Sunday, October 25, 2009

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.

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