Persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous
Falciform fold of detached dysplastic retina encircles the persistent hyaloid artery that extends from the optic nerve head to the retrolental mass.
Classification and external resources
Specialty
ICD-10	Q14.0
ICD-9-CM	xxx
Patient UK	Persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (PHPV), also known as Persistent Fetal Vasculature (PFV), 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

1. Trisomy 13 (Patau syndrome)
2. Norrie disease
3. Walker-Warburg syndrome
4. Autosomal dominant
5. Autosomal recessive

Symptoms

The primary vitreous used in formation of the eye during fetal development remains in the eye upon birth and is hazy and scarred.

See also

• Persistent tunica vasculosa lentis

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