BONES MAY BE CLASSIFIED ACCORDINGLY BASED ON:

1. Shape –long bones, short bones, flat bones, irregular bones,

sesamoid bones , sutural bones.

2. Mode of development- endochondral bones, intramembranous

bones.

3. By their histological appearance –1. Mature bone

A. Compact bone
b. Cancellous bone
Cancellous
bone
2.Immature (or) cancellous bone

Compact bone
Analysis of intraosseous lesions
 Step -1
Localize the abnormality
Unilateral
localized
bilateral
generalized
Position in the jaws
 Step-2

Asses the peripheries and

shape
1.Borders/ margins/peripheries
(a) well-defined borders
Punched out border
Corticated border
Sclerotic border
Soft tissue capsule
(b) ill-defined borders

Blending border
Invasive border
2. Shape
Radiolucent lesions Radiopaque lesions
Geographic Calcific foci
Moth eaten spherules
Permeative massules
Odontogenic calcifications
Septations
scalloped
appearance
 Step-3
Analyze the internal structure
1.Totally radiolucent - cyst

2.Totally radiopaque - osteomas

3.Mixed radiolucent & radiopaque(mixed density)

- osteoblastic Vs osteoclastic

activity
• Internal structure of mixed lesion

trabacular pattern
septa in between

dystrophic calcifications
 Step – 4
Analyze the effect of the lesion on the surrounding structures
1. Teeth -
floating
displaced
5. IAC
2. lamina dura
Knife edged
6. Root – Spike
Resorption
3. Pdl space –
7. Cortex - Intact visible
Intact invisible
4. Bone - Wide
Expansion
narrow
Saucerization

Trabaculae
 Step-5
Formulate a radiographic interpretation
1. Normal Vs abnormal
2. Developmental Vs acquired
3. Classify
4. Proceed
HISTOLOGY
Disease Of Bones And Joints
Defects in extra cellular structural protein
Osteogenesis imperfecta/ brittle bones
Etiology
Inherited - AD
Abnormality in collagen type I
bones, organ capsules, fascia, cornea, sclera, tendons,
meniges, dermis

Mutations in COL1A1 – 17q21, COL1A2 – 17q22.1

Qualitative as well as Quantitative defects in bone

Clinical features
Extreme fragility and porosity of bone – fracture – heals but
imperfect
Blue sclera- Osteopetrosis, Fetal Rickets, Turner Syndrome, Paget
Disease, Marfan Syndrome, Ehlers Danlos Syndrome
Deafness
Dentinogenesis imperfecta
Laxity of ligaments
Peculiar shape of skull
Abnormal electrical reaction of muscles
Capillary bleeding – unassociated with blood dyscrasia
 Physical features – Sillence Classification

Type I – most common form

A – Dentinogenesis imperfecta absent
B – Dentinogenesis imperfecta present
Blue sclera
In utero fracture 10%, # decreases after puberty,
kyphoscoliosis, hearing loss, bruising, short stature
Type II - bone fragility, in utero # 100%, blue sclera, DI,
stillborn / death at 4wks

Type III – DI, sclera variable, short limb, deformed body,

triangular facies with frontal bossing, 50% utero # &
neonatal period, hearing - unaffected

Type IV –
A – DI absent
B – DI present
# - infancy, mild angulation, shortening of long bones
Oral manifestations
Large head – frontal, temporal Bossing
Maxillary hypoplasia –
Class III malocclusion – cross bite – Ant/Post
Impactions, ectopic teeth
Permanent dentition – unerupted Ist and IInd molars
Dentinogenesis imperfecta
 Radiographic features

Osteopenia
Bowing and angulation of long bones
Multiple #S
 Histological features
Cortical bone – thin cortices
Cancellous bone – delicate trabaculae with microfractures
Osteoblastic activity – retarted / imperfect
Failure of fetal collagen to transform into mature collagen
Progressive intermolecular crosslinkage
with adjacent collagen molecule is absent
Defective microvasular system
Calcification proceeds normally
Treatment and prognosis
No treatment

Prognosis – good –very poor

TYPE IA – similar to normal population
TYPE II – pts die in Ist YR of life
Marfan syndrome - President Abraham Lincoln
Etiology - Spectrum of disorders
Heritable genetic defect in connective tissue autosomal
dominant mode
FBN1 gene 15ql5-q23 - codes for the connective tissue
protein, fibrillin.

