Persistent hyperplastic primary vitreous (PHPV)

Abstract

Persistent fetal vasculature (PFV), also known as persistent hyperplastic primary vitreous (PHPV) is a constellation of ophthalmic findings typically consisting of microphthalmia, cataract (Figs. 8.1 and 8.2), centrally dragged ciliary processes, retrolental fibrous tissue, and a persistent hyaloid artery. It has long been recognized in the differential diagnosis of leukokoria or amaurotic pupillary reflex. The full spectrum of findings in PFV was not fully appreciated until 1949 when Reese described the syndrome in more detail, differentiating PFV from retrolental fibroplasia [1]. In the 1956 Jackson Memorial lecture, Reese further elucidated the findings of this disorder [2]. He termed the anterior findings anterior hyperplastic vitreous. Michaelson, and later Pruett and Schepens, termed the posterior component posterior hyperplastic primary vitreous and stressed the anterior and posterior findings could overlap [3]. Goldberg suggested replacing the term PHPV with the more comprehensive PFV, which emphasizes the etiology of the disorder [4]. © 2011 Springer-Verlag Berlin Heidelberg.