fibrous dysplasia

chronic disorder of the skeleton Epidemiology: 1) very uncommon disorder 2) usually diagnosed in children & young adults 3) no sex or racial bias Pathology: 1) expansion of one or more bones due to abnormal development of the fibrous connective tissue within the bone 2) uneven growth, brittleness & deformity in affected bones 3) most common sites of the disease are the femur, tibia, ribs, skull, facial bones, humerus, pelvis a) vertebrae are less frequently involved b) any bone can be affected by fibrous dysplasia, 4) does not spread from one bone to another 5) multiple affected bones are often found on one side of the body Clinical manifestations: 1) bone pain (expanding fibrous tissue in the bone_ 2) bone deformity a) most obvious when it occurs in the skull & facial bones b) fibrous dysplasia of the skull may cause loss of vision & hearing 3) fracture most often in the long bones of the legs 4) arthritis can develop in the hips & knees Laboratory: - bone biopsy Radiology: - characteristic appearance on X-ray Complications: - osteosarcoma (uncommon) Management: 1) surgery a) indications - relieve intractable bone pain - improve mobility that may be impaired due to skeletal deformity - facilitate the healing of fractures - relieve local pressure on the spinal cord, spinal nerves, or brain - excision of osteosarcoma b) surgical procedures - removal of affected bone followed by bone grafting in patients with persistent bone pain - removal of a wedge of bone with placement of pins & bone grafts to correct deformities 2) pharmaceutical agents - pamidronate (Aredia) 3) treat hormonal disturbances (see McCune Albright syndrome) 4) exercise is helpful in avoiding weight gain & in maintaining mobility of the joints - supervision to minimize risk of fracture Prognosis: 1) great variability in the clinical course of this disorde 2) young patients who have fibrous dysplasia in many bones may have more problems

Related

fibrous tissue McCune-Albright syndrome

General

bone disease; osteopathia developmental disorder dysplasia

References

Information for Patients about Fibrous Dysplasia http://www.osteo.org/newfile.asp?doc=p111i&doctitle=Fibrous+Dysplasia&doctype=HTML+Fact+Sheet