Retinopathy of prematurity (ROP) is a complex disease process initiated in part by a lack of complete or normal retinal vascularization in premature infants. ROP has a typical progression pattern, but the earlier disease stages may regress spontaneously at any time. The absence of retinal vessels in portions of the immature retina can result in retinal ischemia, leading to the release of growth factors that promote vascular growth. The normal vascular-growth process is disturbed, and vessels proliferate into the vitreous cavity at the border of the vascular and avascular retina. As the disease progresses, vitreous hemorrhage and tractional retinal detachment can occur. The end stage of untreated ROP is the development of a dense, white, fibrovascular plaque behind the lens and complete tractional retinal detachment. The former name of this condition, retrolental fibroplasia, describes the end stage of ROP. The main risk factors for developing this condition are prematurity and low birth weight. Also see BCSC Section 6, Pediatric Ophthalmology and Strabismus, for additional discussion of ROP.
In the United States, ROP that is severe enough to require treatment occurs in approximately 1100–1500 infants annually. Among these infants, 400–600 will never achieve vision better than 20/200. In resource-limited regions of the world, there has been a rise in the incidence of ROP that corresponds to the establishment of neonatal intervention initiatives to treat premature infants who would not previously have survived. In many instances, there has been an unfortunate lag between successes in saving premature infants and successes in diagnosing and managing their subsequent ROP.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.