Persistent Fetal Vasculature
Persistent fetal vasculature (PFV; previously known as persistent hyperplastic primary vitreous) is characterized by the persistence of variable components of the primary vitreous and is most often unilateral. In most cases of clinically significant PFV, a fibrovascular plaque in the retrolental space extends laterally to involve the ciliary processes, which may be pulled toward the visual axis by traction from the fibrovascular tissue. The clinical and gross appearance of elongated ciliary processes results. The anterior fibrovascular plaque is generally contiguous posteriorly with a remnant of the hyaloid artery that may attach to the optic nerve head (optic disc) (Fig 10-2). Involvement of the posterior structures may be more extensive, with detachment of the peripapillary retina resulting from traction caused by preretinal membranes. The lens is often cataractous, and the posterior capsule may be discontinuous. Eyes affected by the more severe forms of PFV are often microphthalmic. See also Chapter 19 in this volume, BCSC Section 6, Pediatric Ophthalmology and Strabismus, and Section 12, Retina and Vitreous.
Figure 10-2 Persistent fetal vasculature. The photomicrograph shows a prominent anterior fibrovascular plaque adherent to the lens (asterisk). The lens is distorted, and the peripheral retina is adherent to the plaque. The persistent remnant of the hyaloid artery is seen as a stalk traversing the vitreous cavity and attaching to the optic nerve head posteriorly (arrow).
(Courtesy of Nasreen A. Syed, MD.)
Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.