Orthopedic Surgery for Fibrous Dysplasia Treatment & Management

  • Author: Bernardo Vargas; Chief Editor: Harris Gellman, MD   more...
 
Updated: May 15, 2012
 

Medical Therapy

Although there is no specific medical therapy for fibrous dysplasia, studies have shown decreased pain after treatment with bisphosphonates. Bisphosphonates inhibit bone resorption by their action on osteoclasts (see also Future And Controversies ). The most common drug therapy is intravenous pamidronate. An intravenous infusion of pamidronate (total dose of 1 mg/kg/day over 3 days, repeated every 3-6 mo) has been proposed. The total dose must be administered over a 4-hour period. Vitamin D and calcium supplements must be added to this therapy. This therapy in children seems to be safe, but longer follow-up is needed to confirm the absence of collateral effects on the growth plate. An increased growth-plate thickness has been reported in children treated with bisphosphonates.[2]

The PROFIDYS study (Oral Bisphosphonate Effect on Osseous Symptoms in Fibrous Dysplasia of Bone) is a double-blind study evaluating the long-term safety and results of treatment with an oral bisphosphonate (risedronate [Actonel]), which has been ongoing since 2007. The study is evaluating bone pain and the evolution of osteolytic lesions in patients with fibrous dysplasia, and it is expected to be completed by 2013.

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Surgical Therapy

  • If surgical treatment is required for fibrous dysplasia in long bones, intramedullary nails are recommended.[26]
  • This technique provides good stabilization and could prevent deformation.
  • Conservative treatment, use of plates, curettage, or bone grafting should be discouraged.[10, 14, 27]
  • Deformity-correction surgery is indicated in patients with mechanical axis deviation of the lower limbs.
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Intraoperative Details

  • The dysplastic bone in fibrous dysplasia can be quite difficult to ream.
  • Fibrous dysplasia is associated with a high tendency of bone bleeding during surgery.[10]
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Follow-up

  • The main role of the follow-up is to prevent deformity as a result of the disease.
  • The authors recommend yearly radiographs of the involved area or areas until skeletal maturity.
  • Fibrous dysplasia rarely undergoes remission. For this reason, it is appropriate to periodically monitor the disease progression, especially in the skeletally immature patient.
  • Once skeletal maturity has been achieved, it is unusual for monostotic fibrous dysplasia to progress.
  • Early intervention with internal fixation of involved bones may be important in the prevention of deformity.
  • Referral to an endocrinologist for endocrine and metabolic testing is suggested so that endocrine anomalies can be diagnosed and treated.
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Complications

  • Fracture is the most common complication of fibrous dysplasia. In polyostotic disease, fracture occurs in more than 50% of cases.
  • Deformity may occur in weight-bearing bones.
  • Malignant transformation occurs in less than 0.5% of cases. It is more likely to occur if polyostotic disease exists or following treatment with radiation therapy. Typically, malignant transformation occurs during the third or fourth decade of life.[12] Benign tumors have also been associated with fibrous dysplasia.[28]
  • Patients with McCune-Albright syndrome have a high incidence of scoliosis (probably more than 50%).[2]
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Outcome and Prognosis

  • The recurrence rate for fibrous dysplasia has been reported to be 21% following curettage and grafting, but if patients are monitored for many years, the rate is probably closer to 100%.
  • Unless malignant transformation develops, fibrous dysplasia is not a life-threatening disease. The lesions tend to stabilize as skeletal maturity is reached.
  • The majority of the monostotic cases have a good evolution regardless of treatment.
  • Polyostotic lesions are very often associated with one or more fractures.[14]
  • Malignant transformation develops in a minority of patients (< 0.5%)
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Future and Controversies

Major advances have occurred in understanding the molecular basis of fibrous dysplasia. The mutation has been identified, but the actual pathways that lead to abnormal osteoblast differentiation and function are just beginning to be understood.

In the future, effective nonsurgical treatments may be possible. The risk of local recurrence is high, so the decision to treat must be made with informed consent to avoid inappropriate expectations. In general, the goals of surgery should be to stabilize the bone and relieve pain, rather than to excise the involved bone. The condition often is found incidentally, and the need for prophylactic treatment may be difficult to accept for an asymptomatic or minimally symptomatic patient.

The PROFIDYS study (Oral Bisphosphonate Effect on Osseous Symptoms in Fibrous Dysplasia of Bone) is double-blind study evaluating the long-term safety and results of treatment with an oral bisphosphonate (risedronate [Actonel]), which has been ongoing since 2007. The study is evaluating bone pain and the evolution of osteolytic lesions in patients with fibrous dysplasia, and it is expected to be completed by 2013.

