fibroosseous lesions. -.pptx

fibroosseous lesions

1. FIBRO OSSEOUS LESIONS

2. Introduction The term fibro-osseous lesion (FOL) is a generic designation of a group of jaw disorders” characterized by the replacement of bone by a benign connective tissue matrix. (D.S Macdonald Jankowski, 2004) CONTENTS Introduction Classification Fibrous dysplasia Etiology and pathophysiology Epidemiological data Clinical presentation Monostotic type Polyostotic type Radiographic feature Histopathology Differential diagnosis Treatment

3. CLASSIFICATION   I CLASSIFICATION OF FIBRO-OSSEOUS LESIONS   Fibrous Dysplasia (FD) Reactive lesions in the tooth bearing areas Periapical cemento -osseous dysplasia Focal cemento -osseous dysplasia Florid cemento -osseous dysplasia   Fibro-osseous neoplasms Cementifying fibroma , ossifying fibroma or cemento -ossifying fibroma   ( REF: Charles A Waldron, 1985 )  

4. Benign fibro-osseous lesions of the craniofacial complex   1. Bone dysplasias a. Fibrous dysplasia Monostotic Polyostotic Polyostotic with endocrinopathy (McCune-Albright) Osteofibrous dysplasia b. Osteitis deformans c. Pagetoid heritable bone dysplasias of childhood d. Segmental odontomaxillary dysplasia   2. Cemento -osseous dysplasias a. Focal cemento -osseous dysplasia b. Florid cemento -osseous dysplasia ( Roy Eversole , 2008 )   3 . Inflammatory/reactive processes a. Focal sclerosing osteomyelitis b. Diffuse sclerosing osteomyelitis c. Proliferative periostitis 4. Metabolic Disease : hyperparathyroidism  5. Neoplastic lesions (Ossifying fibromas ) a. Ossifying fibroma NOS b. Hyperparathyroidism jaw lesion syndrome c. Juvenile ossifying fibroma Trabecular type Psammomatoid type d. Gigantiform cementomas  

5. FIBROUS DYSPLASIA Central lesions of bone which exhibit general histologic features of fibrosis with varying degrees of simultaneous resorption and repair have been reported for many years under uninformative terms Term ~ by Lichtenstein in 1938 Skeletal developmental anomaly of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation

6. Etiology :

7. Mutation Undiff . stem cells~ early embryologic life Osteoblasts , melanocytes , & endocrine cells ~ carry mutation C/F’s ~ multiple bone lesions, cut. pigmentation & endcrn . disturbances Mutation ~ post natal life, Confined to one site, resulting in FD affecting single bone

8. CLINICAL PRESENTATION Bone Pain Localized pain associated with fractures in high-stress areas in dysplastic bone. Deformity Unlike deformities in monostotic disease, deformities in patients with polyostotic disease may continue to progress after skeletal maturity. Shepherd’s crook deformity of the proximal part of the femur (PFD) CONTENTS Introduction Classification Fibrous dysplasia Etiology and pathophysiology Epidemiological data Clinical presentation Monostotic type Polyostotic type Radiographic feature Histopathology Differential diagnosis Treatment

9. Structural deformation, with both expansion and weakness of the bone, can be caused by fibrous dysplasia Fatigue Fracture common in areas of stress concentration within dysplastic lesions CONTENTS Introduction Classification Fibrous dysplasia Etiology and pathophysiology Epidemiological data Clinical presentation Monostotic type Polyostotic type Radiographic feature Histopathology Differential diagnosis Treatment

10. MONOSTOTIC FD OF JAWS : Disease limited to single bone 80% to 85% of all cases, Jaws ~ most common Late 1 st & early 2 nd decades Slow growing painless osseous expansion with facial asymmetry Maxilla > mandible CONTENTS Introduction Classification Fibrous dysplasia Etiology and pathophysiology Epidemiological data Clinical presentation Monostotic type Polyostotic type Radiographic feature Histopathology Differential diagnosis Treatment

11. R/F ~ vary Early onset ~ R/L later calcify > ‘‘ ground glass’’ or mottled mixed pattern Critical to diagnosis ~ fails to manifest any discrete margins Mandibular involve. ~ expansion of lingual & buccal plates, bulging of lower border Buccal expansion >lingual , does not displace the alveolar canal

12. Narrowing ~ Pdl space , ill-defined lamina dura Maxilla: lesion displaces sinus floor superiorly, obliterates sinus

13. Polyostotic fibrous dysplasia Craniofacial FD 2 or more bones, few to 75 % of entire skeleton 20-30 % of cases Pain, hockey stick deformity, pathologic fracture With cafe au lait pigmentation ~ Jaffe-Lichtenstein syndrome With cafe au lait pigmentation & multiple endocrinopathies ~ McCune-Albright syndrome

14. Cafe au lait : well-defined, unilateral tan macules, trunk & thighs Oral mucosal macule also present, Macule margins irregular, resembling a map of coastline of Maine (neurofibromatosis has smooth borders like coast of California)

15. OSTEITIS DEFORMANS/ Paget disease of bone Disease characterised by excessive and abnormal remodelling of bone, is a common disorder in middle aged & elderly Involved bone warm to touch.

