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FIBROUS DYSPLASIA

PRESENTED BYDr. JAMEELA IMTHYAS

VINAYAKA MISSION’S SANKARACHARYAR DENTAL COLLEGE

SALEM

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IntroductionClassificationFibrous dysplasia-DefinitionEtiologyClinical FeaturesMonostotic FormPolyostotic FormHistologic FeaturesRadiographic FeaturesTreatment &PrognosisOther forms of fibrous dysplasiaConclusion

CONTENTS

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BONE

• BONE is one third connective tissue.

• The inorganic calcium salts make it hard & rigid -avoid resistance to compressive forces

• The organic connective tissue ,collagen fibres make it resilient- afford resistance to tensile forces.

INTRODUCTION

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Fibro-Osseous Lesion

• Fibro-Osseous Lesion refer to a diverse process in which the normal bone architecture is replaced by fibroblast and collagen fibres containing variable amounts of mineralized material.

INTRODUCTION

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CLASSIFICATIONFIBRO-OSSEOUS LESIONS OF JAWS

Fibrous dysplasia• Monostotic• Polyostotic

Reactive(dysplastic) lesions periodontal ligament origin• Periapical cemento-

osseous dysplasia• Focal cemento-

osseous dysplasia• Florid cemento-

osseous dysplasia

Fibro-osseous neoplasms• Cemento

ossifying fibroma

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DEFINITION

Fibrous Dysplasia is a skeletal developmental anomaly of the bone–forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation.

It is a nonhereditory disorder of unknown cause.

FIBROUS DYSPLASIA

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Idiopathic

Non hereditary

Caused by mutation in GNAS1 gene

ETIOLOGY & PATHOGENESIS

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-GNAS1 gene encodes a G-protien-Stimulates production of cAMP-Continuous activation of the G-protien

-Overproduction of cAMP in affected tissues-Hyperfunction of affected endocrine organs

-Increased proliferation of melanocytes-Results in large café-au-lait spots.-cAMP affect on differentiation of osteoblasts

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CLINICAL FEATURES

Monostoyic form

Polyostotic form

Craniofacial form

Three disease patterns are recognized

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Fibrous dysplasia of

maxilla Mc-Cune Albright syndrome

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Incidence equal in males & females.Commonly found in persons aged 3-15yrs

Polyostotic disease persons asymptomatic before 10 years .

Monostotic disease persons asymptomatic as old as 20-30 years .

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Craniofacial Fibrous

dysplasia

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70%-80% of fibrous dysplasia.Occurs in rib, femur , tibia, craniofacial bones and humerus

Pain or pathologic fracture in 10-70yrsBone deformity less severePainless swelling of the jaw

Swelling involves labial or buccal plate Protuberant excrescence of inferior border of mandible

MONOSTOTIC FORM

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MalalignmentTipping

Displacement

Intact over lesion

Maxillary sinus ,zygomatic processFloor of orbit,extend to

base of skull

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20%-30% of fibrous dysplasia.

Sites: Femur, tibia, pelvis, ribs, skull and facial bones,upper extrimites,lumbar spine,clavicle and cervical spine .

Tends to occur in unilateral distribution.Involvement asymmetric and generalized on bilateral lesions.

POLYOSTOTIC FORM

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Pain in involved limb Spontaneous

fracture

Structural integrity weak

Bowing of weight bearing bones,

curvature of femoral

neck,proximal shaft increase

Shepherd’s crook

deformity

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• Involves variable number of bones , accompanied by pigmented lesions of skin or café-au-lait spots

Jaffe Lichenstien syndrome

• Involves nearly all bones in skeleton, pigmented lesions of skin or café-au-lait spots and endocrine disturbances

Mc-Cune Albright

syndrome• Hyperthyroidsm,

hyperparathyroidsm, cushing syndrome,gonadotrophin-Mc-Cune Albright syndrome

Endocrine disturbance

s assc.

