Fast Five Quiz: Fibrodysplasia Ossificans Progressiva

Dirk M. Elston, MD

Disclosures

January 14, 2021

CT is the best method for detection of early FOP lesions. Bone scintigraphy shows increased uptake of radiolabeled diphosphonate before ossification occurs and therefore ossification can be seen via radiographic examination. Although clinical presentation can yield a definitive diagnosis of FOP, these evaluations provide a deeper understanding of the disease process.

Routine biochemical evaluations of bone mineral metabolism are usually normal in patients with FOP, although serum alkaline phosphatase activity and the erythrocyte sedimentation rate may be elevated, especially during disease flare-ups in children.

Biopsy is generally not indicated in FOP owing to the risk of developing lesions proximate to the trauma.

Histologic examination shows a pronounced proliferation of fibroblasts within the muscles in several areas, leading to secondary destruction of muscle fibers. However, smooth and cardiac muscle are not involved, nor are certain skeletal muscles, including the diaphragm, tongue, and extraocular muscles.

Learn more about evaluation of FOP.

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