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  2. 2. Introduction Classification Fibrous dysplasia-Definition Etiology Clinical Features Monostotic Form Polyostotic Form Histologic Features Radiographic Features Treatment &Prognosis Other forms of fibrous dysplasia Conclusion CONTENTS JAMEELA
  3. 3.  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 JAMEELA
  4. 4.  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 JAMEELA
  5. 5.  CLASSIFICATION FIBRO-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 JAMEELA
  6. 6.  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 JAMEELA
  7. 7.  JAMEELA
  8. 8.  Idiopathic Non hereditary Caused by mutation in GNAS1 gene ETIOLOGY & PATHOGENESIS JAMEELA
  9. 9. -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 JAMEELA
  10. 10.  CLINICAL FEATURES Monostoyic form Polyostotic form Craniofacial form Three disease patterns are recognized JAMEELA
  11. 11.  Fibrous dysplasia of maxilla Mc-Cune Albright syndrome JAMEELA
  12. 12. 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 . JAMEELA
  13. 13. Craniofacial Fibrous dysplasia JAMEELA
  14. 14. 70%-80% of fibrous dysplasia. Occurs in rib, femur , tibia, craniofacial bones and humerus Pain or pathologic fracture in 10-70yrs Bone deformity less severe Painless swelling of the jaw Swelling involves labial or buccal plate Protuberant excrescence of inferior border of mandible MONOSTOTIC FORM JAMEELA
  15. 15. Malalignment Tipping Displacement Intact over lesion Maxillary sinus ,zygomatic process Floor of orbit,extend to base of skull JAMEELA
  16. 16. 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 JAMEELA
  17. 17.  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 JAMEELA
  18. 18.  •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 disturbances assc. JAMEELA
  19. 19. Café-au-lait pigmentation JAMEELA
  20. 20.  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 JAMEELA
  21. 21. No significant change in serum calcium/phosphorus Elevated Alkaline phosphatase Moderate increase in Basal Metabolic Rate LAB FINDING JAMEELA
  22. 22. 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 JAMEELA
  23. 23. Microscopic low power photograph of fibrous dysplasia JAMEELA
  24. 24. High power photograph of fibrous dysplasia shows woven bone produced by fibrous osteoblasts lininig bone High power photograph of fibrous dysplasia shows woven bone produced by fibrous osteoblasts lininig bone JAMEELA
  25. 25.  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 JAMEELA
  26. 26.  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 JAMEELA
  27. 27. Orange peel appearance of fine dense trabeculae JAMEELA
  28. 28.  Ossifying Fibroma Pagets disease Osteosarcoma Cherubism Hypeparathyroidsm Differential Diagnosis JAMEELA
  29. 29. 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 JAMEELA
  30. 30.  Usually the prognosis is good although the bad outcomes occur more frequently among young patients or those with polyostotic forms . PROGNOSIS JAMEELA
  31. 31.  Special Forms Of Fibrous Dysplasia Leonetiasis ossea Cherubism JAMEELA
  32. 32. 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 JAMEELA
  33. 33.  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 • Fibrous Dysplasia In the Maxillomandibular region– Journal of IMAB - Annual Proceeding (Scientific Papers) vol. 16, book 4, 2010 JAMEELA