:
International Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

Reporting clinical features of the late course of the disease after long-term follow-up in a bilaterally affected patient with idiopathic retinitis, vasculitis, retinal aneurysms and neuroretinitis (IRVAN) and new peripheral retinal findings on wide-field angiography and multimodal imaging.

Methods

This was a retrospective observational study, based on current diagnostic studies including wide-field fluorescein angiography, optical coherence tomography (OCT) and treatment of aneurysms with argon laser photocoagulation.

Results

A 21-year-old female with bilateral IRVAN syndrome—stage 2 in the right eye and stage 3 in the left eye—previously treated bilaterally with laser photocoagulation for retinal macroaneurysms and ischemic areas between 1985 and 1992. Follow-up interrupted on two occasions, with subsequent fundus re-examinations confirming the prevention of retinal neovascularization in both eyes, as recently evidenced on wide-field angiography.

Conclusions

A case of bilateral IRVAN disease with multiple retinal aneurysms, neuroretinitis and peripheral capillary nonperfusion successfully treated with laser photocoagulation, maintaining normal visual acuity in one eye and preventing retinal neovascular complications.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10

Similar content being viewed by others

References

  1. Karel I, Peleska M, Divisová G (1973) Fluorescence angiography in retinal vasculitis in children´s uveitis. Ophthalmologica 166:251–264

    Article  CAS  PubMed  Google Scholar 

  2. Verdaguer J (1978) Enfermedades vasculares de la retina y su tratamiento. Conferencia Charlin, Arch Chil Oftalm, vol XXXV no 1, pp 35–38

  3. Kincaid J, Schatz H (1983) Bilateral retinal arteritis with multiple aneurysmal dilatations. Retina 3:171–178

    Article  CAS  PubMed  Google Scholar 

  4. Melan P, Ciurana AJ, Boudet C (1986) Arterie retinienne bilaterale avec dilatations aneurysmalls multiples. J Fr Ophthalmol 9:23–28

    Google Scholar 

  5. Chang TS, Aylward GW, Davis JL, Mieler WF, Oliver GL, Maberley AL et al (1995) Idiopathic retinal vasculitis aneurysms and neuroretinitis. Retinal vasculitis study. Ophthalmology 102:1089–1097

    Article  CAS  PubMed  Google Scholar 

  6. Samuel MA, Equi RA, Chang TS, Mieler W, Jampol LM, Hay D, Yannuzzi LA (2007) Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system. Ophthalmology 114(1526–1529):e1

    Google Scholar 

  7. Basha M, Brown GC, Palombaro G, Shields CL, Shields JA (2014) Management of IRVAN syndrome with observation. Ophthalmic Surg Lasers Imaging Retina 1:45

    Google Scholar 

  8. Owens SL, Gregor ZJ (1992) Vanishing retinal arterial aneurysms: a case report. Br J Ophthalmol 76:636–638

    Article  Google Scholar 

  9. Yeshurun I, Recillas-Gispert C, Navarro-Lopez P et al (2003) Extensive dynamics in location, shape, and size of aneurysms in a patient with idiopathic retinal vasculitis, aneuryms, and neuroretinitis (IRVAN) syndrome. Am J Ophthalmol 135:118–120

    Article  PubMed  Google Scholar 

  10. Sawhney GK, Payne JF, Ray R et al (2013) Combination anti-VEGF and corticosteroid therapy for idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. Ophthalmic Surg Lasers Imaging Retina 44(6):599–602

    Article  PubMed  Google Scholar 

  11. Empeslidis T, Banerjee S, Vardarinos A et al (2013) Dexamethasone intravitreal implant for idiopathic retinal vasculitis, aneurysms, and neuroretinitis. Eur J Ophthalmol 23(5):757–760

    Article  PubMed  Google Scholar 

  12. Osman Saatci A, Ayhan Z, Takes O, Yaman A, Soylev Bajin FM (2015) Single Bilateral Dexamethasone implant in addition to panretinal photocoagulation and oral azathioprine treatment in IRVAN Syndrome. Case Rep Ophthalmol 6:56–62

