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Persistent hyperplastic primary vitreous (PHPV) is a rare congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress.
The primary vitreous used in formation of the eye during fetal development remains in the eye upon birth and is hazy and scarred.
It can be present in three forms: purely anterior (persistent tunica vasculosa lentis) and persistent posterior fetal fibrovascular sheath of the lens), purely posterior (falciform retinal septum and ablatio falcicormis congentia) and a combination of both.
Associated conditions:
1. Trisomy 13 (Patau's syndrome) 2. Norries disease 3. Walker Warburg syndrome.
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