Paracentral Acute Middle Maculopathy in IRVAN Syndrome: A Case Report Highlighting Vascular Mechanisms.

IF 2.6 4区 医学 Q2 OPHTHALMOLOGY
Ana Paula Couto, Giovanna Provenzano, Bruno F Fernandes, Raul N G Vianna
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引用次数: 0

Abstract

Purpose: To describe a rare case of paracentral acute middle maculopathy (PAMM) in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome, highlighting potential vascular mechanisms underlying this association.

Methods: Observational Case report.

Results: An 18-year-old male previously diagnosed with bilateral IRVAN syndrome presented with a sudden paracentral scotoma in the left eye. Multimodal imaging showed multiple macroaneurysms, retinal hemorrhages, focal arteriolar vasculitis, and lipid exudation in both eyes. Swept-source optical coherence tomography (SS-OCT) of the left eye revealed a hyperreflective band in the inner nuclear layer in the paracentral temporal superior macular area, consistent with PAMM, without clear evidence of arterial compression. A conservative approach was chosen, and after three years of follow-up, retinal features and visual acuity remained stable.

Conclusion: PAMM may manifest in IRVAN independent of detectable localized aneurysm compression of the deep capillary plexus. The findings underscore the importance of multimodal imaging and long-term monitoring to assess ischemic complications. Further investigations are warranted to clarify the pathophysiological relationship between these conditions.

IRVAN综合征的中央旁急性中黄斑病变:一个强调血管机制的病例报告。
目的:描述一例罕见的伴有特发性视网膜血管炎、动脉瘤和神经性视网膜炎(IRVAN)综合征的患者并发中央旁急性中黄斑病变(PAMM)的病例,强调这种关联的潜在血管机制。方法:观察性病例报告。结果:一名18岁男性,先前诊断为双侧IRVAN综合征,在左眼出现突发性中央旁暗斑。多模态影像显示双眼多发大动脉瘤、视网膜出血、局灶性小动脉血管炎及脂质渗出。左眼扫源光学相干断层扫描(SS-OCT)显示黄斑颞上区中央旁核层高反射带,与PAMM一致,无明显动脉压迫证据。术后随访3年,视网膜特征和视力保持稳定。结论:PAMM可能表现在IRVAN中,而不依赖于可检测到的深部毛细血管丛局部动脉瘤压迫。研究结果强调了多模态成像和长期监测对评估缺血性并发症的重要性。进一步的研究是必要的,以澄清这些条件之间的病理生理关系。
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来源期刊
CiteScore
6.20
自引率
15.20%
发文量
285
审稿时长
6-12 weeks
期刊介绍: Ocular Immunology & Inflammation ranks 18 out of 59 in the Ophthalmology Category.Ocular Immunology and Inflammation is a peer-reviewed, scientific publication that welcomes the submission of original, previously unpublished manuscripts directed to ophthalmologists and vision scientists. Published bimonthly, the journal provides an international medium for basic and clinical research reports on the ocular inflammatory response and its control by the immune system. The journal publishes original research papers, case reports, reviews, letters to the editor, meeting abstracts, and invited editorials.
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