41 - Persistent Hyperplastic Primary Vitreous

Ha, the beauty of a picture, amazing!

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.

37 - Persistent Hyperplastic Primary Vitreous (PHPV)

* It is persistence of the primary vitreous and hyaloid arterial system.

* Almost always unilateral (~90%)

*It is associated with Patau syndrome (Trisomy 13) .

* Presents as white pupillary reflex in the newborn (congenital leukocoria)

*It is usually associated with cataract, glaucoma , long and extended ciliary processes because of pull from contracted retrolental tissue, micropthalmos, intraocular hemorrhage and tractional retinal detachment.

* Investigations : USG and CT scan

* Differential diagnosis with retinoblastoma is done on the basis of micropthalmos and elongated ciliary processes.

* If diagnosed early, treatment is aspiration of lens, excision of retrolental membrane and vitrectomy.

* Visual prognosis is poor.