Persistent hyperplastic primary vitreous
|Persistent hyperplastic primary vitreous|
|Classification and external resources|
Falciform fold of detached dysplastic retina encircles the persistent hyaloid artery that extends from the optic nerve head to the retrolental mass.
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 congenita) and a combination of both. Most examples of PHPV are unilateral and non-hereditary. When bilateral, PHPV may follow an autosomal recessive or autosomal dominant inheritance pattern.
Causes/association of bilateral PHPV
- Trisomy 13 (Patau syndrome)
- Norrie disease
- Walker-Warburg syndrome
- Autosomal dominant
- Autosomal recessive
The primary vitreous used in formation of the eye during fetal development remains in the eye upon birth and is hazy and scarred.
- Silbert, Mira; Gunvood, Andrew S. (2000). "Clinical review, Persistent hyperplastic primary vitreous". Clinical Eye and Vision Care (Ireland: Elsevier Science Ireland Ltd.) 12 (3-4): 131–137. doi:10.1016/S0953-4431(00)00054-0. PMID 11137427. Retrieved 2009-05-11.
- Young, Jane. "Persistent Hyperplastic Primary Vitreous". Retrieved 2009-05-11.
- 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
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