Myriad of distinct clinical problems

Musculoskeletal, cardiac and ocular problems
predominate.
 Clinical features
Incidence - 1 in 3,000 to 1 in 10,000 births which includes
stillbirths.
Skeletal deformities –
arachnodactyly, dolichostenomelia (i.e. long limbs relative
to trunk length), and thoracolumbar scoliosis.
Long and narrow face is characteristic.
Hyperextensibility of joints with habitual dislocations,
kyphosis and flat feet.
cardiovascular system - aortic dilation, aortic
regurgitation, and aneurysms. Mitral valve prolapse
requiring valve replacement can occur as well.

Ocular findings - myopia, cataracts , retinal

detachment, and superior dislocation of the lens.
Oral Manifestations: a high, arched palatal vault, Bifid
uvula malocclusion, multiple odontogenic cyst,
temporomandibular dysarthrosis .
 
 Treatment and Prognosis
• no specific treatment
• management of the cardiovascular manifestations is
essential
 Osteopetrosis
Marble bone disease, Albers-Schonberg disease,
Osteosclerosis fragilis generalisata
A German radiologist - 1904

Etiology –
Osteopetrosis is a rare hereditary bone disease of
heterogeneous pathophysiology in which failure of
osteoclastic bone resorption leads to increased bone
mass.
Bone has poor mechanical properties.
Clinical features
Three distinct forms - based on age and clinical features.
adult onset, lethal,
infantile, transient,
intermediate postinfectious.

infantile and intermediate - autosomal recessive

adult onset - autosomal dominant
 Infantile osteopetrosis (malignant osteopetrosis)
Diagnosed early in life.
Failure to survive and growth retardation.
Bony defects occur.
Nasal stuffiness - mastoid and paranasal sinus malformation

Cranial nerve entrapment neuropathies

- failure of the foramina in the skull to
widen completely
S/S:
Deafness, Proptosis, And Hydrocephalus
Dentition might be delayed.
Osteomyelitis of the mandible due to a deficient blood supply.
Bones are fragile and can fracture easily.
Defective osseous tissue -replace bone marrow – cause marrow
failure - resultant pancytopenia.
Patients might have
anemia, easy bruising and bleeding (thrombocytopenia)
recurrent infections (defects in the immune system).
Extra- medullary hematopoiesis – result in hepatosplenomegaly,
hypersplenism, and hemolysis.
Other manifestations include sleep apnoea and blindness
due to retinal degeneration
Adult osteopetrosis (benign osteopetrosis)
Diagnosed in late adolescence or adulthood.
Patients are asymptomatic - diagnosis is made incidentally

S/S:
Osteomyelitis or fractures.
Bone pain, Bony defects
Cranial nerve entrapment neuropathies
(e.g. with deafness, with facial palsy),
carpal tunnel syndrome, and osteoarthritis
visual impairment due to retinal degeneration /psychomotor retardation.
 Physical findings - short stature, frontal bossing, a large
head, nystagmus, hepatosplenomegaly, and genu valgum in
infantile osteopetrosis.

Bone marrow function is not compromised.

D/D: hypoparathyroidism, myeloproliferative disease,

Paget disease, pseudohypoparathyroidism and lead toxicity.
Oral Manifestations.
↓medullary spaces of the jaws - predilection for osteomyelitis -
complication of dental extraction - large doses of antibiotics
Fracture of the jaw during tooth extraction
Teeth are of defective quality - enamel hypoplasia, microscopic
dentinal defects and arrested root development, prone to dental
caries, retardation of tooth eruption due to the sclerosis of bone.
Roentgenographs Features – heterogeneous disorders
Usually - generalized osteosclerosis – uniformly sclerotic
Alternating sclerotic and lucent bands – (endobone).
Entire skull is thickened and dense, especially at the base.
Sinuses are small and underpneumatized.
Vertebrae are extremely radiodense.
They may show alternating bands, known as the “rugger-jersey”
sign.
Radiographs may show evidence of fractures or osteomyelitis.
When the jaws are affected, the density of the bone maybe such
that the roots are invisible on the dental roentgenogram.
Radio-opaque
Laboratory Findings:
myelopthisic anemia due to the displacement of hematopoeitic
marrow tissue by bone.
Hypocalcemia can occur and cause rickets if it is severe enough.
PTH is often elevated (secondary hyperparathyroidism).
Acid phosphatase and creatinine kinase (CK-BB) levels are
increased due to increased release from defective osteoclasts.
Histologic Features.
Bone biopsy is not essential for diagnosis because radiographs
are usually diagnostic.
endosteal production of bone - lack of physiologic bone
resorption
Osteoblasts are prominent, osteoclasts are seldom found
Predominance of bone formation over resorption typically
leads to the persistence of cartilaginous cores of bony
trabeculae
The trabeculae themselves are disorderly in arrangement, and
the marrow tissue present is usually fibrous.
Treatment / Prognosis.
Infantile osteopetrosis warrants treatment due to the adverse
outcome associated with the disease.
Calcitriol – stimulates dormant osteoclasts - bone resorption.
Erythropoietin - correct anemia.
Corticosteroids - stimulate bone resorption.
gamma interferon - long-term benefits.
Adult osteopetrosis requires no treatment
If untreated, infantile osteopetrosis-results in death due to severe
anemia, bleeding or infection.
Adult patients are usually asymptomatic and have good long-term
survival rates.
 Rickets / English disease ('wricken‘ means to bend)

