Current insights and challenges in the management of perifoveal vascular anomalous complex (PVAC): a systematic review of multimodal imaging features and treatment strategies.

The aim of this article is to comprehensively review the multimodal imaging (MMI) features that define perifoveal vascular anomalous complex (PVAC) and to update the optimal treatment strategies for this disorder with a focus also on the underlying pathogenetic mechanisms. This systematic review was performed based on a search of the PubMed and Embase databases of relevant papers on the subject of PVAC. PVAC is characterized by well-defined MMI findings, including remarkable morphological features with optical coherence tomography (OCT) and OCT angiography (OCTA). The recognition of this lesion is important because of treatment implications as this entity is typically unresponsive to anti-vascular endothelium growth factor (VEGF) therapy. Thermal focal laser (TFL) can have better success in reducing exudation associated with PVAC. Other therapeutic modalities include subthreshold micropulse laser (SML), steroid and anti-inflammatory treatments. In total, this review captured 25 scientific articles covering the treatment of exudative PVAC cases. Accurate multimodal imaging characterization of PVAC is essential for differential diagnosis and management. There are a multitude of similar lesions described in the literature with overlapping MMI features and clinical contexts. These various lesions will be described and defined in an effort to provide clarity of differentiation. We recommend the "PVAC" nomenclature to denote a distinct, primary condition, consistent with its original definition. On the other hand, we support the consolidation of "TelCap" lexicon to characterize the secondary lesion occurring in the context of diabetic retinopathy (DR), retinal vein occlusion (RVO), and other causal retinal vascular disorders.