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
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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
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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
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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
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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
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
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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
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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
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
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21. No significant change in serum
calcium/phosphorus
Elevated Alkaline phosphatase
Moderate increase in Basal
Metabolic Rate
LAB FINDING
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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
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24. 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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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
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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
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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
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30.
Usually the prognosis is
good although the bad
outcomes occur more
frequently among young
patients or those with
polyostotic forms .
PROGNOSIS
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31.
Special Forms Of Fibrous Dysplasia
Leonetiasis
ossea
Cherubism
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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
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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 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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