Purpose: to report a case of exudative perifoveal vascular anomalous complex (ePVAC) in a patient with adult-onset foveomacular vitelliform dystrophy. Observations: A 71-year-old male presented with moderate vision loss in his left eye. His past medical and ocular history were unremarkable. The best-corrected visual acuity was 20/32. Fundus examination disclosed the presence of a perifoveal vascular abnormality, associated with a small egg-yolk lesion. Optical coherence tomography (OCT) scan passing through the perifoveal vascular abnormality seen on fundus, revealed a small round hyperreflective lesion located at the outer plexiform layer-inner nuclear layer, surrounded by intraretinal cysts. Fluorescein angiography displayed an isolated perifoveal aneurysmal lesion with minimal leakage. OCT angiography permitted to localize the lesion flow signal at the deep capillary plexus (DCP) and the avascular slab; choriocapillaris structure was unaffected; any retino-choroidal anastomosis was detected. A systemic workup was suggested to rule out any vascular diseases, and any abnormality was found. He was thus diagnosed with ePVAC. A stability of the clinical picture was demonstrated after 3 months of follow up. Conclusion: ePVAC has recently been described to appear in association with other macular abnormalities, such as age-related macular degeneration and myopic macular degeneration. Our case firstly demonstrates an ePVAC lesion in an eye with adult-onset foveomacular vitelliform dystrophy. This observation highlights the importance of discerning between different vascular disorders of the macula, to offer the right treatment to the patient.
