Pediatr. praxi 2018; 19(2): 72-78 | DOI: 10.36290/ped.2018.015

Fibrous dysplasia – pathophysiology and orthopedic treatment

MUDr. Jiří Pečený, MBA1, MUDr. Eduard ©»astný, Ph.D.2, MUDr. Lukáą Wagenknecht2, MUDr. Jaromír Přidal2
1 MEDTEMPL s. r. o., privátní lékařská praxe Praha
2 Klinika dětské a dospělé ortopedie a traumatologie 2. LF UK a FN v Motole, Praha

Fibrous dysplasia is a benign bone disease occuring in mono- or polyostotic form that affects predominantly long bones of thelimbs and cranium. Its etiology is associated with gene mutation on the 20th chromosome leading to the failure of bone formationand remodeling. Most lesions are asymptomatic, the rest is manifested by pain, the development of deformity or pathologicalfracture. Medication treatment can help reduce pain, surgical therapy involving biopsy is indicated for the eradication of symptomaticlesions, fracture repair and deformity.

Keywords: fibrous dysplasia, osteolysis, ortopedic treatment, osteosynthesis, intramedullary nailing

Published: May 1, 2018  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Pečený J, ©»astný E, Wagenknecht L, Přidal J. Fibrous dysplasia – pathophysiology and orthopedic treatment. Pediatr. praxi. 2018;19(2):72-78. doi: 10.36290/ped.2018.015.
Share...
Download citation
  • BibTeX (.bib)
  • Bookends (.ris)
  • EasyBib (.ris)
  • EndNote (.enw)
  • EndNote 8 (.xml)
  • ISI WoS (.isi)
  • Medlars (.medlars)
  • Mendeley (.ris)
  • MODS (.xml)
  • Papers (.ris)
  • RefWorks (.txt)
  • RefManager (.ris)
  • RIS (.ris)
  • MS Word (.xml)
  • Zotero (.ris)
    • Open full article

    References

    1. Matějovský Z, Povýąil J, Kolář J. Nádory kostí. Praha: Avicenum 1988.
    2. Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg 1938; 36: 874-887. Go to original source...
    3. Iwasko N, Steinbach LS, Disler D, et al. Imaging findings in Mazabraud's syndrome: seven new cases. Skeletal Radiol 2002; 31(2): 81-87. Go to original source... Go to PubMed...
    4. Pollandt K, Lohmann CH, Werner M, et al. Clinical pathological aspects of Mazabraud's syndrome. Pathologe 2002; 23: 357-360. Go to original source... Go to PubMed...
    5. Mařík I. Systémové, končetinové a kombinované vady skeletu - 1. část: diagnostické,terapeutické a biomechanické aspekty. Pohybové ústrojí 2000; 7: 83-214.
    6. Shenker A, Weinstein LS, Sweet DE, et al. An activatingGs alfa mutationis present in fibrous dysplasia of bone in the McCune-Albright syndrome. J Clin Endocrinol Metab 1994; 79(3): 750-755. Go to original source...
    7. Malchoff CD, Reardon G, Mac Gillivray DC, et al. An ususal presentation of McCune-Albright syndrome confirmed by an activating mutation of the Gsalpha-subunit from a bone lesion. J Clin Endocrinol Metab 1994; 78: 803-806. Go to original source...
    8. Kamaleshwaran KK, Joseph J, Kalarikal R, et al. Image findings of polyostotic fibrous dysplasia mimicking metastasis in F-18 FDG positron emission tomography/computed tomography. Indian J Nucl Med 2017; 32: 137-139. Go to original source... Go to PubMed...
    9. Bláha R. Rentgenologie kostí a kloubů. II. díl. Praha: Státní zdravotnické nakladatelství 1963.
    10. Zídková H, Kolář J, Matějovský Z. Malignizace či agresivní růst fibrózní dysplazie? Čes Radiol 1993; 47(3): 139-149.
    11. Noh JH, Lee JW. Fibrous dysplasia in the epiphysis of the distal femur. Knee Surg Relat Res 2017; 129(1): 69-71. Go to original source... Go to PubMed...
    12. Batista KT, Araújo HJ, Schwartzman UP. Monostotic fibrous dysplasia of the metacarpal: a case report. Rev Bras Ortop 2016; 51(6): 730-734. Go to original source... Go to PubMed...
    13. Di Caprio M, Enneking W. Current concepts review, fibrous dysplasia. J Bone Joint Surg Am 2005; 87: 1848-1864. Go to original source... Go to PubMed...
    14. Zídková H, Kolář J, Matějovský Z, et al. Příspěvek k postiľení páteře fibrózní dysplazií. Čes Radiol 1994; 48(3): 146-152.
    15. ®iľkovská K. Osteofibrózní dysplazie. Čes Radiol 1993; 47(1): 22-23.
    16. Huvos AG, Higinbotham NL, Miller TR. Bone sarcomas arising in fibrous dysplasia. J. Bone Joint Surg Am 1972; 54: 1047-1056. Go to original source...
    17. Willert HG, Zichner L, Enderle A. Die Selbstkorrektur von Schaftverbiegungen wahrend des Wachstums. Orthop Prax 1975; 11: 167.
    18. Stephenson RB, London MD, Hankin FM, et al. Fibrous dysplasia, an analysis of options for treatment. J Bone Joint Surg Am 1987; 69(3): 400-409. Go to original source...
    19. O'Sullivan M, Zacharin M. Intramedullary rodding and bisphosphonate treatment of polyostotic fibrous dysplasia associated with the McCune-Albright syndrome. J Pediatr Orthop 2002; 22: 255-259. Go to original source... Go to PubMed...
    20. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg Am. 1981; 63(3): 367-375. Go to original source...
    21. Stanton RP. Surgery for fibrous dysplasia. J Bone Miner Res 2006; Suppl 2: 105-109. Go to original source... Go to PubMed...
    22. Adrisano A, Soncini G, Calderoni PP, et al. Critical review of infantile fibrous dysplasia: surgical treatment. J Pediatr Orthop 1991; 11(4): 478-481. Go to original source... Go to PubMed...
    23. Hefti F, Donnan L, Krieg AH. Treatment of shepherd's crook deformity in patients with polyostotic fibrous dysplasia using a new type of custom made retrograde intramedullary nail: a technical note. J Child Orthop 2017; 11(1): 64-70. Go to original source... Go to PubMed...
    24. Robey PG, Kuznetsov S, Riminucci M, et al. The role of stem cells in fibrous dysplasia of bone and the Mccune-Albright syndrome. Pediatr Endocrinol Rev 2007; Suppl 4: 386-394. Go to PubMed...

    Return to the content


    Pediatrics for Practice

    Madam, Sir,
    please be aware that the website on which you intend to enter, not the general public because it contains technical information about medicines, including advertisements relating to medicinal products. This information and communication professionals are solely under §2 of the Act n.40/1995 Coll. Is active persons authorized to prescribe or supply (hereinafter expert).
    Take note that if you are not an expert, you run the risk of danger to their health or the health of other persons, if you the obtained information improperly understood or interpreted, and especially advertising which may be part of this site, or whether you used it for self-diagnosis or medical treatment, whether in relation to each other in person or in relation to others.

    I declare:

    1. that I have met the above instruction
    2. I'm an expert within the meaning of the Act n.40/1995 Coll. the regulation of advertising, as amended, and I am aware of the risks that would be a person other than the expert input to these sites exhibited

    No
    Yes

    If your statement is not true, please be aware
    that brings the risk of danger to their health or the health of others.