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Fibrous dysplasia.

Selene G Parekh1, Rakesh Donthineni-Rao, Eric Ricchetti

  • 1Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

The Journal of the American Academy of Orthopaedic Surgeons
|October 8, 2004
PubMed
Summary

Fibrous dysplasia is a rare bone disorder affecting young people, often found incidentally. While usually benign, it can cause pain or deformity, with surgical or medical management options available.

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Area of Science:

  • Orthopedics
  • Genetics
  • Pathology

Background:

  • Fibrous dysplasia is a developmental bone disorder with monostotic or polyostotic forms.
  • It affects adolescents and young adults, comprising 7% of benign bone tumors.
  • Lesions are often asymptomatic, discovered incidentally, or present with swelling, deformity, or pain.

Purpose of the Study:

  • To provide a comprehensive overview of fibrous dysplasia.
  • To discuss its etiology, clinical presentation, and management.
  • To highlight associated syndromes and prognosis.

Main Methods:

  • Review of existing literature on fibrous dysplasia.
  • Analysis of radiographic characteristics.
  • Discussion of current treatment strategies including surgery and bisphosphonates.

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Main Results:

  • Fibrous dysplasia has a characteristic radiographic appearance.
  • Most cases are managed conservatively; surgical intervention (curettage, bone grafting, fixation) is used when necessary.
  • Bisphosphonates are an alternative when surgery is not feasible.

Conclusions:

  • Fibrous dysplasia is a benign bone tumor with a generally good prognosis.
  • Poorer outcomes are associated with younger patients and polyostotic forms.
  • The risk of malignant transformation is low.