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PRESENTED BY
Dr. JAMEELA IMTHYAS
VINAYAKA MISSION’S SANKARACHARYAR
DENTAL COLLEGE
SALEM
JAMEELA
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

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

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

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

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

JAMEELA

Idiopathic
Non hereditary
Caused by mutation in GNAS1 gene
ETIOLOGY &
PATHOGENESIS
JAMEELA
-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

CLINICAL FEATURES
Monostoyic form
Polyostotic form
Craniofacial form
Three disease patterns are recognized
JAMEELA

Fibrous dysplasia of maxilla Mc-Cune Albright syndrome
JAMEELA
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
Craniofacial
Fibrous dysplasia
JAMEELA
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
Malalignment
Tipping
Displacement
Intact over lesion
Maxillary sinus ,zygomatic
process
Floor of orbit,extend to base
of skull
JAMEELA
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

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

•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
Café-au-lait pigmentation
JAMEELA

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
No significant change in serum
calcium/phosphorus
Elevated Alkaline phosphatase
Moderate increase in Basal
Metabolic Rate
LAB FINDING
JAMEELA
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
Microscopic low power photograph
of fibrous dysplasia
JAMEELA
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
JAMEELA

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

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
Orange peel appearance of fine dense trabeculae
JAMEELA

Ossifying Fibroma
Pagets disease
Osteosarcoma
Cherubism
Hypeparathyroidsm
Differential Diagnosis
JAMEELA
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

Usually the prognosis is
good although the bad
outcomes occur more
frequently among young
patients or those with
polyostotic forms .
PROGNOSIS
JAMEELA

Special Forms Of Fibrous Dysplasia
Leonetiasis
ossea
Cherubism
JAMEELA
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

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
JAMEELA
THANK YOU
JAMEELA

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FIBROUS DYSPLASIA OF JAW

  • 1. PRESENTED BY Dr. JAMEELA IMTHYAS VINAYAKA MISSION’S SANKARACHARYAR DENTAL COLLEGE SALEM JAMEELA
  • 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.  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.  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.  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.  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
  • 8.  Idiopathic Non hereditary Caused by mutation in GNAS1 gene ETIOLOGY & PATHOGENESIS JAMEELA
  • 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.  CLINICAL FEATURES Monostoyic form Polyostotic form Craniofacial form Three disease patterns are recognized JAMEELA
  • 11.  Fibrous dysplasia of maxilla Mc-Cune Albright syndrome JAMEELA
  • 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 JAMEELA
  • 15. Malalignment Tipping Displacement Intact over lesion Maxillary sinus ,zygomatic process Floor of orbit,extend to base of skull JAMEELA
  • 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.  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.  •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 JAMEELA
  • 21. No significant change in serum calcium/phosphorus Elevated Alkaline phosphatase Moderate increase in Basal Metabolic Rate LAB FINDING JAMEELA
  • 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. Microscopic low power photograph of fibrous dysplasia JAMEELA
  • 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 JAMEELA
  • 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.  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. Orange peel appearance of fine dense trabeculae JAMEELA
  • 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.  Usually the prognosis is good although the bad outcomes occur more frequently among young patients or those with polyostotic forms . PROGNOSIS JAMEELA
  • 31.  Special Forms Of Fibrous Dysplasia Leonetiasis ossea Cherubism JAMEELA
  • 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.  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 JAMEELA