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$Unique_ID{BRK03684}
$Pretitle{}
$Title{Dysplasia, Fibrous}
$Subject{Dysplasia Fibrous Monostotic Fibrous Dysplasia Jaffe-Lichenstein
Disease Polyostotic Fibrous Dysplasia}
$Volume{}
$Log{}
Copyright (C) 1991 National Organization for Rare Disorders, Inc.
839:
Dysplasia, Fibrous
** IMPORTANT **
It is possible that the main title of the article (Fibrous Dysplasia) is
not the name you expected. Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Disorder Subdivisions:
Monostotic Fibrous Dysplasia (Jaffe-Lichenstein Disease)
Polyostotic Fibrous Dysplasia
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.
Fibrous Dysplasia is a disease of the medullary bone in which benign
cysts occur as a result of irregular bone development. This disorder may
involve a single bone (Monostotic Fibrous dysplasia or Jaffe-Lichtenstein
disease) or affect multiple bones (Polyostotic Fibrous dysplasia or McCune
Albright syndrome).
Fibrous Dysplasia first appears during childhood and the bone lesions
usually stop developing at puberty. These lesions may be painful, deforming
and widespread. The bones most often affected are the ribs, skull, facial
bones, femur and tibia of the leg.
Symptoms
The symptoms of Monostotic Fibrous Dysplasia usually develop during childhood
and are detected by a skeletal x-ray. Individuals with this disorder may
have lesions (cysts) of the craniofacial bones, vertebrae or long bones (such
as the femur and tibia). There may be a loss of density at the location
where the bone is being replaced by fibrous tissue and this loss is usually
detected by an x-ray. The tissue around the affected bone (cortical walls)
may atrophy and the bone marrow cavity (medullary cavity) may expand. There
is never more than a single bone affected with this form (monostotic) of
Fibrous Dysplasia.
Individuals with Polyostotic Fibrous Dysplasia may also have McCune
Albright Syndrome which is characterized by signs of early sexual development
(precocious puberty), skin pigmentation (cafe' au lait spots) and multiple
fibrous bone lesions. The bone lesions of Polyostotic Fibrous Dysplasia are
usually present during childhood and may involve a large percentage of the
skeleton. A skeletal x-ray is used to detect this disorder often as a result
of complaints by the patient of bone and joint pain or repeated fractures.
There may be femur deformity and a discrepancy in leg length (known as
"shepherds-crook") as well as facial disfigurement.
Causes
The exact cause of Fibrous Dysplasia is not known. Most cases seem to occur
sporadically. Some scientists have reported that they suspect that this
disorder may be inherited but this has not yet been proven.
Affected Population
Fibrous Dysplasia seems to affect both males and females equally. About
fifty percent of the females with Polyostotic Fibrous Dysplasia have McCune
Albright Syndrome with early sexual development.
Therapies: Standard
Fibrous Dysplasia may be treated with orthopedic procedures. Packing with
bone chips and curettage (scraping) of the lesions may be used although it
has been found that in patients under 18 years of age this procedure (when
used on lower extremities) is usually unsatisfactory. The same procedure
used on patients over 18 years of age may have satisfactory results.
Internal fixation (stabilizing the bone with surgical wires, screws, pins or
plates) may be used on lesions in the lower extremities of patients under 18
years of age.
In cases of McCune Albright Syndrome the drug medroxyprogesterone may be
used to prevent early sexual development.
Therapies: Investigational
There are several experimental drugs being tested for treatment of McCune
Albright Syndrome which is sometimes associated with Polyostotic Fibrous
Dysplasia. To learn about these drugs see the "Investigational Therapies"
section of the McCune-Albright Syndrome entry on the Rare Disease Database.
This disease entry is based upon medical information available through
May 1991. 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 Fibrous Dysplasia, please contact:
National Organization for Rare Disorders
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
International Center for Skeletal Dysplasia
St. Joseph Hospital
7620 York Road
Towson, MD 21204
(301) 337-1250
The National Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Information Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown
and Co., 1987. Pp. 2117-8.
CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp. 1519-20.
BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, M.D. Ed; Blackwell
Scientific Publications., 1990. Pp. 738-39.
FIBROUS DYSPLASIA. AN ANALYSIS OF OPTIONS FOR TREATMENT: R.B.
Stephenson, et al.; J Bone Joint Surg (Am); (Mar 1987, issue 69(3). Pp. 400-
9.
FIBROUS DYSPLASIA OF BONE: B.E. Stompro, et al.; Am Fam Physicians; (Mar
1989, issue 39(3)). Pp. 179-84.