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    Fibrous Dysplasia: DR Bareerah Idrees Resident OMFS 08 May 2021

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    This document provides an overview of fibrous dysplasia, a benign bone disorder characterized by the replacement of normal bone with fibro-osseous tissue. It discusses the pathogenesis, clinical features, diagnosis, and treatment of both monostotic and polyostotic forms of fibrous dysplasia. Radiographic imaging and histopathological features are also summarized. Treatment options include observation, medical therapy with bisphosphonates, surgical remodelling, and in some cases radical resection and reconstruction.

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    Fibrous Dysplasia: DR Bareerah Idrees Resident OMFS 08 May 2021

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    biaidrees
    25 views37 pages
    This document provides an overview of fibrous dysplasia, a benign bone disorder characterized by the replacement of normal bone with fibro-osseous tissue. It discusses the pathogenesis, clinical features, diagnosis, and treatment of both monostotic and polyostotic forms of fibrous dysplasia. Radiographic imaging and histopathological features are also summarized. Treatment options include observation, medical therapy with bisphosphonates, surgical remodelling, and in some cases radical resection and reconstruction.

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    This document provides an overview of fibrous dysplasia, a benign bone disorder characterized by the replacement of normal bone with fibro-osseous tissue. It discusses the pathogenesis, clinical features, diagnosis, and treatment of both monostotic and polyostotic forms of fibrous dysplasia. Radiographic imaging and histopathological features are also summarized. Treatment options include observation, medical therapy with bisphosphonates, surgical remodelling, and in some cases radical resection and reconstruction.

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    25 views37 pages

    Fibrous Dysplasia: DR Bareerah Idrees Resident OMFS 08 May 2021

    Uploaded by

    biaidrees
    This document provides an overview of fibrous dysplasia, a benign bone disorder characterized by the replacement of normal bone with fibro-osseous tissue. It discusses the pathogenesis, clinical features, diagnosis, and treatment of both monostotic and polyostotic forms of fibrous dysplasia. Radiographic imaging and histopathological features are also summarized. Treatment options include observation, medical therapy with bisphosphonates, surgical remodelling, and in some cases radical resection and reconstruction.

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    of 37

    FIBROUS DYSPLASIA

    DR BAREERAH IDREES
    Resident OMFS
    08th May 2021
    OBJECTIVES
    Introduction
    Pathogenesis
    ClinicalFeatures
    Diagnosis
    Radiographic Features
    Histopathological Features
    Treatment and prognosis
    Conclusion
    INTRODUCTION
    Fibrous dysplasia is a benign,
    nonheritable developmental bone disorder
    characterized by the replacement of
    normal bone with haphazardly distributed
    fibro-osseous connective tissue.

    Previously termed as “osteitis fibrosa


    generalisata”
    Pathogenesis

    Idiopathic

    Non hereditary

    Mutation of
    GNAS1 gene
    Molecular Pathogenesis
    Mutated GNAS

    Encodes G-protein

    c-AMP

    Elevtion of c-AMP & stimulation of endocrine receptors

    Activation
    C-fos (proto-oncogene)
    Clinical forms of fibrous dysplasia of the
    jaws
    Monostotic Polyostotic
    Juvenile Craniofacial

    Juvenile, Aggressive McCune-Albright syndrome

    Adult Jaffe Syndrome


    Monostotic Fibrous dysplasia
    70-85% of fibrous Dysplasia, Males and Females are equally
    affected, second-third decade of life

    Occurs most commonly in craniofacial bones, femur, ribs, tibia,

    Painless, Unilateral swelling, bulging of jaw, bone deformity is


    less severe as compared to polyostotic

    Maxilla effected more commonly than mandible in jaw lesions

    Malalignment, tipping or displacement of teeth


    Polyostotic Fibrous Dysplasia
    About 20-30% of fibrous Dysplasia, female predilaction, occurs
    in early age (diagnosed before 10 years)

    Pain, pathological Fracture, limping, leg length discrepency,


    bowing deformity, facial assymetry, vision changes, hearing
    impairement, sinonasal congestion, airway obstruction.

