eMedicine Specialties > Orthopedic Surgery > Neoplasms

Fibrous Dysplasia: Treatment

Author: Bernardo Vargas, MD, Consulting Staff, Department of Orthopedic Surgery, Hôpital Universitaire de Geneva, Switzerland
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
Contributor Information and Disclosures

Updated: Aug 13, 2008

Treatment

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.

Surgical Therapy

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

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

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.

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.23
  • Patients with McCune-Albright syndrome have a high incidence of scoliosis (probably more than 50%).2  

More on Fibrous Dysplasia

Overview: Fibrous Dysplasia
Workup: Fibrous Dysplasia
Treatment: Fibrous Dysplasia
Follow-up: Fibrous Dysplasia
Multimedia: Fibrous Dysplasia
References
[ CLOSE WINDOW ]

References

  1. Alman BA, Greel DA, Wolfe HJ. Activating mutations of Gs protein in monostotic fibrous lesions of bone. J Orthop Res. Mar 1996;14(2):311-5. [Medline].

  2. DiCaprio M. R., Enneking W. F. Fibrous Dysplasia. Pathophysiology, Evaluation, and Treatment. J Bone Joint Surg Am. 2005;87:1848-1864. [Medline].

  3. Marie P. Dysplasie fibreuse : aspects tissulaires, cellulaires et moléculaires. Revue du rhumatisme. 2003;7:681–686.

  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].

  6. Parekh S, Donthineni-Rao R, Ricchetti E. Fibrous dysplasia. J Am Acad Orthop Surg. 2004;12:305-313. [Medline].

  7. Santos CT, Choo CT, Loh AH. Orbital fibrous dysplasia with soft tissue hamartoma--a variant of Mazabraud's syndrome. Orbit. 2008;27(3):207-9. [Medline].

  8. Chapurlat RD, Orcel P. Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol. Mar 2008;22(1):55-69. [Medline].

  9. Lichtenstein L, Jaffe HL:. Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Pathol. 1942;33:777-816.

  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 CellsExpressing 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. Stanton RP, Diamond L. Surgical management of fibrous dysplasia in McCune-Albright syndrome. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:446-52. [Medline].

  16. Kumta SM, Leung PC, Griffith JF, et al. Vascularised bone grafting for fibrous dysplasia of the upper limb. J Bone Joint Surg Br. Apr 2000;82(3):409-12. [Medline].

  17. 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].

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

  19. Zacharin M, O'Sullivan M. I. Intravenous pamidronate treatment of polyostotic fibrous dysplasia associated with the McCune Albright syndrome. J Pediatr. 2000;137(3):403-9. [Medline].

  20. DiMeglio LA. Bisphosphonate therapy for fibrous dysplasia. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:440-5. [Medline].

  21. Lietman SA, Ding C, Levine MA. A. A highly sensitive polymerase chain reaction method detects activating mutations of the GNAS gene in peripheral blood cells in McCune-Albright syndrome or isolated fibrous dysplasia. J Bone Joint Surg Am. 2005;87:2489-2494. [Medline].

  22. 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].

  23. Bridge JA, Rosenthal H, Sanger W. Desmoplastic fibroma arising in fibrous dysplasia. Clin Orthop. 1998;247:272-8. [Medline].

  24. 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.

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

fibrous dysplasia, bone dysplasia, osteochondrodysplasia, Albright disease, bone disease, dysplasia, fibrous dysplasia of bone, fibrous dysplasia bone, cherubism, dysplastic disorder, connective tissue, fibroosseous tissue, lamellar bone, monostotic fibrous dysplasia, polyostotic fibrous dysplasia, precocious puberty, skin pigmentation, McCune-Albright syndrome, Mazabraud syndrome, McCune-Albright's syndrome, Mazabraud's syndrome, Albright's disease, Albright syndrome, Albright's syndrome

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Bernardo Vargas, MD, Consulting Staff, Department of Orthopedic Surgery, Hôpital Universitaire de 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

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

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, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of 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 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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.