Department of Ophthalmology, Fattouma Bourguiba University Hospital, Faculty of Medicine, University of Monastir, Monastir, Tunisia.
Department of Ophthalmology, Fattouma Bourguiba University Hospital, Faculty of Medicine, University of Monastir, Monastir, Tunisia.
J Fr Ophtalmol. 2022 Nov;45(9):1012-1020. doi: 10.1016/j.jfo.2022.04.015. Epub 2022 Jul 16.
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.
Retrospective review of the charts of nine patients (18 eyes) diagnosed with IRVAN syndrome at Fattouma Bourguiba University Hospital, Monastir, Tunisia, from January 1, 2011 to January 1, 2022.
Nine patients were included with bilateral involvement in all cases. Mean initial best-corrected visual acuity (VA) was 20/32 (range, 20/1600-20/20). Clinical findings at presentation included vitreous cells (10 eyes, 55.6%), peripapillary exudates (12 eyes, 66.7%), partial or complete macular star (11 eyes, 61.1%), and vascular sheathing (11 eyes, 61.1%). Fluorescein angiography showed arteriolar aneurysms (18 eyes, 100%), areas of peripheral capillary non-perfusion (16 eyes, 88.9%), and retinal neovascularization (6 eyes, 33.3%). Optical coherence tomography showed macular edema in 5 eyes (27.8%). Optical coherence tomography angiography of the optic disc demonstrated papillary aneurysms in 4 eyes of 2 patients. Indocyanine green angiography showed retinal arteriolar aneurysmal dilatations in 4 eyes of 2 patients. Ten eyes (55.6%) had stage 2 disease, 6 eyes (33.3%) had stage 3, and 2 eyes (11.1%) had stage 1. Treatment modalities included peripheral photocoagulation (16 eyes, 88.9%), intravitreal bevacizumab (4 eyes, 22.2%), and intravitreal triamcinolone acetonide (1 eye, 5.6%). Mean final best-corrected VA was 20/32 (range, 20/600-20/20). Ocular complications included vitreous hemorrhage in 3 eyes (16.7%), branch retinal artery occlusion in 2 eyes (11.1%) and submacular fibrosis in 3 eyes (16.7%).
IRVAN syndrome should be highly suspected in patients with peripapillary exudates associated with vascular sheathing and vitreous cells. Multimodal imaging confirms the diagnosis by showing retinal macroaneurysms. Early treatment of macular edema and/or peripheral retinal non-perfusion is mandatory to improve prognosis.
描述 9 例（18 只眼）特发性视网膜血管炎、动脉瘤和神经视网膜炎（IRVAN）综合征患者的临床特征、相关影像学表现、疾病过程和治疗反应。
对 2011 年 1 月 1 日至 2022 年 1 月 1 日在突尼斯莫纳斯提尔法图玛·布尔吉巴大学医院诊断为 IRVAN 综合征的 9 例（18 只眼）患者的病历进行回顾性分析。
9 例患者均为双侧受累。初始最佳矫正视力（BCVA）平均为 20/32（范围，20/1600-20/20）。发病时的临床发现包括玻璃体细胞（10 只眼，55.6%）、视盘周围渗出物（12 只眼，66.7%）、部分或完全黄斑星状病变（11 只眼，61.1%）和血管鞘（11 只眼，61.1%）。荧光素血管造影显示小动脉动脉瘤（18 只眼，100%）、周边毛细血管无灌注区（16 只眼，88.9%）和视网膜新生血管（6 只眼，33.3%）。光学相干断层扫描显示 5 只眼（27.8%）存在黄斑水肿。2 例 4 只眼的视神经盘光学相干断层血管造影显示乳头动脉瘤。吲哚青绿血管造影显示 2 例 4 只眼视网膜小动脉动脉瘤样扩张。10 只眼（55.6%）为 2 期疾病，6 只眼（33.3%）为 3 期，2 只眼（11.1%）为 1 期。治疗方法包括周边光凝（16 只眼，88.9%）、玻璃体内贝伐单抗（4 只眼，22.2%）和玻璃体内曲安奈德（1 只眼，5.6%）。最终最佳矫正视力平均为 20/32（范围，20/600-20/20）。眼部并发症包括 3 只眼（16.7%）玻璃体出血、2 只眼（11.1%）视网膜分支动脉阻塞和 3 只眼（16.7%）黄斑下纤维化。
特发性视网膜血管炎、动脉瘤和神经视网膜炎（IRVAN）综合征患者应高度怀疑视盘周围渗出物伴血管鞘和玻璃体细胞。多模态成像通过显示视网膜大动脉瘤来确认诊断。早期治疗黄斑水肿和/或周边视网膜无灌注是改善预后的必要条件。
Zotero 插件