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Fibrous dysplasia

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Dr/ Hytham Nafady

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replacement of the normal lamellar cancellous bone by abnormal fibrous tissue.

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Types of fibrous dysplasia

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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


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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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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)