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Contributor Information and Disclosures
Author

Bernardo Vargas  MD, Consulting Staff, Department of Orthopedic Surgery, University Hospital of Geneva, Switzerland

Disclosure: Nothing to disclose.

Coauthor(s)

Mark Clayer, MD, MBBS, FRACS, FAOrthA  Head of Musculoskeletal Tumor Service, Department of Orthopaedics and Trauma, Queen Elizabeth Hospital; Senior Visiting Medical Specialist, Royal Adelaide Hospital and Women's and Children's Hospital, Australia

Mark Clayer, MD, MBBS, FRACS, FAOrthA is a member of the following medical societies: Australian Medical Association and Australian Orthopaedic Association

Disclosure: Orthopedics hyperguide Honoraria Independent contractor; Stryker Grant/research funds Employment

Specialty Editor Board

Howard A Chansky, MD  Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center

Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Sean P Scully, MD, PhD  Professor, Department of Orthopedics, University of Miami

Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD  Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society

Disclosure: Nothing to disclose.

References

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  4. Albright F, Butler AM, Hampton AO. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females. New Engl J Med. 1937;216:727-46.

  5. Fraser WD, Walsh CA, Birch MA. Parathyroid hormone-related protein in the aetiology of fibrous dysplasia of bone in the McCune Albright syndrome. Clin Endocrinol. 2000;53(5):621-8. [Medline].

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  10. Ippolito E. Bray E. W., Corsic A. et als. Natural history and treatment of fibrous dysplasia of bone:a multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society. J of Ped Orthop Part B. 2003,;12:155-177. [Medline].

  11. Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer. 1994;73(5):1411-24. [Medline].

  12. Yabut SM Jr, Kenan S, Sissons HA, Lewis MM. Malignant transformation of fibrous dysplasia. A case report and review of the literature. Clin Orthop. 1988;228:281-9. [Medline].

  13. Marie PJ, de Pollak C, Chanson P, Lomri A. Increased Proliferation of Osteoblastic Cells Expressing the Activating Gsa Mutation in Monostotic and Polyostotic Fibrous Dysplasia. Am J Pathol. 1997;150:1059-1069. [Medline].

  14. Guille JT, Kumar SJ, MacEwen GD. Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment. J Bone Joint Surg Am. May 1998;80(5):648-58. [Medline].

  15. McCune DJ:. Osteitis fibrosa cystica: the case of a nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism. Am J Dis Child. 1936;52:743-747.

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  18. Chapurlat RD, Hugueny P, Delmas PD, Meunier PJ. Treatment of fibrous dysplasia of bone with intravenous pamidronate: long-term effectiveness and evaluation of predictors of response to treatment. Bone. 2004;Volume 35, Issue 1:235-242. [Medline].

  19. Liens D, Delmas PD, Meunier PJ:. Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet. 1994;343:953-954. [Medline].

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  23. Murray DJ, Edwards G, Mainprize JG, Antonyshyn O. Advanced technology in the management of fibrous dysplasia. J Plast Reconstr Aesthet Surg. Aug 2008;61(8):906-16. [Medline].

  24. Li P, Zhang ZR, Jiang Y, Xia XD, Wang D, Li XF. MR and CT findings of cyst degeneration of sphenoid bone in McCune-Albright syndrome: a case report. Cases J. Dec 22 2009;2:9376. [Medline]. [Full Text].

  25. Ferreira EC, Brito CC, Domingues RC, Bernardes M, Marchiori E, Gasparetto EL. Whole-body MR imaging for the evaluation of McCune-albright syndrome. J Magn Reson Imaging. Mar 2010;31(3):706-10. [Medline].

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Intermediate-power view of typical histology of fibrous dysplasia. Note the bland fibrous stromal tissue with islands of disorganized, immature osteoid. A key feature is the absence of rimming osteoblasts around the osteoid. While not present in this slide, foci of cartilage also may occasionally be present.
Plain radiograph of a tibia in a patient who is skeletally mature, demonstrating expansion of the metaphysis and diaphysis, endosteal scalloping, and a ground-glass appearance of the matrix.
Technetium-99m methylene diphosphonate (MDP) bone scan demonstrating increased uptake in the tibia corresponding to the radiographic margins.
CT scan of the tibia demonstrating expansion of the tibia due to an expanding intramedullary lesion.
A T1-weighted MRI image demonstrating intermediate signal intensity and no soft tissue component.
A T2-weighted MRI image demonstrating increased signal intensity of the matrix of the lesion.
The metaplastic bone formed by fibrous dysplasia has the appearance of Chinese letters.
 
 
 
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