Persistent Hyperplastic Primary Vitreous

Persistent hyperplastic primary vitreous (PHPV) is a congenital condition caused by incomplete regression of the embryonic ocular blood supply. On physical examination, there is Leukocoria (white retinal light reflex)

Anteriorly, we see soft tissue behind the lens that corresponds to the persistent hyperplastic primary vitreous. Posteriorly, there is a stalk of hyaloid remnant that extends from this structure to the retina.

PHPV may present as an isolated anterior form (best prognosis for preserved vision), isolated posterior form, or combined form (most common). Unilateral involvement is three times more common than bilateral involvement.

On cross-sectional imaging, there is a hyperattenuating/hyperintense small globe with soft tissue behind the lens. No calcification is seen.

Differential considerations for leukocoria with microphthalmia include PHPV (if unilateral) and retinopathy of prematurity and PHPV (if bilateral). For completeness, leukocoria in a normal-sized eye can be seen with calcifications in retinoblastoma and retinal astrocytoma and without calcifications in toxocaral endophthalmitis, and Coats disease.