NON HERIDETRY BONE PATHOLOGY. COMMONLY SEEN IN PELVIC BONE BUT IN ORAL CAVITY IT AFFECTS MANDIBULAR JAWS MORE.

1. FIBROUS DYSPLASIA
KRITI N. JAIN
FINAL YEAR BDS
TEERTHANKAR MAHAVEER DENTAL
COLLEGE AND RESEARCH CENTRE
1

2. INTRODUCTION
EVERSOLE 2008
CLASSIFICATION
FIBROUS DYSPLASIA 2
• •“Replacement of normal bone by tissue
composed of collagen fibers and fibroblasts,
containing varying amount of mineralized
substance” – Waldron 1985.
• •“A collection of non – neoplastic
intraosseous lesions that replace bone and
consists of a cellular fibrous connective
tissue within which non- functional osseous
structures form” Eversole
1. Fibrous Dysplasia :
a) Monostotic Fibrous Dysplasia
b) Polyostotic Fibrous Dysplasia
2. Cement-Osseous Dysplasia
a. Periapical Cement-Osseous Dysplasia
b. Focal Cement-Osseous Dysplasia c. Florid Cement-
Osseous Dysplasia
3. Fibro-Osseous Neoplasm :
a. Cementifying Fibroma, Ossifying Fibroma, Cement-
Ossifying Fibroma

3. • Introduction
• Etiology
• Clinical Features
• Monostotic Form
• Polyostotic Form
• Craniofacial form
• Oral Manifestations
• Histologic Features
• Radiographic Features
• Differential Diagnosis
• Treatment &Prognosis
• Conclusion
• References
FIBROUS DYSPLASIA 3
20XX

4. FIBROUS
DYSPLASIA
(Fibrocystic Disease, Osteitis Fibrosa
Localisata, Focal Osteitis Fibrosa,
Fibroosteodystrophy, Jaffé-lichtenstein)
“A benign lesion, presumably
developmental in nature,
characterized by the presence of
fibrous connective tissue with a
characteristic whorled pattern and
containing trabeculae of immature
non lamellar bone.”
Waldron 1985.
FIBROUS DYSPLASIA 4
DEFINITION

5. ETIOLOGY & PATHOGENESIS
FIBROUS DYSPLASIA
20XX
1 • IDIOPATHIC
2
• NON HEREDITARY
3
• CAUSED BY MUTATION IN GNAS1 GENE on
chromosome 20q13.32
5

6. MOLECULAR PATHOGENESIS
C-fos (proto-oncogene)
Elevtion of c-AMP & stimulation of endocrine receptors
•Activation
c-AMP
Mutated GNAS
Encodes G-protein Activation
FIBROUS DYSPLASIA 6

7. FIBROUS DYSPLASIA 7

8. Mutated osteoblasts
Over expression IL-6
Stimulate osteoclastic activity
Bone Lesion Expansion
FIBROUS DYSPLASIA 8

9. Monostotic form
FIBROUS DYSPLASIA 9
Polyostotic form Craniofacial form

10. •70%-80% of fibrous dysplasia.
•Occurs in rib, femur , tibia, craniofacial bones and humerus.
•Pain or pathologic fracture
•Bone deformity less severe Painless swelling of the jaw.
•Swelling involves labial or buccal plate Protuberance - inferior border
of mandible
•Monostotic-asymptomatic -20-30 years
FIBROUS DYSPLASIA 10

11. •20%-30% of fibrous dysplasia
•Sites: Femur, tibia, pelvis, ribs, skull and facial bones, upper extrimites,
lumbar spine & clavicle.
•Tends to occur in unilateral distribution.
•Involvement asymmetric and generalized on bilateral lesions.
• Equal in males & females.
• Commonly-3-15yrs
• Polyostotic-asymptomatic before 10 years.
FIBROUS DYSPLASIA 11

12. JAFFE-LICHTENSTEIN
SYNDROME:-:
Involves multiple bones,
accompanied by pigmented lesions
of the skin or café au lait spots.
Found in children usually less than 10
years of age.
Lesion usually become static when
skeletal growth stops.
Expansion and deformity of the jaws.
Euption pattern of the teeth is
disturbed because of loss of support of
the developing teeth.
Asymmetry of the facial bones, with
ballooning of the jaws which results in
gross enlargement and deformity
MCCUNE-ALBRIGHT
SYNDROME:-
• usually found in females and in
addition shows endocrinal
disturbances like precocious
puberty, goiter, hyperthyroidism,
Cushing's syndrome and
acromegaly.
• Secondary sexual characteristics
are evident by the age of 5 years.
• Bone and skin lesions are usually
unilateral, long bones are
frequently affected
• asymmetry of the facial bones,
with ballooning of the jaws which
results in gross enlargement and
deformity.
Mazabraud
syndrome:-:
• Here the dysplasia extends to
involve the
• maxillary sinus, zygomatic
process, floor of the orbit and
sometimes the base of the
skull.
• It results in severe facial
deformity and malocclusion.
• The lesions may produce
anosmia, deafness and
blindness, with proptosis of
the affected eye.
FIBROUS DYSPLASIA 12

13. Pain in involved
limb
Spontaneous
fracture
Structural
integrity weak
Bowing of weight
bearing bones
curvature of
femoral neck
Shepherd’s crook
deformity
FIBROUS DYSPLASIA 13

14. •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.
•Extreme prominence of zygomatic process, facial deformity
•Vestibular dysfunction, tinnitus, hearing loss, Hypertelorism, cranial
asymmetry, facial deformity, visual impairment, exophthalmos, blindness
•When the maxilla and associated bones are affected, the patient gives
the typical leonine appearance (leontiasis ossea).
FIBROUS DYSPLASIA 14

