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Persistent Hyperplastic Primary Vitreous (PHPV)

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Pediatric Retina

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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].

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Authors and Affiliations

  1. University of Illinois at Chicago, UIC Eye Center, 1905 West Taylor Avenue, Chicago, IL, 60612, USA

    Bruce M. Buerk, Mithlesh C. Sharma & Michael J. Shapiro

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Correspondence to Michael J. Shapiro .

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Editors and Affiliations

  1. Ross Eye Inst., State University of New York, Buffalo, Main Street 1176, Buffalo, 14209, New York, USA

    James Reynolds

  2. Children's Mercy Hospital, Gillham St. 2401, Kansas City, 64108, Missouri, USA

    Scott Olitsky

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Buerk, B.M., Sharma, M.C., Shapiro, M.J. (2011). Persistent Hyperplastic Primary Vitreous (PHPV). In: Reynolds, J., Olitsky, S. (eds) Pediatric Retina. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-12041-1_8

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  • DOI: https://doi.org/10.1007/978-3-642-12041-1_8

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