Nonexudative Perifoveal Vascular Anomalous Complex: The Subclinical Stage of Perifoveal Exudative Vascular Anomalous Complex?

Am J Ophthalmol. 2020 Oct:218:59-67. doi: 10.1016/j.ajo.2020.04.025. Epub 2020 Apr 28.

Abstract

Purpose: To describe the pre-exudative stage of exudative perifoveal vascular anomalous complex (ePVAC), referred to as nonexudative PVAC (nePVAC).

Design: Retrospective noncomparative case series.

Methods: Patients diagnosed with nePVAC were identified at 4 retina referral centers worldwide. Multimodal retinal imaging, including structural optical coherence tomography (OCT) and OCT-angiography (OCT-A), were performed at baseline and follow-up visits.

Results: Six eyes (6 patients, mean 75 ± 10 years of age) were included. Unrelated chorioretinal diseases were diagnosed in the affected eyes in 5 of 6 cases. At baseline, nePVAC is characterized by microvascular abnormalities featuring an isolated, perifoveal, large intraretinal aneurysm surrounded by capillary rarefaction at OCT-A examination, without any sign of exudation with structural OCT, and without visual impairment. Four patients were followed for a mean of 21 ± 14 months. During the follow-up, 3 of 4 eyes (75%) developed signs of exudation after a mean of 15 ± 9 months, associated with metamorphopsia and visual decline at the time of exudation. Best-corrected visual acuity decreased from 20/25 to 20/40 Snellen equivalent (P = .035) and central macular thickness increased from 268 ± 27 μm to 339 ± 65 μm (P = .145). Three patients were treated with 2.3 ± 0.6 intravitreal injections of anti-vascular endothelial growth factor without significant improvement of best-corrected visual acuity or macular edema.

Conclusions: nePVAC may represent the subclinical pre-exudative stage of ePVAC, notable for an absence of exudation or visual impairment. nePVAC and ePVAC should be considered as part of the same spectrum, namely PVAC. Typically, nePVAC develops signs of exudation over time, causing metamorphopsia and visual decline and therefore these lesions warrant continued close monitoring with multimodal retinal imaging.

Publication types

  • Multicenter Study

MeSH terms

  • Aged
  • Aged, 80 and over
  • Angiogenesis Inhibitors / therapeutic use
  • Exudates and Transudates
  • Female
  • Fluorescein Angiography
  • Fovea Centralis / blood supply*
  • Humans
  • Intravitreal Injections
  • Macular Degeneration / diagnosis*
  • Macular Degeneration / drug therapy
  • Macular Degeneration / physiopathology
  • Macular Edema / diagnosis*
  • Macular Edema / drug therapy
  • Macular Edema / physiopathology
  • Male
  • Middle Aged
  • Multimodal Imaging
  • Retinal Arterial Macroaneurysm / diagnosis*
  • Retinal Arterial Macroaneurysm / drug therapy
  • Retinal Arterial Macroaneurysm / physiopathology
  • Retrospective Studies
  • Tomography, Optical Coherence
  • Vascular Endothelial Growth Factor A / antagonists & inhibitors
  • Vascular Malformations / diagnosis*
  • Vascular Malformations / drug therapy
  • Vascular Malformations / physiopathology
  • Visual Acuity / physiology

Substances

  • Angiogenesis Inhibitors
  • VEGFA protein, human
  • Vascular Endothelial Growth Factor A