Abstract
PURPOSE: To discuss the evolution in retinopathy of prematurity since its first description as retrolental fibroplasia in the United States, including the changes in the understanding of pathophysiology; methods of diagnosis; destructive, anti-vascular endothelial growth factor (anti-VEGF), and supportive treatments; and differences in retinopathy of prematurity manifestations worldwide. The overall goal is to clarify retinopathy of prematurity currently and formulate questions to optimize future care. STUDY DESIGN: Literature review and synthesis. METHODS: Critical review and consideration of the literature with inclusion of historical articles and those regarding pathophysiologic risk factors, retinopathy of prematurity worldwide, basic and clinical science particularly regarding anti-VEGF mechanisms and agents tested in clinical trials. RESULTS: Retinopathy of prematurity has evolved from affecting infants approximately 2 months premature to affecting extremely premature infants. Worldwide, retinopathy of prematurity differs and, in emerging countries, has features similar to that experienced in the United States when retinopathy of prematurity first manifested. Treatments have evolved from destruction of the peripheral avascular retina to inhibit angiogenic stimuli to anti-VEGF agents, which inhibit pathologic angiogenesis but also extend normal intraretinal angiogenesis by ordering the development of intraretinal vessels. Clinical trial evidence is accruing with the goal to develop less destructive treatments to optimize vision and that are protective to the retina and infant. CONCLUSIONS: Goals for retinopathy of prematurity are to optimize prenatal and perinatal care, improve diagnostic acumen worldwide and refine treatment strategies, including with anti-VEGF agents, to inhibit intravitreal angiogenesis and facilitate vascularization of the previously avascular retina, which include supporting neural and vascular development of the premature infant and retina.