Persistent hyperplastic primary vitreous
Encyclopedia
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. 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.
2. Norries disease
3. Walker Warburg syndrome
following failure of the embryological, primary vitreous and hyaloid vasculature to regress. It can be present in three forms: purely anterior (persistent tunica vasculosa lentis
Persistent tunica vasculosa lentis
Persistent tunica vasculosa lentis is a congenital ocular anomaly. It is a form of persistent hyperplastic primary vitreous .* It is a developmental disorder of the vitreous.* Usually unilateral and first noticed in the neonatal period....
and persistent posterior fetal fibrovascular sheath of the lens), purely posterior (falciform retinal septum and ablatio falcicormis congentia) and a combination of both.
Causes/association of bilateral PHPV
1. Trisomy 13 (Patau's syndrome)2. Norries disease
3. Walker Warburg syndrome
Symptoms
The primary vitreous used in formation of the eye during fetal development remains in the eye upon birth and is hazy and scarred.External links
- GeneReviews/NIH/NCBI/UW entry on NDP-Related Retinopathies
- Persistent Hyperplastic Primary Vitreous Involving the Anterior Eye report by Mauricio Castillo, David K. Wallace, and Suresh K. Mukherji