The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex

To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A).

Retrospective cohort study.

Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups.

Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti-vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC.

PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization.