$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.