For some baby boomers, the fact that the condition which caused their blindness is still one of the leading causes of congenital blindness comes as a shock. For most of their lives, they have believed that the condition which caused their blindness was the result of receiving "too much oxygen" during the first few weeks of life and that since the cause was known the condition no longer occurred. In fact, during the 1950's and 1960's this theory was so widely accepted that if a child was found to have this condition, it was assumed that the child had been overexposed to oxygen.14
The condition was first discovered in 1941, when two Boston infants were found to have a membrane of scar tissue behind the lens. The condition was eventually named retrolental fibroplasia, or RLF.13 RLF was responsible for 10,000 cases of legal blindness during the 1940's and early 1950's. This figure does not include cases in which the condition regressed and did not result in legal blindness.
This "epidemic of blindness" was of great concern to doctors caring for premature infants. In the early 1950's, two studies were conducted to examine the relationship of oxygen exposure to the occurrence of RLF. The results indicated that lowering the amount of oxygen given to premature infants would result in a decrease in the occurrence of RLF.13
Several things happened which caused doctors, researchers, and even some patients to question the validity of these studies. New technology allowed for the monitoring of blood gas levels, and this allowed for more appropriate amounts of oxygen to be administered. New technology also allowed ophthalmologists to examine the infants' eyes while they were still in the neonatal intensive care unit (NICU). In the 1970's, the number of cases of RLF began to increase despite the restrictions in the use of oxygen. This prompted more investigations into possible causes of ROP.1,3,6,7,9,10,12,18 The name of the condition was eventually changed. The new name represented two things. The condition did not always progress to the point where the membrane was present. Furthermore, it did not always occur in infants who had received too much oxygen--in fact, a significant number of cases were reported in infants who had not received enough oxygen.1 It did occur almost exclusively in premature infants. Therefore, the new name for the condition is retinopathy of prematurity (ROP).
The body of literature about ROP which has become available since its discovery reveals a number of things which are important for adults who were born prematurely. Adults with mild ROP which regressed are at risk for certain complications which can cause vision loss.5 Some of these adults do not know they are experiencing complications of a condition they had during their infancy. Why were they not told? Perhaps they were not born at a place where interested ophthalmologists were watching premature babies' eyes for the early signs of ROP. Both near-sightedness and lazy eye are common in the population as a whole and might not have been attributed to premature birth. Because these adults were able to live fairly normal lives, there was no cause to be concerned with what might have happened during their infancy until they began to experience complications such as cataracts, glaucoma and retinal detachment.
The late onset complications of ROP are often treatable if detected early.8,11,15,16,17 Treatments can also help people with more severe ROP to retain some usable vision.2 Understanding the condition of one's own eyes is crucial in making an informed decision about whether or not to pursue an intervention. Regular visits to the ophthalmologist and good communication during those visits are the first steps toward that understanding.
1. Adamkin DH, Shott RJ, CookLN, Andrews BF. Nonhyperoxic retrolental fibroplasia. Pediatrics, 60:828-830. 1977.
2. Bradford JD, Trese MT Management of advanced retinopathy of prematurity in the older patient. Ophthalmology, 98:1105-1108. 1991.
3. Brooks SE, Marcus DM, Gillis D, Pirie E, Johnson CS, Bhatia J. The effect of blood transfusion protocol on retinopathy of prematurity: A prospective, randomized study. Pediatrics, 104:514-518. 1999.
4. Krolicki TJ, Tasman W. Cataract extraction in adults with retinopathy of prematurity. Arch ophthalmology, 113:173-177. 1995.
5. Kushner BJ. Long-term follow-up of regressed retinopathy of prematurity. Birth defects, 24:193-199. 1988.
6. Kushner BJ, Gloeckner E. Retrolental fibroplasia in full-term infants without exposure to supplemental oxygen. Am j ophthalmol 97:148-153. 1984.
7. Lucey JF, Dangman B. A reexamination of the role of oxygen in retrolental fibroplasia. Pediatrics, 73:82-96. 1984.
8. Michael AJ, Pesin SR, Katz J, Tasman WS. Management of late-onset angle-closure glaucoma associated with retinopathy of prematurity. Ophthalmology, 98:1093-1098. 1991.
9. National Eye Institute. Effects of Light Reduction on Retinopathy of Prematurity.
10. Phelps DL. Vitamin E: where do we stand? Pediatrics, 63(6), 933-935. 1979.
11. Richards SC. Late complications of ROP.
12. Richards SC. Risk factors for ROP.
13. Silverman WA The lesson of retrolental fibroplasia. Scientific American, 236:100-107. 1977.
14. Silverman WA Retrolental fibroplasia: A modern parable. New York: Grune and Stratton, 1980.
15. Smith J, Shivitz I. Angle-closure glaucoma in adults with cicatricial retinopathy of prematurity. Arch ophthalmol, 102:371-372. 1984.
16. Sneed SR, Pulido JS, Blodi CF, Clarkson JG, Flynn HW Jr, Mieler WF. Surgical management of late-onset retinal detachments associated with regressed retinopathy of prematurity. Ophthalmology, 97:179-183. 1990.
17. Tasman W. Late complications of retrolental fibroplasia. Ophthalmology, 86:1724-1740. 1979.
18. Valentine PH, Jackson JC, Kalina RE, Woodrum DE. Increased survival of low birth weight infants: impact on the incidence of retinopathy of prematurity. Pediatrics, 84:442-445. 1989.