$Unique_ID{BRK04183} $Pretitle{} $Title{Retrolental Fibroplasia} $Subject{Retrolental Fibroplasia Retinopathy of Prematurity RLF ROP } $Volume{} $Log{} Copyright (C) 1986, 1988 National Organization for Rare Disorders, Inc. 231: Retrolental Fibroplasia ** IMPORTANT ** It is possible the main title of the article (Retrolental Fibroplasia) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article Synonyms Retinopathy of Prematurity RLF ROP General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Retrolental Fibroplasia is a bilateral eye disorder characterized by abnormality of the retinal vessels. It occurs in premature infants with immature retinas who are exposed to oxygen in an incubator, and possibly to bright lights. The degree of sight impairment appears to be relative to the oxygen levels in incubators. Oxygen levels of 30% or more can cause more severe vision impairment. Symptoms Infants affected with Retrolental Fibroplasia are born prematurely, usually weighing less than 1500 gram (about 3 lbs.) at birth and are consequently placed in incubators. If too much oxygen is fed into the incubator, it results in initial constriction of the retinal blood vessels. Then obliteration of the small blood vessels occurs, especially in the temporal retinal periphery. This may be followed by the formation of new blood vessels. If severe, fibrovascular invasion of the vitreous or glass-like body of the eyeball, and retinal detachment may result. If mild, the abnormal vessels may regress and retention of useful vision is possible. Delayed scar changes occur in some infants during the first year, resulting in dragging of the retinal vessels and macula into a temporal retinal fold. Nearsightedness (myopia) is common. Other associated problems include glaucoma, retinal detachment (which causes blindness), or mental retardation (due to premature birth). Causes Retrolental Fibroplasia is caused by a premature infant's exposure to oxygen when blood vessels in the retina are already damaged or too immature. Other factors such as bright lights and even the oxygen normally present in the atmosphere may cause damage to the retina and therefore impair the infant's sight. Affected Population Retrolental Fibroplasia causes vision loss in approximately 2,600 American infants annually, and blindness in approximately 650. Premature infants who are exposed to a high concentration of oxygen after birth may develop Retrolental Fibroplasia. This condition affected numerous children born during the 1950's. After the cause was discovered, hospitals have reduced the amount of oxygen given to babies in incubators. As a result, the incidence of the disorder was significantly reduced. Therapies: Standard Careful monitoring of the amount of oxygen used in the incubator is needed to minimize the incidence of Retrolental Fibroplasia as a complication of prematurity. The lowest concentration necessary for maintenance should be used. The ocular danger increases as the oxygen concentration is increased beyond 30%. An ophthalmologist should be consulted not only by the neonatologist, but also in later years so that long-term complications can be diagnosed and treated. A recently developed surgical procedure called open sky vitrectomy is used to repair a damaged or detached retina in an infant with Retrolental Fibroplasia. Extra small instruments have been developed for this surgery at the Eye Research Institute of Retina Foundation in Boston. This procedure is currently 38% successful. For evaluation of visual function, tests have been developed to measure the electrical responses made by the retina, the optic nerve, and the visual centers in the brain. The visually evoked response (VER), is evoked by changing light patterns. The electrical signals sent to the brain during this response are measured and registered in an electroretinogram. Therapies: Investigational Researchers are investigating cryotherapy as a possible treatment for Retrolental Fibroplasia. Cryotherapy requires physicians to apply a probe cooled to minus eighty degrees Celsius (minus 179 degrees Fahrenheit) to the white area of the eye at points in front of the normal tissue line. The probe destroys cells at 50 or so points and forms a ring of scar tissue to prevent further abnormal growth of blood vessels. Researchers do not fully understand why freezing part of the undeveloped retina of the eye helps normal development. More testing is necessary to determine long-term effects of the treatment. This disease entry is based upon medical information available through September 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Retrolental Fibroplasia, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Eye Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5248 National Association for Parents of the Visually Impaired, Inc. P.O. Box 180806 Austin, TX 78718 (512) 459-6651 National Association for the Visually Handicapped 305 East 24th Street, Room 17-C New York, NY 10010 (212) 889-3141 Eye Research Institute of Retina Foundation 20 Staniford St. Boston, MA 02114 (617) 742-3140 For information on genetics and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. P. 2297. THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 1854.