FIBROUS DYSPLASIA

DR BAREERAH IDREES
Resident OMFS
08th May 2021
OBJECTIVES
Introduction
Pathogenesis
ClinicalFeatures
Diagnosis
Radiographic Features
Histopathological Features
Treatment and prognosis
Conclusion
INTRODUCTION
Fibrous dysplasia is a benign,
nonheritable developmental bone disorder
characterized by the replacement of
normal bone with haphazardly distributed
fibro-osseous connective tissue.

Previously termed as “osteitis fibrosa

generalisata”
Pathogenesis

Idiopathic

Non hereditary

Mutation of
GNAS1 gene
Molecular Pathogenesis
Mutated GNAS

Encodes G-protein

c-AMP

Elevtion of c-AMP & stimulation of endocrine receptors

Activation
C-fos (proto-oncogene)
Clinical forms of fibrous dysplasia of the
jaws
Monostotic Polyostotic
Juvenile Craniofacial

Juvenile, Aggressive McCune-Albright syndrome

Adult Jaffe Syndrome

Monostotic Fibrous dysplasia
70-85% of fibrous Dysplasia, Males and Females are equally
affected, second-third decade of life

Occurs most commonly in craniofacial bones, femur, ribs, tibia,

Painless, Unilateral swelling, bulging of jaw, bone deformity is

less severe as compared to polyostotic

Maxilla effected more commonly than mandible in jaw lesions

Malalignment, tipping or displacement of teeth

 Polyostotic Fibrous Dysplasia
About 20-30% of fibrous Dysplasia, female predilaction, occurs
in early age (diagnosed before 10 years)

Pain, pathological Fracture, limping, leg length discrepency,

bowing deformity, facial assymetry, vision changes, hearing
impairement, sinonasal congestion, airway obstruction.

Expansion and deformity of jaws, eruption pattern is disturbed,

intraoral pigmentation

Sites: Femur, tibia, pelvis, ribs, skull and facial bones, upper
extrimites, lumbar spine & clavicle.
Jaffe-
Lichtenstein
Syndrome
• Polyostotic FD
• Café au lait
pigmentation
McCune- Mazabraud
Albright Syndrome
Syndrome
• Fibrous dysplasia
• Polyostotic FD
• Café au lait
• Intramuscular
pigmentation myxomas
• Multiple
endocrinopathies
• Sexual precocity
Craniofacial Fibrous Dysplasia
In 10-25% patients with monostotic FD
In 50% patients with polyostotic FD

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
Cherubism
Special variant of Fibrous Dysplasia, Autosomal-Dominant disorder
Occurs in children, more severe in boys. Usually regress after
adolescence

Protruding and broad jaw, effect the jaws bilaterally and

symetrically

Dental abnormalities such as agenesis of 2 nd and 3rd molar,

displacement of teeth, premature exfoliation of primary teeth,
delayed eruption of permanent teeth

Radiographically multiilocular cystic expansion of the jaws

Histologically Collagenous stroma with spindle shaped fibroblasts,

Lesion usually reveals multinucleated giant cells
Diagnosis
Based on following criteria
Clinical
Histological
Radiographic
Radiographic Imaging
Plain Radiographs

Bone scintigraphy

CT scans

MRI
CT Radiographic Features Classification

Pagetoid or
Sclerotic Cystic
Ground Glass
• 56% cases • 23% cases • 21% cases
• Alternating • Shows • Single oval
radiolucency homogenous or round
and density lesion with
radiodensity well defined
margins
 Radiolucent
(cystic)

Radiopaque
(sclerotic)

Ground
glass
Rind sign
Histopathological Features
The Chinese The pagetoid The hypercellular
letters model model
• Thin, • Similar to bone • Discontinuous
disconnected found in Paget’s bone trabeculae
bone trabeculae disease distributed in
• Active osseus • Dense and ordered and
resorption by sclerotic parallel fashion
osteoclast trabecullar • Honeycomb
• Star shaped tissue appearence
osteogenic cells
• Plenty of
sharpey fibres
Differential Diagnosis
Ossifyin Periapica
g l cement-
fibroma osseus

Florid
Focal cement-
hyperparathyr cement-
osseus
-oidism osseus
dysplasi
dysplasia
a

Paget’s Osteo-
Chronic
sarcoma
disease osteomyelitis
Management
Recommended treatment options can be
divided into 4 categories:
1. Observation.
2. Medical therapy.
3. Surgical remodelling.
4. Radical resection and reconstruction.
Medical Therapy
No therapy to cure or halt the disease process
Bisphosphonates
Pamidronate (osteoclast inhibitor)
Given 60gm/day on 3 consecutive days every 6
months for 18 months
Decrease bone pain, reduce bone turnover,
prevent bone resorption
Minimize chance of recurrence
Calcitonin (vitamin D and calcium supplements)
 SURGICAL
PROCEDURE
Surgical Remodelling
Conservative procedure
Aim is to achieve reasonably acceptable
esthetics
15-20% chances of recurrence specially
in growth phase
Careful assessment of the disease
Radical resection and reconstruction
Malignant transformation
 0.4% - 1%
 Osteosarcoma
 Fibrosarcoma
 Chondrosarcoma
 28% - seen in radiation
(Radiotherapy is contraindicated)
 PROGNOSIS

Although bad outcomes are more frequent

among young patients or with polyostotic
forms.
References

 Shafer’s textbook of oral pathology 7th Edition

Contemporary Oral and Maxillofacial Pathology Textbook by J. Philip Sapp

and Lewis R Eversole

Maxillofacialsurgery by  Peter Ward-Booth , By (author)  

Stephen A. Schendel , By (author)  Jarg-Erich Hausamen

Neville, Damm, Allen, Bouquot. Oral & maxillofacial pathology ,1st

south asia edition

Fibrous Dysplasia. Evaluation, and Treatment www.jbjs.org

 THANK YOU