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$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.