Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy: A Case Report.

IF 0.7 Q3 MEDICINE, GENERAL & INTERNAL
International Medical Case Reports Journal Pub Date : 2025-02-17 eCollection Date: 2025-01-01 DOI:10.2147/IMCRJ.S507569
Ana Faria Pereira, João Tavares-Ferreira, Renato Santos-Silva, Cláudia Oliveira-Ferreira
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引用次数: 0

Abstract

Purpose: Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare retinal disorder first described by Gass in 1988. Characterized by multifocal yellow-white lesions at the level of the retinal pigment epithelium (RPE) and associated with serous retinal detachments, AEPVM typically presents with acute visual disturbances and, in some cases, headaches. Despite its rarity, with fewer than 40 cases reported in the literature, its clinical course and etiology remain poorly understood. Patients generally experience gradual recovery of vision, though retinal abnormalities may persist. Various hypotheses suggest infectious, inflammatory, autoimmune, or even paraneoplastic mechanisms, but definitive causes remain elusive. In this report, we describe the clinical course and multimodal imaging findings of a patient diagnosed with AEPVM, contributing to the limited understanding of this condition's progression and management.

Case presentation: We report the case of a 40-year-old Caucasian female presenting with blurred vision for two weeks, with a best corrected visual acuity of 20/20 in both eyes, preceded by flu-like symptoms. Initial clinical evaluation, including fundus examination and spectral-domain optical coherence tomography (SD-OCT), revealed multiple small serous retinal detachments bilaterally. Multimodal imaging (fundus autofluorescence, fluorescein angiography, and indocyanine green angiography) appeared normal initially. A comprehensive systemic workup excluded autoimmune, infectious, and neoplastic etiologies. Upon follow-up, yellowish retinal lesions and hyperautofluorescence emerged, leading to the diagnosis of idiopathic AEPVM. The patient continues to be monitored for visual recovery and potential complications.

Conclusion: AEPVM is a rare macular disorder that requires comprehensive multimodal evaluation to establish an accurate diagnosis. Ruling out autoimmune, infectious, and especially neoplastic causes, including paraneoplastic syndromes, is critical for confirming its idiopathic nature. While visual recovery is common, the risk of recurrence and complications such as choroidal neovascularization necessitates vigilant long-term monitoring.

特发性急性渗出性多形黄斑病1例报告。
目的:急性渗出性多形性黄斑病变(AEPVM)是一种罕见的视网膜疾病,由Gass于1988年首次描述。AEPVM以视网膜色素上皮(RPE)水平的多灶黄白色病变为特征,并伴有浆液性视网膜脱离,典型表现为急性视力障碍,在某些情况下,还会出现头痛。尽管它很罕见,文献中报道的病例不到40例,但其临床过程和病因仍然知之甚少。患者通常会逐渐恢复视力,但视网膜异常可能持续存在。各种假说提出了感染、炎症、自身免疫甚至副肿瘤的机制,但明确的原因仍然难以捉摸。在这篇报告中,我们描述了一位被诊断为AEPVM的患者的临床过程和多模态影像学表现,有助于对这种疾病的进展和治疗的有限理解。病例介绍:我们报告一例40岁白人女性,视力模糊两周,双眼最佳矫正视力为20/20,前有流感样症状。初步的临床评估,包括眼底检查和光谱域光学相干断层扫描(SD-OCT),发现双侧有多个小浆液性视网膜脱离。多模式成像(眼底自身荧光、荧光素血管造影和吲哚菁绿血管造影)最初显示正常。全面的系统检查排除了自身免疫、感染和肿瘤病因。随访时,出现淡黄色视网膜病变和高自身荧光,诊断为特发性AEPVM。继续监测患者的视力恢复情况和潜在的并发症。结论:AEPVM是一种罕见的黄斑疾病,需要综合多模式评估才能准确诊断。排除自身免疫,感染性,特别是肿瘤原因,包括副肿瘤综合征,是确认其特发性的关键。虽然视力恢复是常见的,复发的风险和并发症,如脉络膜新生血管需要警惕的长期监测。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
International Medical Case Reports Journal
International Medical Case Reports Journal MEDICINE, GENERAL & INTERNAL-
CiteScore
1.40
自引率
0.00%
发文量
135
审稿时长
16 weeks
期刊介绍: International Medical Case Reports Journal is an international, peer-reviewed, open access, online journal publishing original case reports from all medical specialties. Submissions should not normally exceed 3,000 words or 4 published pages including figures, diagrams and references. As of 1st April 2019, the International Medical Case Reports Journal will no longer consider meta-analyses for publication.
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