Bilateral Persistent Hyperplastic Primary Vitreous: A Close Mimic of Retinoblastoma

Abstract

Bilateral persistent hyperplastic primary vitreous (PHPV) is a rare disorder of eye. It is one of the most important differential diagnoses of retinoblastoma, hence early and accurate diagnosis is important. We here report a case of an 11-month-old child which was referred to ocular OPD with complaints of bilateral leukocoria. Examination revealed greyish-white masses posterior to both lenses, raising the clinical suspicion of retinoblastoma. Ultrasonography demonstrated echogenic masses extending from the posterior surface of the lens to the optic disc with reduced axial lengths. These masses demonstrated flow on color Doppler evaluation. CT scan revealed hyperdense masses behind the lens without any evidence of intralesional calcification. Clinical features and imaging findings point towards the diagnosis of bilateral PHPV. PHPV is a developmental disorder of the globe in which the hyaloid vasculature fails to regress normally. While unilateral PHPV is common, bilateral PHPV is a rare entity. It is one of the most important conditions mimicking retinoblastoma; hence early and accurate diagnosis is required. Ophthalmological examination is still the best way to confirm the diagnosis. However, if the diagnosis remain unclear, further evaluation using ultrasonography, Color Doppler, and CT scan is useful.