Lens & vitreous humor

Persistent hyperplastic primary vitreous

Topic Completed: 1 February 2014

Minor changes: 28 December 2020

Copyright: 2004-2021, PathologyOutlines.com, Inc.

PubMed Search: Persistent hyperplastic primary vitreous [title]

Nat Pernick, M.D.
Page views in 2020: 278
Page views in 2021 to date: 340
Cite this page: Pernick N. Persistent hyperplastic primary vitreous. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyelenspersistenthyperplasticprimaryvitreous.html. Accessed December 6th, 2021.
Definition / general
  • Congenital, due to incomplete regression of fetal vasculature of vitreous humor, producing a retrolental mass
  • Usually unilateral
  • Associated with small eye, white reflex behind pupil (leukokoria), fibrous tissue behind lens, often cataract
  • Retina normal or with retinal dysplasia
Radiology images

AFIP images
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CT scan: microphthalmic eye
with total retinal detachment

Gross images

AFIP images
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Mild microphthalmos with
persistent hyaloid artery
extending from optic nerve head
to white retrolental mass

Clinical images

AFIP images
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Wrinkling and rupture of posterior
lens capsule caused by
hemorrhagic retrolental mass

Microscopic (histologic) description
  • Dense fibrovascular retrolental mass containing elongated ciliary processes and part of hyaloid vascular system
Whole mount images

AFIP images
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Falciform fold of detached
dysplastic retina circles the
persistent hyaloids artery,
which extends from optic nerve
head to retrolental mass

Microscopic (histologic) images

AFIP images
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Retrolental mass with rupture of
posterior lens capsule and anterior
insertion of the retina; epithelium
of pars plana of ciliary body is absent

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Retrolental fibrovascular mass has
cataractous changes and
elongation of ciliary processes

Differential diagnosis
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