Presenters: Dr Imen Ksiaa, Dr Sourour Zina, Dr Moncef Khairallah
Department of Ophthalmology, Fattouma Bourguiba University Hospital
Monastir, Tunisia
This was a case of an overlooked early-stage 1 IRVAN syndrome presenting with branch retinal artery occlusion which progressed to stage 2 IRVAN syndrome less than one year later.
Case Summary:
A 33-year-old woman, with unremarkable past medical history, was referred to our department for the evaluation of branch retinal artery occulsion (BRAO in the right eye (RE) which had occurred 8 months earlier. Etiologic work-up had been performed, showing negative or normal results. An underlying early stage 1 IRVAN syndrome had been initially missed, but the diagnosis became evident at our examination as the disease had progressed to stage 2. Best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. The hard exudates had increased, and fluorescein angiography clearly delineated bilateral typical retinal arteriolar macroaneuryms and showed extensive areas of peripheral retinal capillary nonperfusion without new vessels in both eyes.
Optical coherence tomography (OCT) showed a localized retinal thinning corresponding to the prior BRAO. Fundus autofluorescence showed nodulo-linear periarterial hypoautofluorescence. OCT angiography (OCTA) showed localized ischemic changes, mainly involving the deep capillary plexus, corresponding to the area of resolved BRAO. It also clearly delineated the optic disc aneurysms. The patient received bilateral scatter laser photocoagulation directed to areas of peripheral capillary nonperfusion. Over a 6-month follow-up period, visual acuity remained unchanged, and there was no evidence of disease progression. There are only a few reported cases of IRVAN syndrome complicated with BRAO or more rarely central retinal artery occlusion (CRAO) [1, 2]. Multimodal imaging approach including fluorescein angiography, OCT, and OCTA is essential in the diagnosis, monitoring of disease progression, and treatment. Treatment options include early panretinal photocoagulation to areas of peripheral ischemia, intravitreal injections of anti-VEGF or steroids for exudative and/or edematous maculopathy. The role of systemic corticosteroidand immunosuppressive therapy to control intraocular inflammation remains controversial. Anti TNF alpha agents recently showed promising results in controlling disease activity [3].
- Zacharia JA, Chin AT, Rebhun CB, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome present[1]ing with branch retinal artery occlusion. Ophthalmic Surg Lasers Imaging Retina 2017; 48(11): 948–995.
- Terrada C, Dethorey G, Ducos G, et al. Spontaneous branch artery occlusion in idiopathic retinitis, vasculitis, aneu[1]rysms, and neuroretinitis syndrome despite panretinal laser photocoagulation of widespread retina nonperfusion. Acta Ophthalmol 2011; 89(6): e542–e543.
- Cheema RA, Al-Askar E, Cheema HR. Infliximab therapy for idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome. J Ocul Pharmacol Ther. 2011 Aug;27(4):407-10. doi: 10.1089/jop.2010.0189. Epub 2011 May 12. PMID: 21563921.
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Presented during EURETINA Case Club – Series 1, Episode 8 with Bahram Bodaghi and Carlos Pavesio (29 Jun, 2022) – RECORDING AVAILABLE TO MEMBERS ONLY.
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