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Bilateral persistent hyperplastic primary vitreous

Tarun P Jain1

  • 1Department of Radiology, Getwell Polyclinic and Hospital, Jaipur - 302 004, India. drtjain@yahoo.com

Indian Journal of Ophthalmology
|December 17, 2008
PubMed

Insights

Bilateral persistent hyperplastic primary vitreous (PHPV) is a rare condition presenting as leukokoria in infants. Imaging findings can help differentiate bilateral PHPV from retinoblastoma, aiding diagnosis.

Area of Science:

  • Ophthalmology
  • Pediatric Imaging
  • Medical Diagnostics

Background:

  • Persistent hyperplastic primary vitreous (PHPV) is a congenital developmental anomaly of the eye.
  • While typically unilateral and sporadic, bilateral PHPV presents a diagnostic challenge, often mimicking retinoblastoma.
  • Leukokoria, or a white pupillary reflex, is a key clinical sign necessitating prompt investigation.

Observation:

  • A 3-month-old male infant presented with bilateral leukokoria.
  • Imaging was performed due to clinical suspicion of retinoblastoma.
  • Gray-scale ultrasound revealed echogenic bands from the lens to the optic disc in both eyes.

Findings:

  • Doppler examination confirmed arterial flow within the echogenic bands in both globes.
  • Computed tomography confirmed associated echogenic hemorrhage.
  • The imaging features were consistent with bilateral PHPV, distinguishing it from retinoblastoma.

Implications:

  • This case highlights the importance of considering bilateral PHPV in the differential diagnosis of bilateral leukokoria.
  • Advanced imaging techniques, including ultrasound and CT, are crucial for accurate diagnosis.
  • Early and correct diagnosis of bilateral PHPV is essential for appropriate management and to avoid unnecessary treatment for retinoblastoma.

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