It is found only in children prior to the closure of the growth

plates, while osteomalacia occurs in persons of any age.

Etiology:
Deficiency or abnormal metabolism of vitamin D or inorganic
phosphate.

women who rarely leave the house

wear veils
Babies born to these women are at higher risk of developing
rickets.

In most developing countries, rickets is seldom seen, due to high

exposure to sunlight
A useful mnemonic for remembering the findings of rickets is as
follows;
Reaction of the periosteum (may occur)
Indistinct cortex
Coarse trabeculation
Knees, wrists, and ankles affected predominantly
Epiphyseal plates, widened and irregular
Tremendous metaphysis (cupping, fraying, splaying)
Spur (metaphyseal )
Histologic changes are seen at the level of the growth plates,
at the level of the hypertrophic zone,
increased number of disorganized cells is found.
The increased number of cells results in increased width and thickness
of the hypertrophic zone (Rachetic metaphysis).

Treatment:
When patients receive adequate treatment, no mortality is associated
with this disease
Concomitant diseases, such as pneumonia, tuberculosis and enteritis
occur with higher frequency and may cause death.
Vitamin D-resistant Rickets
(Familial hypophosphatemic rickets, refractory rickets, phosphate
diabetes)

Since the early 20th century, ultraviolet radiation or vitamin D

ingestion - cure for nutritional rickets, certain forms of rachitic
diseases have remained refractory to this therapy.
low serum phosphate concentration / Familial occurrence
- familial hypophosphatemic rickets.
Treatment with vitamin D produced no change - vitamin D-resistant
rickets.
Etiology:
Sex linked:
PEX gene (X chromosome - Xp22.1)
↓
Produce unknown hormone
↓
involved in phosphate regulation.

It is a X-linked dominant.
Autosomal:
12pl3 is the gene locus for autosomal dominant
hypophosphatemic rickets
phosphate wasting at the proximal tubule level
↓
defective ossification.
This phenomenon is secondary to defective regulation of the
sodium-phosphate cotransporter in the epithelial cell brush border.
Clinical Features
The disease is present from conception.
Affected new­borns are of normal weight / normal intelligence –
growth retardation
Widened joint spaces
flaring at the knees – apparent at first birthday - in boys
When a child begins to stand and walk -
bowing of the weight-bearing long bones quickly - evident.
Dentition may be absent or delayed - due to abnormal tooth formation
multiple dental abscesses.
Laboratory Findings
calcium levels -↓↓
alkaline phosphatase -↑↑
PTH -↑
Calcitriol -↓
Serum phosphate levels must he carefully evaluated in the first
year because the concentra­tion reference range for infants (5.0-7.5
mg/dL) is high compared to adults (2.7-4.5 mg/dL).

urinary loss of phosphate - ↑

Treatment and Prognosis
Usual vitamin D preparations are not useful for treatment
- lack significant 1-alpha-hydroxylase activity.
vitamin D at levels of 25,000 – 50,000 U/d
Calcitriol. Amiloride and hydrochlorothiazide - enhance calcium
reabsorption and reduce the risk of nephrocalcinosis.
Surgical care - osteotomy to realign extremely distorted leg curvatures
Skull deformity may require treatment for synostosis.
Spontaneous abscesses often require periodic dental procedures.
Apart from the short stature of most affected adults, the prognosis for a
normal lifespan and normal health is stood.
Fibro osseous lesions
FIBRO OSSEOUS = FIBRO + OSSEOUS

Fibro = fibroblasts and collagen.

