[Persistence and hyperplasia of primary vitreous]

The persistence of the fetal vascular system is a rare ocular malformation whose origin remains unknown. It comprises a group of complex ocular malformations from which the various clinical forms have a heterogeneous functional prognosis. The diagnosis depends on a precise and complete examination of the child's eye, often under general anesthesia. Various clinical forms can be found together, ranging from the tiny form with no visual repercussion or ocular or systemic anomaly associated with the major forms involving the totality of the ocular structures and systemic syndromes. A systematic general pediatric examination is therefore recommended. The treatment is adapted individually for early and specific surgical and medical treatment. In the event of intervention, the induced aphakia will be corrected in one procedure with an implantation, generally sutured with the sclera, or at the time of a second intervention after the wearing of glasses or a contact lens. However, the complications can sometimes lead to rapid phthisis bulbi. Whatever solution is chosen, the final functional result has progressed thanks to early diagnosis, the surgical techniques suggested, even if implantation continues to be debated. Amblyopia must be treated for the entire sensitive period. However, despite good rehabilitation, the average functional prognosis remains disappointing, probably because of the overall ocular malformation.