Vitreous Retina Macula Consultants of New York, New York, New York; The LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York.
Vitreous Retina Macula Consultants of New York, New York, New York; The LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York.
Ophthalmology. 2018 Jan;125(1):75-88. doi: 10.1016/j.ophtha.2017.07.020. Epub 2017 Aug 23.
To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM).
Retrospective, observational, multicenter case series review.
Consecutive patients diagnosed with idiopathic AEPVM.
Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature.
Clinical features of idiopathic AEPVM and differential diagnosis.
Eighteen patients (age range, 21-74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations.
Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis.
描述急性渗出性多形性玻璃膜疣（AEPVM）患者的临床发现。
回顾性、观察性、多中心病例系列回顾。
连续诊断为特发性 AEPVM 的患者。
对之前未发表的患者的临床图表、多模态成像、电生理发现和遗传发现进行回顾，并对文献进行回顾。
特发性 AEPVM 的临床特征和鉴别诊断。
18 名患者（年龄范围 21-74 岁）具有 AEPVM 的典型特征，包括最初的局部性浆液性脱离，随后在玻璃膜下空间出现典型的黄白色沉积物。随着时间的推移，这种高自发荧光物质在较大病变内下沉，导致后期典型的曲线状沉积物。症状和临床发现持续数周至数年。一些患者表现出以前未描述的特征，如荧光素阴性的视网膜内囊样改变、脉络膜新生血管、类似视网膜皱襞的浆液性视网膜抬高、脉络膜增厚、快速视力恢复缺失以及初始表现完全消退后数年的复发。
急性渗出性多形性玻璃膜疣的自然病程可能比以前描述的更为多变。副肿瘤性视网膜病变和常染色体隐性 Best 病与 AEPVM 非常相似，需要进行医学和遗传评估以排除这些临床可能性。本系列 AEPVM 患者扩展了该疾病的临床谱，包括人口统计学、临床表现、影像学特征、自然病程和视觉预后。