16. Phases Lytic Mixed lytic and blastic Sclerotic or burned out

17. Clinical and Radiographic Features : Late adult onset M ~ F Rapid turnover of bone ~ osseous expansion, skeletal deformities Tubular bones ~ bowing, spinal curvature ~ vertebral collapse in later stages Resulting in Simian stance (monkey like) All bones of craniofacial complex ~ affected to varying degrees Cranial nerve neuropathies because of foramina narrowing ~ deafness O/M Maxilla > Mandible, 2.3:1, alv . Ridge ~ widened, Palate ~ flattened Leontiasis ossea : lion like facial deformity Teeth ~ loose, migrate, spacing Prominent dental finding ~ hypercementosis , on PM & M teeth Mouth remains open Edentulous pt.s ~ appliances remade

18. In early stages of disease, radiolucent ‘‘coin shaped’’ lesions appear in flat bones of skull ~ osteitis circumscripta With progression, sclerosis is seen radiographically yielding the so-called ‘‘ cotton wool ’’ appearance

19. CHERUBISM rare inherited (AD) (autosomal dominant) limited to jaws, fullness like cherub develops in early childhood

20. Clinical features Starts after the age of 3yrs and growth starts to decrease when patient reaches 5yrs of age and stops at 12-15yrs of age Sex: Males > Females In case of maxillary swelling the pressure on the floor of orbit may result in upward looking pupil which is refereed to as  “heaven ward look”, Eyes towards heaven look,   the maxillary arch achieves a V shaped appearance bilateral involvement of mandible. Cherubic look: chubby cheeks

21. The main diagnostic features are related to Dental problems - Premature exfoliation of  deciduous teeth Abnormal growth of permanent teeth due to displacement by cysts and lesions Absence of 2nd and 3rd mandibular molars Increased cheek fullness, expansion and widening of alveolar ridge, flattening of the palatal vault. Bilaterally symmetrical enlargement of the jaw

22. Grading of Cherubism: Grade I: Involvement of both mandibular ascending ramus. Grade II: Involvement of both maxillary tuberosities and the mandibular ascending ramus. Grade III: Involvement of whole maxilla and mandible.

23. Radiologic Features bilateral radiolucent cyst-like lesions begins in third molar- ramus area extends posteriorly and anteriorly occasionally into condyle maxilla usually after mandible causes enlargement of jaws cortex remains intact encroach upon maxillary sinuses displacment of developing teeth early exfoliation of deciduous teeth Floating tooth

24. Cherubism

25. CEMENTO-OSSEOUS DYSPLASIAS (OSSEOUS DYSPLASIA) Most common fibro-osseous lesion Based on the clinical and radiographic features, it is separated into 3 groups: (1) focal, (2) periapical , and (3) florid Occurs in tooth bearing areas of jaws Arises in close proximity to PdL , H/F similarities with structure, some suggest ~ PdL origin Others believe ~ defect in extraligamentary bone remodeling , triggered by local factors & possibly correlated to an underlying hormonal imbalance

26. Clinical and Radiographic Features Focal cemento -osseous dysplasia: Single site of involvement Sex: 90% cases occur in females Age: mean age of 38 (3 rd to 6 th decade) Race: whites Site: posterior mandible Lesions occur in dentulous & edentulous areas, with many examples noted in extraction sites S/S: typically asymptomatic R/F: smaller than 1.5 cm, lesion varies from completely R/L to densely R/O with a thin peripheral R/L rim. Most commonly, however, there is a mixed R/L / R/O pattern, well defined with irregular borders

27. Periapical cemento -osseous dysplasia (Osseous dys­plasia; Cemental dysplasia; Cementomas ) Periapical region of the anterior mandible Solitary lesions may occur, but multiple foci are present more frequently Sex: female patients (ranging from 10:1 to 14:1) Race: 70% of cases affect blacks Age: 30 -50 Years Teeth associated: are almost invariably vital & seldom have restorations S/S: asymptomatic condition

28. R/F ~ Early lesions ~ cir­cumscribed R/L at apical area of a tooth (D/D: periapical granuloma or periapical cyst) With time, adj. lesions fuse to form linear pattern of R/L that envelopes apices of several teeth Lesion "mature' over time ~ mixed Rl –Ro appearance End stage, circumscribed dense calcification surrounded by narrow Rl rim PdL intact, fusion to tooth not seen Lesion ~ self-limiting & doesn’t typically expand the cortex

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