fibrous dysplasia
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FIBROUS DYSPLASIA
Dr/ Hytham Nafady
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Definition
replacement of the normal lamellar cancellous bone by abnormal fibrous tissue.
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Types of fibrous dysplasia
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Demographics
Age: 3-30. Sex: F > M.
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Natural history
Polystotic FDis more aggressive than monostotic FD.
The lesions usually progress in number till the end of skeletal maturation, by then they become quiescent
in only about 5% of cases it continue to enlarge after that.
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Complications of fibrous dysplasia
1. Pathological fracture.2. Bone deformity.3. Massive cartilage hyperplasia.4. Accelerated bone growth.5. Sarcomatous degeneration.
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Pathological fracture
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Shepherd crock deformity
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Sarcomatous degeneration
is very rear, it may occur spontaneously or following radiation therapy.
Radiological Criteria: Cortical destruction. Extraosseous soft tissue
component.
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Radiological criteria
Location : Long bones specially the neck of femur Skull (including calvarium, skull base, facial
bones & mandible), Pelvis Ribs (fibrous dysplasia is the most common
cause of rib expansion).Distribution: Unilateral or rarely bilateral asymmetrical.
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Long bone fibrous dysplasia
Meta-diaphyseal. The epiphysis is usually spared. Central May be expansile. Cortex thinned or scalloped with no cortical break through or
periosteal reaction (smooth outer cortex).Density: depend upon the ratio between the fibrous & osseous
tissues, Sclerotic (increased osseous content), or Lytic (increased fibrous content) or Ground glass, (relatively equal ratios between fibrous & osseous
content).Margin: Well defined sclerotic margin (Geographic bone
destruction). The sclerotic margin may be thick (rind sign).
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Pelvis fibrous dysplasia
Expansile, lytic, bubbly lesion. The degree of expansion & the bubbly nature
are quite marked in the pelvis rather than long bones.
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Rib fibrous dysplasia
Expansile lytic lesion with no rib destruction.
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Calvarium Skull base Facial bones
Widening of the diploic space.Displacement of the outer table.Sparing of the inner table.
Narrowing of the neural foramina.
Facial deformities
Density: •Sclerotic or Ground glass, •Common
•Lytic or mixed •Rare
DDx: Paget disease Skull base tumors Ossifying fibroma
(the inner table is involved). Fibrous dysplasia conforms to the shape of the involved bone.
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Special forms of fibrous dysplasia
Leontiasis ossea. Cherubism. Mc Cune Albright syndrome.
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Leontiasis ossea
A special form of polystotic fibrous dysplasia that affects the skull & facial bones.
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Cherubism
Familial fibrous dysplasia of the jaws.
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Mc Cune Albrightsyndrome
Almost exclusively affect females.
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Mc Cune Albright syndrome Neurofibromatosis
Never cross the midline and Cross the midline.
Irregular borders
(coast of Maine)
Smooth borders
(coast of California)