Author: Sana Khochtali (Tunisia)
Co-authors: Molka Khairallah, Olfa Beizig, Sakr Bouchoucha, Nesrine Abroug, Moncef Khairallah
To describe clinical features, relevant imaging findings, disease course, and response to treatment in 9 patients (18 eyes) with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome.
Department of Ophthalmology, Fattouma Bourguiba University Hospital, Monastir, Tunisia.
The charts of nine patients (18 eyes) diagnosed with IRVAN syndrome were retrospectively reviewed. Detailed ophthalmic examination, fundus photography, fluorescein angiography (FA), and optical coherence tomography (OCT) were performed in all patients. OCT angiography was performed in 2 of 9 patients. Indocyanine green (ICG) angiography was performed in 2 of 9 patients. Mean follow-up period was 35.4 months (range, 15-84).
Mean age was 33.5 years (4 males and 5 females). Mean initial best-corrected visual acuity (BCVA) was 20/32 (range, 20/1600-20/20). Visual blurring was the presenting symptom in 7 patients. Clinical findings included peripapillary exudates in 11 eyes, partial or complete macular star in 11 eyes, multiple aneurysmal dilatations of the retinal and/or optic nerve-head arterioles in 8 eyes, and vascular sheathing in 11 eyes. There were associated vitritis in 8 eyes, vitreous hemorrhage in 3 eyes, and branch retinal artery occlusion in 2 eyes. FA showed arteriolar aneurysms in 15 eyes, areas of peripheral capillary nonperfusion in 14 eyes, and retinal neovascularization in 3 eyes. OCT showed macular edema in 5 eyes. OCT angiography demonstrated papillary aneurysms in 4/4 eyes. ICG angiography showed retinal arteriolar aneurysmal dilatations in 4/4 eyes. Thirteen eyes had the stage 2 of the disease, 3 eyes had the stage 3, and 2 eyes had the stage 1. Treatment modalities included peripheral scatter photocoagulation (14 eyes), intravitreal bevacizumab (4 eyes), and intravitreal triamcinolone acetonide (1 eye). Final BCVA was on average 20/32 (range, 20/1600-20/20). It was less than or equal to 20/200 in 5 eyes. There was a persistent subretinal macular fibrosis in 3 eyes.
IRVAN is a rare idiopathic ocular condition. Multimodal imaging provides valuable clues for the diagnosis. Treatment is based on photocoagulation of areas of retinal capillary non-perfusion before the development of neovascularization, and on anti-vascular endothelial growth factor intravitreal injections for macular edema secondary to chronic exudation from arteriolar aneurysms. Prognosis may be guarded despite treatment.
No financial interest for all authors