Fibrous dysplasia (FD)
- Fibrous dysplasia (FD) is a developmental disorder of bone
- Seventy-five per cent of cases present before the age of 30 years
- no sex predilection.
- Two types : monostotic (70–85%) or polyostotic.
- The commonest sites of monostotic FD are the ribs (28%), proximal femur (23%) and craniofacial bones (20%).
- Polyostotic FD may range from the involvement of two bones to more than 75% of the skeleton.
- Approximately 30–50% of patients with polyostotic disease have café-au-lait spots. FD may be associated with a variety of syndromes.
- McCune–Albright syndrome consists of polyostotic FD (typically unilateral), ipsilateral café-au-lait spots and endocrine disturbance, most commonly precocious puberty in girls.
- Mazabraud's syndrome consists of FD (most commonly polyostotic) and soft tissue myxomata.
Radiological features
1.The metadiaphyseal region typically affected in long bones.
2. geographic lesion that may cause bone expansion and
deformity
3.diffuse ground-glass matrix mineralization.
4.A thick sclerotic
margin (‘rind’ sign) (characteristic ).5.Periosteal reaction is not a feature in the absence of fracture.
6.Varus deformity of the proximal femur (shepherd's crook deformity) (characteristic late finding.)
7.Malignant change in fibrous dysplasia rare
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