Persistent hyperplastic primary vitreous (PHPV) results from the failure of the fetal hyaloid vascular system to regress during ocular development. Normally, this vascular system, which nourishes the developing lens, involutes before birth. When it persists, it causes a fibrovascular mass behind the lens, leading to leukocoria, microphthalmia, and potential retinal detachment. Recognition of this condition is critical as surgical intervention may be needed to prevent amblyopia and preserve vision. Early diagnosis with imaging and careful vitrectomy can improve outcomes.

Reference: Ophthalmology, Yanoff & Duker, 6th Edition.