    Expansion and deformity of jaws, eruption pattern is disturbed,


    intraoral pigmentation

    Sites: Femur, tibia, pelvis, ribs, skull and facial bones, upper
    extrimites, lumbar spine & clavicle.
    Jaffe-
    Lichtenstein
    Syndrome
    • Polyostotic FD
    • Café au lait
    pigmentation
    McCune- Mazabraud
    Albright Syndrome
    Syndrome
    • Fibrous dysplasia
    • Polyostotic FD
    • Café au lait
    • Intramuscular
    pigmentation myxomas
    • Multiple
    endocrinopathies
    • Sexual precocity
    Craniofacial Fibrous Dysplasia
    In 10-25% patients with monostotic FD
    In 50% patients with polyostotic FD

    Also in isolated craniofacial form. No extracranial lesions present

    Sites: frontal, sphenoid, maxillary, ethmoid bones.

    Extreme prominence of zygomatic process, Facial deformity

    Vestibular dysfunction, tinnitus, hearing loss, Hypertelorism, cranial


    asymmetry, facial deformity, visual impairment, exophthalmos,
    blindness
    Cherubism
    Special variant of Fibrous Dysplasia, Autosomal-Dominant disorder
    Occurs in children, more severe in boys. Usually regress after
    adolescence

    Protruding and broad jaw, effect the jaws bilaterally and


    symetrically

    Dental abnormalities such as agenesis of 2 nd and 3rd molar,


    displacement of teeth, premature exfoliation of primary teeth,
    delayed eruption of permanent teeth

    Radiographically multiilocular cystic expansion of the jaws

    Histologically Collagenous stroma with spindle shaped fibroblasts,


    Lesion usually reveals multinucleated giant cells
    Diagnosis
    Based on following criteria
    Clinical
    Histological
    Radiographic
    Radiographic Imaging
    Plain Radiographs

    Bone scintigraphy

    CT scans

    MRI
    CT Radiographic Features Classification

    Pagetoid or
    Sclerotic Cystic
    Ground Glass
    • 56% cases • 23% cases • 21% cases
    • Alternating • Shows • Single oval
    radiolucency homogenous or round
    and density lesion with
    radiodensity well defined
    margins
    Radiolucent
    (cystic)

    Radiopaque
    (sclerotic)

    Ground
    glass
    Rind sign
    Histopathological Features
    The Chinese The pagetoid The hypercellular
    letters model model
    • Thin, • Similar to bone • Discontinuous
    disconnected found in Paget’s bone trabeculae
    bone trabeculae disease distributed in
    • Active osseus • Dense and ordered and
    resorption by sclerotic parallel fashion
    osteoclast trabecullar • Honeycomb
    • Star shaped tissue appearence
    osteogenic cells
    • Plenty of
    sharpey fibres
    Differential Diagnosis
    Ossifyin Periapica
    g l cement-
    fibroma osseus

    Florid
    Focal cement-
    hyperparathyr cement-
    osseus
    -oidism osseus
    dysplasi
    dysplasia
    a

    Paget’s Osteo-
    Chronic
    sarcoma
    disease osteomyelitis
    Management
    Recommended treatment options can be
    divided into 4 categories:
    1. Observation.
    2. Medical therapy.
    3. Surgical remodelling.
    4. Radical resection and reconstruction.
    Medical Therapy
    No therapy to cure or halt the disease process
    Bisphosphonates
    Pamidronate (osteoclast inhibitor)
    Given 60gm/day on 3 consecutive days every 6
    months for 18 months
    Decrease bone pain, reduce bone turnover,
    prevent bone resorption
    Minimize chance of recurrence
    Calcitonin (vitamin D and calcium supplements)
    SURGICAL
    PROCEDURE
    Surgical Remodelling
    Conservative procedure
    Aim is to achieve reasonably acceptable
    esthetics
    15-20% chances of recurrence specially
    in growth phase
    Careful assessment of the disease
    Radical resection and reconstruction
    Malignant transformation
     0.4% - 1%
     Osteosarcoma
     Fibrosarcoma
     Chondrosarcoma
     28% - seen in radiation
    (Radiotherapy is contraindicated)
    PROGNOSIS

    Although bad outcomes are more frequent


    among young patients or with polyostotic
    forms.
    References

     Shafer’s textbook of oral pathology 7th Edition

    Contemporary Oral and Maxillofacial Pathology Textbook by J. Philip Sapp


    and Lewis R Eversole

    Maxillofacialsurgery by  Peter Ward-Booth , By (author)  


    Stephen A. Schendel , By (author)  Jarg-Erich Hausamen

    Neville, Damm, Allen, Bouquot. Oral & maxillofacial pathology ,1st


    south asia edition

    Fibrous Dysplasia. Evaluation, and Treatment www.jbjs.org


     THANK YOU

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