Osseous = osteoblasts and bone

• Group of lesions in which normal bone is replaced by fibrous

tissue composed of collagen fibers, fibroblasts.

"A group of pathological changes within the jaw bones, in which

normal bone is replaced by fibrous tissue with/without
calcification” Waldron -1970

It indicates a broad group of several entities.

The lesions of the jaws include developmental (hamartomatous)

lesions, reactive or dysplastic processes, and neoplasms.
 CLASSIFICATION
According to Waldron (1993)
[ Review of nomenclature revision of fibro-ossous lesions in the maxillofacial region Byung-Do Lee, (Korean J Oral Maxillofac Radiol
2007; 37 : 1-7) ]

I . Fibrous dysplasia 

II. Reactive (dysplastic) lesions arising in the tooth-bearing area.

These are presumably of periodontal ligament origin.
Periapical cemento-osseous dysplasia
Focal cemento-osseous dysplasia
Florid cemento-osseous dysplasia
 
III. Fibro-osseous neoplasms
These are widely designated as
cementifying fibroma, ossifying fibroma, or cemento-ossifying
fibroma.
Fibrous dysplasia is defined as a "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 is a skeletal developmental anomaly of the
bone-forming mesenchyme that manifests as a defect in
osteoblastic differentiation and maturation.
Virtually any bone in the body can be affected.
 Classification
Fibrous dysplasia are divided into four basic types:
Monostotic form, Polyostotic form, Craniofacial form and
cherubism

Polyostotic fibrous dysplasia can be further subdivided

into two types.

Lichtenstein-Jaffe Type: Multiple bones of the skeleton

are affected and in some instances, there are café-au-lait
spots on skin and rare endocrinopathies.

Albright’s syndrome: Characterised by the triad of severe

polyostotic fibrous dysplasia (mostly unilateral), café-au-
lait spots or lesions on the skin, and various endocrine
dysfunctions, especially precocious puberty in girls.
 Etiology:
It is a nonhereditary disorder of unknown cause.
mutation in the GNAS1 gene (20ql3.2)
↓
gene encodes G- protein – continuous activation
↓
Over production of cAMP
Hyperfunction of
, endocrine
affected
organs increased proliferation
of melanocytes
precocious puberty, resulting in large café-
hyperthyroidism, growth au-lait spots
hormone and Cortisol
overproduction.
effect on the differentation
of osteoblasts leading to
fibrous dysplasia
Clinical features:
Age : 3—15 years.
polyostotic disease - asymptomatic till 10 years.
monostotic disease - till 20 or 30 years are asymptomatic
Race: Nil
Sex: equal
S/S:
Clinical findings of increasing pain and an enlarging soft tissue
mass suggest malignant change
Oral Manifestations.

expansion and deformity of the jaws,

eruption pattern of the teeth is distorted

-loss of normal support of the developing teeth.

-The endocrine disturbance

MONOSTOTIC FIBROUS DYSPLASIA

Monostotic fibrous dysplasia, less serious than polyostotic

form - greater concern to the dentist - jaws are affected

The clinical term “Leontiasis ossea” applied to fibrous

dysplasia of maxilla or facial bones

Clinical Features.
mild predominance of females.
children and young adults than in older persons
S/S:
painless swelling of the jaw involving the labial or buccal plate,
seldom the lingual aspect,
when it involves the mandible - a protuberant excrescence at the
inferior border is noted
malalignment, tipping or displacement of teeth
tenderness may ultimately develop
Mucosa is invariably intact
Fibrous dysplasia - maxilla in children - require surgery.
These lesions extend into - maxillary sinus, zygomatic process the
floor of the orbit, and base of skull.

Severe malocclusion and bulging of the canine fossa or extreme

prominence of zygomatic process, producing a marked facial
deformity, are typical sequelae of disease in this location need
not be truly monostotic, but neither is it usually clarified as a
polyostotic type.
Radiographic features:

There are three basic patterns

Type I: small unilocular radiolucency
Larger multilocular radiolucency,
well-circumscribed border with network of fine bony trabaculae.

Type II: the pattern is similar with increased trabeculation –

more opaque / mottled appearance.

Type III: More opaque many delicate trabeculae giving a “ground-

glass” or “peau d'orange”.

This is not well circumscribed and blends into the adjacent normal
bone.
 cortical bone becomes thinned - seldom is perforated.

