Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy: A five-year follow-up

Purpose: The pathophysiology of idiopathic acute exudative polymorphic vitelliform maculopathy (AEPVM) is not fully understood. It is a rare disorder and very few cases are reported with long-term follow-up. Here we report a case of AEPVM followed-up over 5 years. Methods: Review of medical chart, electrophysiologic findings and multimodal imaging including spectral-domain optical coherence tomography (OCT) (Spectralis OCT, Heidelberg Engineering, Germany), fundus autofluorescence (FAF), fluorescein angiography (FA) and indocyanine green angiography (ICGA). Patients: A 34-year-old patient. Results: Initial visual acuity (VA) was 20/20 in both eyes. Fundus exam revealed bilateral symmetrical multifocal serous retinal detachments and vitelliform lesions affecting the fovea. Complete systemic work-up ruled out neoplastic disorders. A decreased Arden ratio on EOG was present. Genetic testing for Best disease was negative. The long-term follow-up revealed improvement but incomplete resolution of vitelliform lesions. The patient’s final VA was 20/20 in both eyes. Conclusion: We describe a case of AEPVM which maintained good central VA despite long-term persistence of central vitelliform lesions.