Persistent Fetal Vasculature
Persistent fetal vasculature (PFV), also known as persistent hyperplastic primary vitreous (PHPV), is a congenital, nonhereditary ocular malformation that frequently involves the lens (Fig 4-10). In 90% of patients, it is unilateral. A white, fibrous retrolental tissue is present, often in association with posterior cortical opacification. Progressive cataract formation often occurs, sometimes leading to a complete cataract. Other abnormalities associated with PFV include elongated ciliary processes, prominent radial iris vessels, and persistent hyaloid artery. (See BCSC Section 6, Pediatric Ophthalmology and Strabismus, and Section 12, Retina and Vitreous, for additional discussion.)
Figure 4-9 Cataract in a patient with aniridia.
Figure 4-10 Persistent fetal vasculature (PFV). A, Mild variant with central retrolental membrane. B, Elongated ciliary processes are adherent to the lens. Note the dense fibrous plaque on the posterior lens capsule. C, Ultrasonogram of an eye with PFV. Note the dense stalk arising from the optic nerve and attaching to the posterior lens.
(Part A courtesy of David A. Plager, MD; part C courtesy of Edward L. Raab, MD.)
Excerpted from BCSC 2020-2021 series: Section 11 - Lens and Cataract. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.