Atypical perifoveal exudative vascular anomalous complex (PEVAC) with multifocal and bilateral presentation

PURPOSE: To report an unusual case of multifocal and bilateral perifoveal exudative vascular anomalous complex (PEVAC) lesions in an otherwise healthy patient. OBSERVATIONS: A 62-year-old male with complaints of long-term progressive blurred vision in both eyes. There was no history of diabetes, arterial hypertension or inflammatory disease. Best-corrected visual acuity was 75 and 80 letters in his right eye (OD) and left eye (OS) respectively. Fundus examination showed three perifoveal isolated large aneurysmal lesions in his OD and an additional one in his OS, associated with smaller microaneurysms, haemorrhages and intraretinal exudation. On optical coherence tomography PEVAC appeared as a round lesions with a hyperreflective wall and lumen containing variably reflective material, typically surrounded by intraretinal fluid. Dye-based angiography demonstrated three and one well-defined large hyperfluorescent aneurysmal lesions in his OD and OS respectively, with no leakage. The optical coherence tomography angiography images showed flow signal within the intraretinal aneurysmal lesions. No anomalous flow signal in the outer retina and choriocapillaris was present. After one bilateral anti-vascular endothelial growth factor intravitreal injection no anatomical or functional improvement was observed, and no changes in the lesions or visual acuity were observed at 6 months follow-up. CONCLUSIONS AND IMPORTANCE: We describe an atypical case of PEVAC, an uncommon and poorly known macular disorder, with bilateral presentation and multifocal lesions in one eye. To our knowledge, this is the first reported case of bilateral PEVAC lesions. Hence, PEVAC should be included in the differential diagnosis of bilateral perifoveal aneurysmal lesions with macular edema.