Persistent hyperplastic primary vitreous.

Case report of a pars plana surgery for persistent hyperplastic primary vitreous (PHPV) with a clear lens. This situation enabled us to show the important features of PHPV: the initial small but clear lens, the fibrovascular membrane giving rise to the leukokoria, the elongated processi ciliares visible in the pupil and a patent A. hyaloidea, feeding the retrolental fibrovascular membrane. The surgery includes removal of the lens, the fibrovascular membrane and the endocoagulation of the feeding vessel in order to interrupt the natural cause of PHPV leading to blindness. After the surgery the baby is adapted with a soft contact lens and an occlusion treatment is started.