    Article  Google Scholar 

  13. Akesbi J, Brousseaud FX, Adam R et al (2010) Intravitreal bevacizumab (Avastin) in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. Acta Ophthalmol 88:e40–e41

    Article  PubMed  Google Scholar 

  14. Karagiannis D, Soumplis V, Georgalas I, Kandarakis A (2010) Ranibizumab for idiopathic retinal vasculitis, aneurysms, and neuroretinitis: favorable results. Eur J Ophthalmol 20:792–794

    Article  PubMed  Google Scholar 

  15. Cheema RA, Al-Askar E, Cheema HR (2011) Infliximab therapy for idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome. J Ocul Pharmacol Ther 27:407–410

    Article  CAS  PubMed  Google Scholar 

  16. Tomita M, Matsubara T, Yamada H et al (2004) Long term follow up in a case of successfully treated idiopathic retinal vasculitis, aneurysms, and neuro retinitis (IRVAN). Br J Ophthalmol 88(2):302–303

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Rouvas A, Nikita E, Markomichelakis N et al (2013) Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment. J Ophthalmic Inflamm Infect 3:21

    Article  PubMed  PubMed Central  Google Scholar 

  18. Kowal L, Steiner H (1991) Arterial macroaneurysm of the optic disc. Aust N Z J Ophthalmol 19(1):75–77

    Article  CAS  PubMed  Google Scholar 

  19. Soheilian M, Nourinia R, Tavallali A, Peyman GA (2010) Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody (P-ANCA). Retinal Cases Brief Rep 4:198–201

    Article  Google Scholar 

  20. Diloreto DA Jr, Sadda SR (2003) Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) with preserved perfusion. Retina 23:554–557

    Article  PubMed  Google Scholar 

  21. Alasil T, Ketner S, Coady PA, Adelman RA, Nwanyanwu KH (2016) Idiopathic retinal vasculitis, aneurysms and neuroretinitis case report. Am J Ophthalmol Case Rep 2:33–36

    Article  PubMed  PubMed Central  Google Scholar 

  22. Pichi F, Ciardella AP (2012) Imaging in the diagnosis and management of idiopathic retinal vasculitis, aneurysms, and neuroretinintis. Int Ophthalmol Clin 52(4):275–282

    Article  PubMed  Google Scholar 

  23. Shah SP, Jain A, Coffee RE, McCannel TA (2009) Optos panoramic 200MA ultrawide-field imagining of peripheral RPE adenoma. Semin Ophthalmol 24:37–39

    Article  PubMed  Google Scholar 

  24. Kang KB, Wessel MM, Tong J et al (2013) Ultra-widfield imaging for the management of pediatric retinal diseases. J Pediatr Ophthalmol Strabismus 50:282–288

    Article  PubMed  Google Scholar 

  25. Shah A, Abbey A, Yonekawa Y, Khandan S, Wolfe J, Trese M, Williams G, Capone A (2016) Widefield fluorescein angiography in patients without peripheral disease. A study of normal peripheral findings. Retina 36:1087–1092

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We thank to Jean J Delaey MD and Rosario Camacho for draft manuscript review. Also thanks to Marcela Valencia MD, Claudia Castaño, Claudia Rojas and Julieth Cortés for their collaboration.

Author information

Authors and Affiliations

  1. Fundación Oftalmológica Nacional, Carrera 13 #13-50, Bogotá D.C., Colombia

    Álvaro Rodríguez, Willy Carpio-Rosso & Francisco J. Rodríguez

  2. Universidad del Rosario, Bogotá D.C., Colombia

    Álvaro Rodríguez, Willy Carpio-Rosso & Francisco J. Rodríguez

Authors
  1. Álvaro Rodríguez
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Willy Carpio-Rosso
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Francisco J. Rodríguez
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Álvaro Rodríguez.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Other methods of treatment, currently available, can be used for comparison, if required. Written informed consent was obtained from the patient for publication of this bilateral case report and the accompanying images.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Rodríguez, Á., Carpio-Rosso, W. & Rodríguez, F.J. Further observations on a bilateral IRVAN syndrome case. Int Ophthalmol 39, 1179–1186 (2019). https://doi.org/10.1007/s10792-018-0917-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10792-018-0917-z

Keywords

Search

Navigation