eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Fibrodysplasia Ossificans Progressiva (Myositis Ossificans): Follow-up

Author: Robert J Pignolo, MD, PhD, Assistant Professor of Medicine, Director, Ralston-Penn Clinic for Osteoporosis and Related Bone Disorders, Department of Medicine, Division of Geriatric Medicine, Associate Director, Structure-Function Biomechanical Core, Penn Center for Musculoskeletal Disorders, University of Pennsylvania School of Medicine
Coauthor(s): Frederick S Kaplan, MD, Isaac and Rose Nassau Professor of Orthopedic Molecular Medicine, Chief, Division of Orthopedic Molecular Medicine, Director, Center for Research in FOP and Related Disorders, The University of Pennsylvania School of Medicine; Consulting Surgeon, Department of Orthopedic Surgery, Hospital of The University of Pennsylvania; Eileen M Shore, PhD, Research Associate Professor, Departments of Orthopaedic Surgery and Genetics, University of Pennsylvania School of Medicine
Contributor Information and Disclosures

Updated: Jul 30, 2009

Follow-up

Deterrence/Prevention

  • If a parent has fibrodysplasia ossificans progressiva (FOP), the chance that a child will have fibrodysplasia ossificans progressiva is 50%. In addition to the usual risks that any woman might encounter during pregnancy, a woman with fibrodysplasia ossificans progressiva has additional concerns that must be carefully considered, including substantial life-threatening risks to both the mother and child.
  • Present and future rehabilitation approaches should be focused on enhancing activities of daily living. Occupational therapy and vocational education consultations may be extremely useful. Passive range of motion must be avoided, as it likely leads to disease exacerbations.
  • Measures to prevent falls should be directed at modification of activity, improvement in household safety, use of ambulatory devices (such as a cane, if possible), and use of protective headgear.
  • Prophylactic measures to maximize pulmonary function, minimize respiratory compromise, and prevent influenza and pneumonia are helpful in decreasing the morbidity and mortality from thoracic insufficiency syndrome.

Prognosis

  • As heterotopic bone accumulates in fibrodysplasia ossificans progressiva, range of motion is progressively lost, leading to near complete immobility.
  • Patients with fibrodysplasia ossificans progressiva develop thoracic insufficiency syndrome (TIS) that can lead to life-threatening complications. Pneumonia and right-sided heart failure are the major life-threatening hazards that result from TIS in patients with fibrodysplasia ossificans progressiva.
  • The median age of survival is approximately 41 years, and death often results from complications of TIS.

Miscellaneous

Special Concerns

Other medical situations

  • The acute and often severe limb swelling seen with flare-ups of fibrodysplasia ossificans progressiva (FOP) may grow to extraordinary and alarming size and lead to extravascular compression of nerves and tissue lymphatics. The appearance of such massive acute swelling in the lower limbs can provoke serious considerations of a deep vein thrombosis.
  • Some patients who have advanced fibrodysplasia ossificans progressiva involving the lower limbs have venous stasis and/or lymphedema. Definitive studies to exclude deep vein thrombosis may be difficult to obtain and interpret due to severe existing deformity and joint ankylosis from previous flare-ups. Limb swelling is often difficult to treat effectively in patients who have fibrodysplasia ossificans progressiva. Nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids have generally not been effective. Support stockings are poorly tolerated by most patients, and elevation of the affected limbs is often impossible because of ankylosis of the major joints, especially later in the disease process.
  • Skin breakdown and pressure sores are common and troublesome problems in individuals who have fibrodysplasia ossificans progressiva. Skin breakdown can occur from increased pressure over normotopic or heterotopic bone.
  • In fibrodysplasia ossificans progressiva, fractures can occur in both normotopic and heterotopic bone. Fractures of heterotopic bone heal rapidly. Elevation, rest, splinting, and local application of ice are often helpful in controlling pain and swelling and may be supplemented by narcotic analgesia, as needed. Fractures of normotopic bone need to be carefully evaluated, as in any patient. Closed reduction and splinting is sufficient for most fractures. Open reduction or internal fixation is almost never warranted and can lead to rapid onset of heterotopic ossification. Healing may be delayed in osteoporotic bone.

 


More on Fibrodysplasia Ossificans Progressiva (Myositis Ossificans)

Overview: Fibrodysplasia Ossificans Progressiva (Myositis Ossificans)
Differential Diagnoses & Workup: Fibrodysplasia Ossificans Progressiva (Myositis Ossificans)
Treatment & Medication: Fibrodysplasia Ossificans Progressiva (Myositis Ossificans)
Follow-up: Fibrodysplasia Ossificans Progressiva (Myositis Ossificans)
Multimedia: Fibrodysplasia Ossificans Progressiva (Myositis Ossificans)
References
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References

  1. Cohen RB, Hahn GV, Tabas JA, et al. The natural history of heterotopic ossification in patients who have fibrodysplasia ossificans progressiva. A study of forty-four patients. J Bone Joint Surg Am. Feb 1993;75(2):215-9. [Medline].

  2. Kaplan FS, Glaser DL, Shore EM, et al. The phenotype of fibrodysplasia ossificans progressiva. Clin Rev Bone Miner Metab. 2005;3:183-188.

  3. Kaplan FS, Groppe J, Pignolo RJ, Shore EM. Morphogen receptor genes and metamorphogenes: skeleton keys to metamorphosis. Ann N Y Acad Sci. Nov 2007;1116:113-33. [Medline].

