n SA Journal of Radiology - Persistent hyperplastic primary vitreous - the martini glass sign : signs

Volume 18, Issue 1
  • ISSN : 1027-202X
  • E-ISSN: 2078-6778



Persistent hyperplastic primary vitreous (PHPV) is a congenital lesion due to incomplete regression of the embryonic ocular blood supply (hyaloid vasculature). It represents 28% of childhood presentations of leukocoria and is almost always accompanied by poor vision, micropthalmia and often retinal detachment. The absence of ocular calcifications helps distinguish PHPV from the more common retinoblastoma.

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