Persistent primitive vitreous hyperplasia

Persistent hyperplastic primary vitreous (PHPV) is a congenital anomaly that occurs at birth and is the result of the original vitreous not degenerating and proliferating behind the lens. Often monocular, full-term babies are found at birth. Small eyeballs are associated with white pupil due to fibrous blood vessel mass behind the lens. Although the back of the lens may be cloudy to varying degrees, the lens is generally transparent. If left untreated, most eyes with PHPV develop spontaneous intraocular hemorrhage, corneal opacities, glaucoma, retinal detachment, or eye atrophy. In the past, many eyes were removed due to glaucoma and eyeballs.

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