The roots of teeth in the involved areas displaced / resorbed.

Sometimes the bone appears so opaque that the roots of teeth

may be indistinct.
Thickening of the base of the skull.
The lucent lesion has a thick sclerotic border and is called the rind sign.
skull and face - frontal bone is involved frequently than the
sphenoid, with obliteration of the sphenoid and frontal sinuses.
long and short tubular bones - lucent lesion in the diaphysis or
metaphysis, with endosteal scalloping
Shepherd’s crook deformity - fermur
 Histologic Features.
fibrillar connective tissue within which are numerous trabeculae
of coarse, woven immature bone
some of these trabeculae are C-shaped, Chinese character-
shaped.

The osteocytes are quite large, and collagen fibers of these

trabeculae can often be seen extending out into the fibrous tissue.

Bone formation by stellate osteoblasts can be observed, although

rows of cuboidal osteoblasts lined up on the surfaces of trabeculae
are absent (osteoblastic riming).

These trabeculae typically have wide osteoid seams.

Osteoclastic activity may be seen where the calcification of

osteoid extends to the surface of the trabeculae.
 Treatment
usually conservative and primarily to prevent deformity.
No specific medical treatment exists
vitamin D and bisphosphonates (after epiphyseal closure)
Surgical therapy with curettage and replacement of the bone
defect with autograft or allograft
 Prognosis:
Estimated frequency of malignant transformation is 0.4-1 per cent
monostotic disease - skull and facial bones undergo malignancy
polyostotic disease - femoral and facial bones undergo malignancy
Osteosarcoma and fibrosarcoma are the most common tumors.
Chondrosarcomas occur less frequently.

Character of malignancy:
Roentgenographic features - rapid increase in the size of the lesion
- a change from a previously mineralized bony lesion to a lytic lesion.
Clinical findings - increasing pain and an enlarging soft tissue mass
 McCune-Albright Syndrome
(Polyostotic fibrous dysplasia)
It is associated with
polyostotic fibrous dysplasia
precocious puberty - gonadotrophin-independent
Endocrinopathies

Clinical Features.
Associated endocrine disturbances
•Cushing syndrome,
•hyperparathyroidism,
•hyperthyroidism, •McCunc-Albright syndrome,
•acromegaly, •hypophosphatemic rickets
•gonadotrophin-McCune-Albright
syndrome,
•hyperprolactinemia,
•rickets
 severely affected patients - hepatic, cardiac, and GI dysfunction
Cutaneous pigmentation - ipsilateral to the side of bony lesions,
a feature that differentiates this disease from pigmentation in
neurofibromatosis.
cafe-au-lait spots - arranged in a linear or segmental pattern near
the midline
The association of fibrous dysplasia and intra- muscular myxoma is a
rare disease known as Mazajabraud’s syndrome
malignant transformation is less –
Osteosarco
Chondrosarco
Fibrosarco McCune-AIbright syndrome.
 Treatment and Prognosis.

McCune-Albright syndrome is a multisystem condition with a host

of variable presentations.

Management often is challenging and requires a multidisciplinary

approach.
Cemento osseous dysplasia
Cemento-osseous dysplasia
occurs in the tooth- bearing areas of the jaws
most common fibro-osseous lesion

Pathogenesis:
close approximation - PDL origin.
defect in extraligamentary bone remodeling - hormonal imbalance.

Based on the clinical and radiographic features

(1)Focal
(2) periapical
(3)Florid
 Focal cemento-osseous dysplasia
single site of involvement
Clinical Features
Age : mean age of 38 / third to sixth decades
Sex : 90% in females,
Race: whites.
Site: posterior mandible
S/S: asymptomatic and is detected only on a
radiographic examination.
Lesion < 1.5 cm in diameter
Radiographic Features

radiolucent to densely
radiopaque peripheral
radiolucent rim

mixed radiolucency
 Periapical cemento-osseous dysplasia
(osseous dysplasia; cemental dysplasia; cementomas)
Lesion at periapical region of the anterior mandible.
Clinical features:
Solitary / multiple foci
Age: 30 and 50 yrs never under 20 years
Sex: female (ranging from 10:1 to 14:1)
Race: blacks.
S/S:
associated tooth – vital
asymptomatic condition – discovered on radiographs are
taken for other purposes.
X-Ray
Lesion 1cm in diameter
Stage I: periapical region – circumscribed radiolucency IIIr
periapical granuloma or periapical cyst
Stage II: lesions "mature" over time to create a mixed
radiolucency
Stage III: lesions show a circumscribed dense calcification
surrounded by a narrow radiolucent rim.
Periodontal ligament is intact

Lesion is not fused to the tooth

Florid cemento-osseous dysplasia
multifocal involvement anterior / posterior mandible synchronous
Age: middle- aged to the elderly
Sex: women
Race: black

S/S :
bilateral / symmetric involvement / all four posterior quadrants.

