Bilateral persistent hyperplastic primary vitreous

We report a case of bilateral persistent hyperplastic primary vitreous (PHPV) in a 5-month-old infant who presented with bilateral leukokoria. The child was referred for ocular ultrasound with a clinical suspicion of retinoblastoma. Grey-scale evaluation revealed an echogenic band in the posterior segment of the left globe extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of blood flow in the band. Ultrasound assessment of the contralateral globe showed an elevated mass of echogenic tissue in the posterior segment, in contact with the optic disc. Most cases of PHPV are sporadic and unilateral. Bilateral PHPV is rare. In a study by Pollard of 83 cases, only two patients (2.4%) had bilateral PHPV. The imaging features in this case point toward the diagnosis of bilateral PHPV. We suggest that this entity, although rare, should be considered in the differential diagnosis while evaluating bilateral leukokoria.