Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content +++ Key Features ++ Dysplastic fibrous tissue replacement of the medullary canal is accompanied by the formation of metaplastic bone in areas with fibrous dysplasia Three forms of the disease are recognized Monostotic Polyostotic Polyostotic with endocrine disturbances (precocious puberty in females, hyperthyroidism, and hyperadrenalism [Albright syndrome]) +++ Clinical Findings ++ Characteristics of lesions May be asymptomatic Begins centrally within the medullary canal, usually of a long bone, and expands slowly Pain, if present, is probably due to pathologic fractures In females, endocrine disturbances may be present in the polyostotic variety and are associated with café au lait spots +++ Diagnosis ++ If metaplastic bone predominates, the contents of the lesion have the density of bone The disease is often asymmetrical, and limb length disturbances may occur as a result of stimulation of epiphysial cartilage growth Marked deformity of the bone may result, and a shepherd's crook deformity of the upper femur is a classic feature of the disease +++ Treatment ++ No treatment is needed if the lesion is small and asymptomatic Curettage and bone grafting are indicated if the lesion is large and produces or threatens pathologic fracture GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options