Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy Clinical Spectrum and Multimodal Imaging Characteristics

Purpose: To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM). Design: Retrospective, observational, multicenter case series review. Participants: Consecutive patients diagnosed with idiopathic AEPVM. Methods: Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature. Main Outcome Measures: Clinical features of idiopathic AEPVM and differential diagnosis. Results: Eighteen patients (age range, 21e74 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. Conclusions: 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. (C) 2017 by the American Academy of Ophthalmology