Astakhov Y S, Astakhov S Y, Lisochkina A B, Nechiporenko P A
Pavlov First Saint Petersburg State Medical University, St Petersburg, Russia.
Pavlov First Saint Petersburg State Medical University, St Petersburg, Russia.
J Fr Ophtalmol. 2020 Jun;43(6):500-516. doi: 10.1016/j.jfo.2020.01.004. Epub 2020 Mar 5.
To describe and analyze clinical findings in a patient with recurrent idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM), followed in detail, and to propose the diagnostic and follow-up algorithm.
Retrospective observational analysis.
A young adult male patient diagnosed with idiopathic AEPVM who developed two relapses in a 12-month period eight years after the initial onset.
Review of clinical charts, multimodal imaging, and electrophysiology findings. The patient repeatedly underwent complete ophthalmic examinations, including best-corrected visual acuity testing (BCVA), slit-lamp and fundus examinations; digital fundus photography, time-domain optical coherence tomography (OCT) in 2009 (Stratus OCT, Carl Zeiss Meditec, USA) and spectral-domain OCT in 2017-2018 (Spectralis-OCT, Heidelberg Engineering, Germany), together with fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICGA), all with HRA2 (Heidelberg Engineering, Germany); microperimetry (MP-1 microperimeter, Nidek, Japan). Laser flare photometry (Kowa FM-600, Japan) and electrophysiology testing were also performed.
Clinical features of long-lasting recurrent idiopathic AEPVM, and diagnostic and follow-up algorithm in such rare cases.
Case report of a 25-year-old male Caucasian patient with typical features of AEPVM, including serous neuroepithelial detachment with irregular retinal elevations, ophthalmoscopically resembling retinal folds, with subsequent subretinal accumulation of characteristic yellow-white vitelliform deposits. Features in this case rarely described, or even not yet reported, include indocyanine- and fluorescein-negative intraretinal cystic changes, optic disc hyperfluorescence on FA, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and very slow anatomical improvement of the relapses. Bimonthly fundus autofluorescence evaluation together with SD-OCT were the most informative diagnostic methods, demonstrating the evolution of pathological signs.
AEPVM may be a recurrent or even chronic condition with uncertain long-term visual outcomes. It may have variable clinical presentations depending on the stage of the disease, and both clinical manifestations and imaging features of different stages of the pathologic process may overlap. Patients should be made aware that visual improvement occurs very slowly, if at all. Bimonthly fundus autofluorescence evaluation together with SD-OCT should be recommended in such cases.
描述并分析一名复发性特发性急性渗出性多形性卵黄样黄斑病变（AEPVM）患者的详细临床发现，并提出诊断及随访方案。
回顾性观察分析。
一名年轻成年男性患者，被诊断为特发性AEPVM，在首次发病8年后的12个月内复发了两次。
回顾临床病历、多模态影像学检查及电生理检查结果。患者多次接受全面眼科检查，包括最佳矫正视力测试（BCVA）、裂隙灯及眼底检查；数码眼底照相、2009年的时域光学相干断层扫描（OCT）（美国卡尔蔡司医疗科技公司的Stratus OCT）以及2017 - 2018年的频域OCT（德国海德堡工程公司的Spectralis - OCT），同时进行眼底自发荧光（FAF）、荧光素血管造影（FA）和吲哚菁绿血管造影（ICGA），均使用德国海德堡工程公司的HRA2；微视野检查（日本尼德克公司的MP - 1微视野计）。还进行了激光散射光度测定（日本拓普康公司的Kowa FM - 600）和电生理测试。
持续性复发性特发性AEPVM的临床特征，以及此类罕见病例的诊断和随访方案。
一名25岁白种男性患者的病例报告，具有AEPVM的典型特征，包括浆液性神经上皮脱离伴视网膜不规则隆起，眼底镜下类似视网膜皱褶，随后出现特征性黄白色卵黄样沉积物的视网膜下积聚。该病例中很少被描述甚至尚未报道的特征包括吲哚菁绿和荧光素阴性的视网膜内囊性改变、FA上视盘高荧光、类似视网膜皱褶的浆液性视网膜隆起、脉络膜厚度增加、视力恢复缓慢以及复发后的解剖学改善非常缓慢。每两个月进行一次眼底自发荧光评估并结合频域OCT是最具诊断价值的方法，可显示病理体征的演变。
AEPVM可能是一种复发性甚至慢性疾病，长期视力预后不确定。其临床表现可能因疾病阶段而异，病理过程不同阶段的临床表现和影像学特征可能重叠。应告知患者视力改善非常缓慢，甚至可能无法改善。对于此类病例，建议每两个月进行一次眼底自发荧光评估并结合频域OCT。
