Computed tomography in the evaluation of patients with persistent hyperplastic primary vitreous (PHPV)

Area of Science:

• Ophthalmology
• Radiology
• Pediatric Imaging

Background:

• Persistent hyperplastic primary vitreous (PHPV) is a congenital developmental anomaly.
• It results from the incomplete regression of the embryonic hyaloid vascular system.
• PHPV is a significant differential diagnosis for leukokoria, mimicking retinoblastoma.

Observation:

• This study describes computed tomographic (CT) findings in five patients diagnosed with PHPV.
• CT imaging provides valuable insights into the ocular and orbital structures affected by PHPV.

Findings:

• Key CT findings include vitreous soft-tissue infiltration and retrolental soft tissue along the Cloquet canal.
• Absence of intraorbital calcification, microphthalmos, retrohyaloid hemorrhage, and vitreous hypervascularity are also noted.
• These CT features aid in distinguishing PHPV from other intraocular pathologies.

Implications:

• Accurate CT interpretation is crucial for diagnosing PHPV, especially in cases presenting with leukokoria.
• Early and correct diagnosis facilitates timely management and can prevent unnecessary treatment for conditions like retinoblastoma.
• CT imaging plays a vital role in the diagnostic workup of pediatric leukocoria.