Persistent hyperplastic primary vitreous in an adult: Case report with fluorescein angiographic findings

Persistent hyperplastic primary vitreous (PHPV) is one of the most serious lesions induced by abnormal development of the embryonic intraocular vasculature. It often progresses to secondary complications such as rupture of the lens capsule and cataract formation, intraocular hemorrhage, secondary glaucoma, tractional retinal folds (with occasional associated detachment), and eventual phtisis bulbi. There have been only a few patients with this condition whose eyes are known to have survived to adulthood. This report describes the clinical and fluorescein angiographic findings of PHPV in an 18-year-old patient. To the best of our knowledge, angiography of the fibrovascular retrolental mass has not been previously reported. The clinical course of this case is unusual in that no catastrophic complication had occurred and no therapeutic intervention was required. The lens resorbed spontaneously, thereby permitting study of the retrolental mass by fluorescein angiography. Fluorescein leakage from the persistent vessels was very slow but present, thus indicating a probable disruption of the endothelial tight junctions of the tunica vasculosa lentis.