Persistent Fetal Vasculature: Mechanisms of Development, Current Treatment Approaches, and Advancements in Therapy

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Persistent fetal vasculature (PFV) is a developmental disorder in which the fetal hyaloid vascular network fails to regress normally.
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PFV reflects disrupted coordination among HIF-VEGF angiogenic signaling, macrophage-mediated regression, Wnt pathways, and ocular growth.
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Mutations in NDP, FZD4, LRP5, TSPAN12, and ATOH7 are associated with PFV-like features or autosomal-recessive PFV.
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PFV is classified as anterior, posterior, or combined, and may include a retrolental plaque, a disc-to-lens stalk, retinal folds, or microphthalmia.
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Ultrasound with Doppler, wide-field fluorescein angiography, OCT, OCT angiography, and intraoperative endoscopy help characterize PFV and inform surgical assessment.

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