15. Monostotic:
• Sites: Maxilla>mandible
• Appearance:unilateral facial
swelling
• Symptoms: Swelling is usually
painless
• Cortical plates: Enlarging
deformities of alveolar
process
• Mandible:protuberant
excrescence of the inferior
border of mandible
• Teeth: The teeth present in
the affected area are either
malaligned and tipped or
displaced
Craniofacial
• Sites: extending to involve the
maxillary sinus, the zygomatic
process, floor of orbit and
sometimes, it extends toward
the base of the skull
• Symptoms: It results in severe
malocclusion and marked
facial deformity.
• Craniofacial lesions may lead
to anosmia (loss of sense of
smell), deafness and blindness
• Signs: There may be proptosis
of the affected eye.
Polyostotic
• Appearance: Expansion and
deformities of jaws.
• There is ballooning of jaws, so
there is gross enlargement
and deformity
• Teeth: The eruption pattern of
teeth is disturbed because of
loss of support of the
developing teeth
• Pigmentation: In some cases,
intraoral pigmentation can be
seen.
FIBROUS DYSPLASIA 15

16. HISTOLOGIC FEATURES
• Proliferating fibroblasts in a compact stroma of
interlacing collagen fibres.
• Irregular bony trabeculae scattered throughout
lesion.
• Chinese character shaped.
• Trabeculae usually coarse woven bone
• Lesions rich in spindle shaped fibroblasts with a
swirled appearance within the marrow space
• Lesional bone fuses directly with normal bone at the
periphery
FIBROUS DYSPLASIA 16

17. LAB FINDINGS
• No significant change in serum
calcium/phosphorusIrregular bony
trabeculae scattered throughout lesion.
• Elevated Alkaline phosphatase.
• Moderate increase in Basal Metabolic
Rates
FIBROUS DYSPLASIA 17

18. RADIOGRAPHIC FEATURES
• Imaging Features:
 Location: maxilla almost twice as often as the mandible.
Lesions more commonly are unilateral.
 Periphery: The periphery of fibrous dysplasia lesions most commonly is ill defined,
boundary between normal bone and the lesion can appear sharp and even corticated,
especially in young lesions.
 Internal Structure: density and trabecular pattern variation is more pronounced
in the mandible and more homogeneous in the maxilla. The internal density is more
radiopaque in the maxilla and the base of the skull.Various radiopaque pattern appear
are:
*granular appearance (or ground-glass appearance)
*resembling the small fragments of a shattered windshield)
*orange (peau d’orange)
*wispy arrangement (cotton wool)
 Effects on Surrounding Structure:- Effects on the involved bone may include
expansion with maintenance of a thinned-outer cortex.
FIBROUS DYSPLASIA 18

19. Café-au-lait pigmentation
Fibrous Dysplasia:
café au lait pigmentation consists
of well-defined, generally unilateral
tan macules on the trunk and thighs.
The margins of the
café au lait spots are typically very
irregular
Neurofibromatosisi:
Six or more cafe au lait macules highly
suggestive of NF1.
This is in contrast to the café au lait
spots of neurofibromatosis which have
smooth borders .
FIBROUS DYSPLASIA 19

20. FIBROUS DYSPLASIA 20
Lesions likely to be confused with Lesions likely to be confused with Lesions likely to be confused with
the osteolytic stage of fibrous dysplasia : the mottled stage of fibrous dysplasia : the mature stage of fibrous dysplasia :
*Central giant cell granuloma * Lymphoma of the bone * Paget’s disease
*Chondrosarcoma * Giant cell lesion of hyperparathyroidism
*Traumatic bone cyst *Osteoblastic
metastatic carcinoma
*Dental cyst *Osteosarcoma
*Aneurysmal bone cyst *Periapical cemental dysplasia
*Chronic osteitis * Cementifying and ossifying fibroma
*Chronic osteomyelitis * Chronic osteomyelitis
*Peripheral and central : * Paget’s disease
squamous cell carcinoma
*Metastatic tumor:
*Reticular cell sarcoma, Ewing’s sarcoma

21. PROGNOSIS
• Prognosis-good
• Although-bad outcomes-
more frequently among
young patients or with
polyostotic forms
• Malignant transformation
Malignant
transformation
• 0.4% - 4%
• Osteosarcoma
• Fibrosarcoma
• Chondrosarcoma
• 28% - seen in radiated-
Radiotherapy
contraindicated
Treatment
• Conservative treatment-
prevent deformity.
Management requires a
multidisciplinary approach –
polyostotic
• Bisphosphonate therapy may
help to improve function,
decrease pain, & lower
fracture risk -some patients.
• Surgery-complete resection,
restoring function &improving
facial aesthetics
FIBROUS DYSPLASIA 21

22. CONCLUSION
• Fibrous dysplasia is a lesion of bone
commonly affecting the younger age
group. It shows similarities with other
fibro osseous lesions clinically,
radiological & histopathologically.
• Hence through knowledge about these
lesions is necessary for proper diagnosis
& treatment plan.
FIBROUS DYSPLASIA 22

23. THANK YOU!
KRITI N JAIN
REFERENCES:
1.WHITE AND PHAROH- ORAL
RADIOLOGY Principles and Interpretation, 7th edition
2. Essentials of Oral and Maxillofacial Radiology, 2nd edition
3. Textbook of Oral Medicine,3rd edition
4. Shafer's Textbook of ORAL PATHOLOGY,8th edition
5.Oral and Maxillofacial Pathology, 3rd edition
6. Burket’sORAL MEDICINE ,12th edition
23
FIBROUS DYSPLASIA