  4. Shore EM, Xu M, Feldman GJ, et al. A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressiva. Nat Genet. May 2006;38(5):525-7. [Medline].

  5. Kaplan FS, Xu M, Seemann P, Connor JM, Glaser DL, Carroll L, et al. Classic and atypical fibrodysplasia ossificans progressiva (FOP) phenotypes are caused by mutations in the bone morphogenetic protein (BMP) type I receptor ACVR1. Hum Mutat. Mar 2009;30(3):379-90. [Medline].

  6. Kaplan FS, Le Merrer M, Glaser DL, et al. Fibrodysplasia ossificans progressiva. Best Pract Res Clin Rheumatol. Mar 2008;22(1):191-205. [Medline].

  7. Rocke DM, Zasloff M, Peeper J, Cohen RB, Kaplan FS. Age- and joint-specific risk of initial heterotopic ossification in patients who have fibrodysplasia ossificans progressiva. Clin Orthop Relat Res. Apr 1994;243-8. [Medline].

  8. Schaffer AA, Kaplan FS, Tracy MR, et al. Developmental anomalies of the cervical spine in patients with fibrodysplasia ossificans progressiva are distinctly different from those in patients with Klippel-Feil syndrome: clues from the BMP signaling pathway. Spine (Phila Pa 1976). Jun 15 2005;30(12):1379-85. [Medline].

  9. Deirmengian GK, Hebela NM, O'Connell M, Glaser DL, Shore EM, Kaplan FS. Proximal tibial osteochondromas in patients with fibrodysplasia ossificans progressiva. J Bone Joint Surg Am. Feb 2008;90(2):366-74. [Medline].

  10. Adegbite NS, Xu M, Kaplan FS, Shore EM, Pignolo RJ. Diagnostic and mutational spectrum of progressive osseous heteroplasia (POH) and other forms of GNAS-based heterotopic ossification. Am J Med Genet A. Jul 15 2008;146A(14):1788-96. [Medline].

  11. Pignolo RJ, Foley, KL. Non-hereditary heterotopic ossification. Implications for injury, arthropathy, and aging. Clin Rev Bone Miner Metabol. 2005;3:261-266.

  12. [Guideline] Morrison WB, Dalinka MK, Daffner RH, et al. Expert Panel on Musculoskeletal Imaging. Soft tissue masses. [online publication]. Reston (VA): American College of Radiology (ACR);. 2005;6 p.

  13. Kitterman JA, Kantanie S, Rocke DM, Kaplan FS. Iatrogenic harm caused by diagnostic errors in fibrodysplasia ossificans progressiva. Pediatrics. Nov 2005;116(5):e654-61. [Medline].

  14. Kaplan FS, Strear CM, Zasloff MA. Radiographic and scintigraphic features of modeling and remodeling in the heterotopic skeleton of patients who have fibrodysplasia ossificans progressiva. Clin Orthop Relat Res. Jul 1994;238-47. [Medline].

  15. Kaplan FS, Xu M, Glaser DL, et al. Early diagnosis of fibrodysplasia ossificans progressiva. Pediatrics. May 2008;121(5):e1295-300. [Medline].

  16. Pignolo RJ, Suda RK, Kaplan FS. The fibrodysplasia ossificans progressiva lesion. Clin Rev Bone Miner Metabol. 2005;3:195-200.

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Further Reading

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Keywords

fibrodysplasia ossificans progressiva, FOP, myositis ossificans circumscripta, soft tissue ossification, heterotopic ossification, HO, progressive osseous heteroplasia, nonhereditary heterotopic ossification, NHHO, soft connective tissue swelling, trauma, blunt muscle trauma, malformation of the great toes, toe malformation, neck stiffness, pneumonia, heart failure, diagnosis, treatment

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Contributor Information and Disclosures

Author

Robert J Pignolo, MD, PhD, Assistant Professor of Medicine, Director, Ralston-Penn Clinic for Osteoporosis and Related Bone Disorders, Department of Medicine, Division of Geriatric Medicine, Associate Director, Structure-Function Biomechanical Core, Penn Center for Musculoskeletal Disorders, University of Pennsylvania School of Medicine
Robert J Pignolo, MD, PhD is a member of the following medical societies: American College of Physicians, American Geriatrics Society, American Society for Bone and Mineral Research, Gerontological Society of America, and National Osteoporosis Foundation
Disclosure: Nothing to disclose.

Coauthor(s)

Frederick S Kaplan, MD, Isaac and Rose Nassau Professor of Orthopedic Molecular Medicine, Chief, Division of Orthopedic Molecular Medicine, Director, Center for Research in FOP and Related Disorders, The University of Pennsylvania School of Medicine; Consulting Surgeon, Department of Orthopedic Surgery, Hospital of The University of Pennsylvania
Frederick S Kaplan, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Society for Bone and Mineral Research, American Society for Microbiology, Johns Hopkins Medical and Surgical Association, and Orthopaedic Research Society
Disclosure: Nothing to disclose.

Eileen M Shore, PhD, Research Associate Professor, Departments of Orthopaedic Surgery and Genetics, University of Pennsylvania School of Medicine
Eileen M Shore, PhD is a member of the following medical societies: American Society for Bone and Mineral Research and Royal Society of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David D Sherry, MD, Director, Clinical Rheumatology, Attending Physician, Pain Management, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania
David D Sherry, MD is a member of the following medical societies: American College of Rheumatology and American Pain Society
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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