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  • Fibrous Dysplasia Bone Condition

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    Nurul Ramadani
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    Fibrous dysplasia is a benign condition where normal bone is replaced by fibrous connective tissue. It most commonly presents as a monostotic lesion affecting one bone, such as the ribs, femur or craniofacial bones. Imaging shows a "ground glass" appearance and lesions can be cystic, sclerotic or mixed. While there is no cure, treatment involves curettage, bone grafting and fixation to address pain, swelling or nerve compression. Malignant transformation is rare, occurring in less than 1% of cases.

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    KP 3.2.4.4 b Displasia Fibrosa

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    Fibrous dysplasia is a benign condition where normal bone is replaced by fibrous connective tissue. It most commonly presents as a monostotic lesion affecting one bone, such as the ribs, femur or craniofacial bones. Imaging shows a "ground glass" appearance and lesions can be cystic, sclerotic or mixed. While there is no cure, treatment involves curettage, bone grafting and fixation to address pain, swelling or nerve compression. Malignant transformation is rare, occurring in less than 1% of cases.

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    © All Rights Reserved
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    35 views19 pages

    Fibrous Dysplasia Bone Condition

    Uploaded by

    Nurul Ramadani
    Fibrous dysplasia is a benign condition where normal bone is replaced by fibrous connective tissue. It most commonly presents as a monostotic lesion affecting one bone, such as the ribs, femur or craniofacial bones. Imaging shows a "ground glass" appearance and lesions can be cystic, sclerotic or mixed. While there is no cure, treatment involves curettage, bone grafting and fixation to address pain, swelling or nerve compression. Malignant transformation is rare, occurring in less than 1% of cases.

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    © All Rights Reserved
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    of 19

    Fibrous Dysplasia

    Definition
    • Benign condition in which normal bone is
    replaced by fibrous connective tissue due to a
    defect in osteoblast differentiation and
    maturation
    Epidemiology
    • Incidence not known
    • Females > males
    • No race predilection
    • Initial symptoms manifest age 3-15
    • Not heritable
    • Questionable genetic transformation
    • Malignant transformation in < 1%
    Variations
    • Cystic (21%)
    - Radiolucency surrounded by solid rim

    • Sclerotic (23%)
    - Dense and homogenous

    • Mixed (56%)
    - “Ground glass appearance”
    Variations, cont’
    cont’d
    • Monostotic
    - Most common
    - 25% involve head and neck
    • Polyostotic
    - 15% of cases
    - 50% involve head and neck
    Location
    Monostotic form
    •ribs: 28%, most common 6,7
    •proximal femur: 23%
    •tibia
    •craniofacial bones: 10-25% 4
    •humerus
    Polyostotic form

    • ribs
    • often unilateral and
    monomelic: one limb • skull and facial bones:
    6 50% 4
    • femur: 91% • upper extremities
    • tibia: 81% • lumbar spine: 14%
    • pelvis: 78% • clavicle: 10%
    • foot: 73% • cervical spine: 7%
    Radiographic features
    • Plain radiograph
    • ground-glass matrix
    • may be completely lucent (cystic) or sclerotic
    • well circumscribed lesions
    • no periosteal reaction
    • rind sign
    Images
    • Left temporal bone
    involvement
    Images
    • Right temporal bone
    lesion
    Images
    Presentation
    • Local pain
    • Swelling
    • Abnormal pigmentation
    • CN compression
    • Spontaneous scalp hemorrhage
    • Part of McCune-Albright’s syndrome
    McCune--Albright
    McCune Albright’’s Syndrome
    • Polyostotic fibrous dysplasia
    • Café-au-lait spots
    • Endocrinopathology:
    - Hyperthyroidism
    - Precocious puberty in females
    Imaging
    • Plain radiography is first line
    • Computed tomography for complex regions
    Histology
    • Fibroblasts within
    woven cancellous bone
    Differential Diagnosis
    • Eosinophilic granuloma
    • Nonossifying fibroma
    • Bone hemangioma
    • Hyperparathyroidism
    • Paget’s disease
    • Brown’s tumor
    • Aneurysmal bone cyst
    Treatment
    • No available cure
    • Curettage
    • Bone graft
    • Fixation
    • Biphosponate
    • Calcitonin
    The One Slide To Remember
    • Genetic, non-heritable disorder
    • <1% transformation to malignancy
    • Treatment is curretage , bone graft and
    fixation
    Thank You

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