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Café-au-lait pigmentation

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CAFE-AU-LAIT

SPOTS• Increased

melanin in basal cells of epidermis

• Cutaneous pigmentation seen ipsilateral to side of bone lesion

• Occur at birth precedes skeletal& endocrine diseases.

MAZABRAUDS SYNDROME

• Fibrous dysplasia and intramuscular myxoma, risk of sarcomatous malformation

MALIGNANCIES

• Osteosarco Mc-Cune albright syndrome

• Chonddrosarco Mc-Cune albright syndrome

• FibrosarcoMc-Cune albright syndrome

• LiposarcoMc-Cune albright syndrome

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No significant change in serum calcium/phosphorus

Elevated Alkaline phosphatase

Moderate increase in Basal Metabolic Rate

LAB FINDING

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In 10-25% of pt. with monostotic form.In 50% of pt. with polyostotic form.

Also in isolated craniofacial form.No extracranial lesions present.

Sites:frontal,sphenoid,maxillary,ethmoid bones.

Hypertelorism,cranial asymmetry,facial deformity,visual impairment,exophthalmos,blindness

Vestibular dysfunction, tinnitus, hearing loss.

Craniofacial Fibrous Dysplasia

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Microscopic low power photograph of fibrous dysplasia

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High power photograph of fibrous dysplasia shows woven bone produced by fibrous tissue.no osteoblasts lininig bone

High power photograph of fibrous dysplasia shows woven bone produced by fibrous tissue.no osteoblasts lininig bone

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HISTOLOGIC FEATURES

Monostotic Proliferating fibroblasts in

a compact stroma of interlacing collagen fibres.

Irregular bony trabeculae scattered throuout lesion .

No definite pattern. C-shaped or Chinese

character shaped . Trabeculae usually

coarse woven bone

Lesions rich in spindle shaped fibroblasts with a swirled appearance within the marrow space

Islands of cartilaginous tissue within lesions

Affected bones may have cystic lesions lined by multinucleated giant cells

Polyostotic

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Network of fine bone trabeculae

Increased trabeculation – lesion more opaque & mottled appearance

Opaque with many delicate trabeculae :’ground –glass’ ‘or ‘peau d’ orange’ appearance

Cortical bone becomes thinned

Roots of teeth separated or moved out of normal position

RADIOGRAPHIC FEATURES

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Orange peel appearance of fine dense trabeculae

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Ossifying Fibroma

Pagets disease

Osteosarcoma

Cherubism

Hypeparathyroidsm

Differential Diagnosis

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Conservative treatment to prevent deformity. Management requires a multidisciplinary approach in polyostotic.

Bisphosphonate therapy may help to improve function, decrease pain, and lower fracture risk in some patients.

Surgery indicated for confirmatory biopsy, correction of deformity, prevention of pathologic fracture, eradication of symptomatic lesions.

TREATMENT

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Usually the prognosis is good although the badoutcomes occur more frequently among young patients or those with polyostotic forms .

PROGNOSIS

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Special Forms Of Fibrous Dysplasia

Leonetiasis ossea

Cherubism

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Asymptomatic cases of fibrous dysplasia conservative management appropriate

Advances in modern surgical techniques and imaging technologies

Allow symptomatic cases to be treated reliably & efficiently

With complete resection, restoring function &improving facial aesthetics

CONCLUSION

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Reference Books• Shafer’s textbook of oral pathology 7th

Edition• Burkitt’s oral medicine,11th edition.• Neville, Damm, Allen, Bouquot. Oral &

maxillofacial pathology ,3rd edition• Lucas Pathology Of Tumours Of The Oral

Tissues• Color Atlas Of Clinical Oral Pathology 2nd

edition Neville• B.D.Chaurassia General Handbook Of

Anatomy• Fibrous Dysplasia. Pathophysiology,

Evaluation, and Treatment www.jbjs.org• Fibrous Dysplasia In the Maxillomandibular

region– Journal of IMAB - Annual Proceeding (Scientific Papers) vol. 16, book 4, 2010

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