Bilateral Persistent Hyperplastic Primary Vitreous: A Rare Entity

Persistent hyperplastic primary vitreous (PHPV) is a rare congenital, nonhereditary malformation of the eye which results from persistence of the foetal fibrovascular primitive hyaloid system which should normally regress otherwise. Most commonly seen is unilateral and sporadic while bilateral is rare. We report a case of bilateral PHPV in a 10 year old male patient who presented with bilateral leukocoria and dimness of vision along with microphthalmos. Child was referred for ultrasound, CT and MRI imaging with a clinical suspicion of retinoblastoma which is a close differential of PHPV. The imaging findings are as described in the article which confirm the diagnosis of bilateral PHPV.