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Fibrous Dysplasia

Incidence

  • 5 - 20% benign bone lesions
  • Relatively common and usually monostotic (80%)
  • Affects children and adolescents (10-25 y/o)
  • Median age at onset 8 years
  • Male more than Female

Clinically

  • Ribs are commonest sites (40%)
  • Lower limbs more than upper limbs
  • Craniofacial; skull deformity
  • Epiphyses usually spared
  • Monostotic disease almost 50% have an asymptomatic rib lesion
  • Polyostotic disease
    • Pain
    • Fracture (85%)
    • Deformity 
    • Skin pigmentation (coast of Maine)

X-Rays

  • Ground glass appearance typical
  • Shepherds crook deformity of proximal femur
  • Variable appearance with expansion of cortex

Pathology

  • ? developmental hamartoma
  • Bone replaced by firm, whitish tissue of gritty consistency (alphabet soup or chinese letters)
  • Vascular tumour with poorly orientated bone trabeculae separated by fibrous tissue
  • Bone is woven rather than lamellar and lacks osteoblastic rimming of trabeculae

Treatment

  • Monostotic : curettage and grafting, if symptomatic
  • Polyostotic : symptomatic treatment
  • May require osteotomy for deformity or lengthening / shortening procedures

Prognosis

  • Monostotic lesions cease activity at puberty, but may be reactivated by pregnancy
  • Polyostotic presents with pathological fracture in 85%
  • Rarely show malignant change (<1%), unless after radiotherapy

Albright Disease

  • A varaint of Fibrous Dysplasia
  • Female more than Male
  • Consists of
    • Polyostotic disease (unilateral usually)
    • Skin pigmentation (coast of Maine)
    • Endocrinopathy (Precocious puberty)

Osteofibrous Dysplasia

  • Rare variant of Fibrous Dysplasia
  • Seen in young children
  • Usually affects tibial cortex
  • Mostly asymptomatic
  • May present with local tenderness or bowing of tibia
  • Highly vaiable appearance on X-Ray
  • Histology is similar to Fibrous Dysplasia, but has osteoblastic rimming
  • No treatment necessary, unless symptomatic
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