Retinopathy of Prematurity: The Life of a Lifetime Disease

To provide information on retrolental fibroplasias (RLF), later known as retinopathy of prematurity. Review of the literature on the subject and a first-person account of what was then RLF by one of the authors (A.P.) who was involved in the earliest days in research regarding RLF. MEDLINE search on the topics of RLF and retinopathy of prematurity plus a first-person historic review of original work that dealt with RLF. In 1942, elevated levels of oxygen were thought to play a major role in the development of the disease; at that time, no treatment was available. During the lifetime of this disease, other possible causes have been investigated. These include vitamin E as a prophylaxis against retinopathy of prematurity and the efficacy of light reduction to prevent retinopathy of prematurity. It has been shown that the light reduction does not play a role in reducing the progression of retinopathy of prematurity. Vitamin E studies were inconclusive; some studies show a positive effect and others do not. A major advance occurred with the development of the International Classification of Ophthalmology in 1984, which laid the groundwork for collaborative studies to determine whether cryotherapy of the avascular zone of retina would reduce the incidence of blindness in newborn infants, when compared with control subjects. The study showed that cryotherapy was effective; this was followed by laser photocoagulation when lasers became portable enough to take to the neonatal intensive care unit. At the same time, improved surgical techniques moved from scleral buckling for retinal detachment to vitrectomies (some lens sparing) for more desperate cases that had progressed to stage 4 and stage 5 retinopathy of prematurity. Late changes in adults who were born before any treatment and are now baby boomers ran the gamut from the dragging of the retina in the posterior pole to retinal detachment, cataract, and myopia. Retinopathy of prematurity is a lifetime disease for which preventive and better treatment modalities continue to evolve.