Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi 110029, India.
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi 110029, India.
Med Hypotheses. 2018 Feb;111:12-14. doi: 10.1016/j.mehy.2017.10.031. Epub 2017 Dec 12.
Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a rare entity of unknown etiology for which many hypotheses have been proposed with inflammation being the most commonly accepted hypothesis. We report cases of a 9 year old girl and a 22 year old male patient with diagnosis of IRVAN syndrome. The conclusions drawn from the clinical examination and multimodal imaging including optical coherence tomography angiography of the patients are discussed. Our conclusions and interpretation point towards IRVAN being a developmental vascular anomaly rather than a consequence of inflammation. It is thus proposed to be renamed as Idiopathic retinal arteriolar aneurysm syndrome (IRAA).
特发性视网膜血管炎、动脉瘤和神经视网膜炎（IRVAN）综合征是一种罕见的病因不明的疾病，为此提出了许多假说，其中炎症是最被广泛接受的假说。我们报告了两例 IRVAN 综合征患者，分别为 9 岁女孩和 22 岁男性。讨论了从临床检查和多模态成像（包括光学相干断层扫描血管造影）得出的结论。我们的结论和解释表明，IRVAN 是一种发育性血管异常，而不是炎症的结果。因此，建议将其更名为特发性视网膜小动脉动脉瘤综合征（IRAA）。