Asymptomatic / dull pain

alveolar sinus tract (expose) yellowish avascular bone
Bone expansion may be noted
 X-Ray:
pattern identical to other two forms.
On occasion, a lesion can become almost totally radiopaque and
blend with the adjacent normal appearing bone.
 Histopathologic Features
All three patterns of cemento-osseous dysplasia demonstrate
similar

Stage I: cellular mesenchymal tissue

spindle shaped fibroblasts
collagen fibers
numerous small blood vessels
Free hemorrhage is interspersed throughout the lesion

Stage II: Within fibrous connective tissue

mixture of woven bone,
lamellar bone
cementum like particles
 Stage III: sclerotic
fibrous connective tissue: mineralized material
the bone trabeculae become thick curvilinear structures -
ginger roots.

final radiopaque stage - individual trabeculae fuse and form

lobular masses composed of sheets or fused globules of
relatively acellular and disorganized cemento-osseous material
Treatment and Prognosis
The lesion is not neoplastic - do not require removal.

asymptomatic patient, - regular recall examinations

prophylaxis

good home hygiene care

biopsy or elective extraction of – avoided

( ↓ vascular - ↑ fracture)
FAMILIAL GIGANTIFORM CEMENTOMA

uncommon hereditary disorder

autosomal dominant disorder

Familial gigantiform cementoma is a disorder of gnathic
bone that ultimately leads to the formation of massive
sclerotic masses of disorganized mineralized material.
 Clinical and Radiographic Findings
a rapid and expansive growth pattern

Jaws - maxilla and mandible

gnathic enlargement -facial deformity,

impaction, malposition, malocclusion.

If not treated, the osseous enlargement – ceases -fifth decade

X-ray : similar to Cemental dysplasia

Lab findings:
elevated serum alkaline phosphatase
Anemia.

Histopathologic Features
same spectrum of changes seen in florid cemento-osseous
dysplasia

Treatment and Prognosis

Resection and reconstruction of the altered bone - functional and
aesthetic results.
 OSSIFYING FIBROMA
(CEMENTIFYING FIBROMA;
CEMENTO-OSSIFYING FIBROMA)

It is a true neoplasm with a significant growth potential.

X-Ray & H/P resembles cemento-osseous dysplasia.

Etiopathogenesis:
odontogenic or from periodontal ligament,
identical lesion in the orbital, frontal, ethmoid, sphenoid, and
temporal bones ?.
 Clinical Features
Age: wide age range - third and fourth decades
Sex: female predilection,
Site: mandible > maxilla.
premolar and molar area
S/S:
Small lesions asymptomatic and are detected only on
radiographic examination.
Larger tumors result in a painless cause obvious facial
asymmetry, which on occasion reaches grotesque size.
Pain and paresthesia are rare.
 Radiographic features:
Radiolucent - well defined and unilocular / sclerotic border.
varying degrees of radiopacity.
radiopaque with a thin radiolucent periphery are
uncommon
Root divergence or resorption of roots of teeth
Large lesions of the mandible - downward bowing of the
inferior cortex of the mandible.
 Histopathologic Features
fibrous tissue - varying degrees of cellularity
mineralized material - bony trabeculae, cementum- like
spherules, or both.
fibrous capsule sometimes well demarcated
The spherules demonstrate peripheral brush borders that blend
into the adjacent connective tissue
Variation in the types of mineralized material distinguish fibrous
dysplasia
Treatment and Prognosis

enucleation of the tumor with relative ease.

large - surgical resection and bone grafting.

The prognosis is very good, and recurrence is rare

no malignant change.
 JUVENILE OSSIFYING FIBROMA
(JUVENILE ACTIVE OSSIFYING FIBROMA;
JUVENILE AGGRESSIVE OSSIFYING FIBROMA)

Based on –

age of the patients

most common sites of involvement

clinical behavior.

Two Patterns

(1) trabecular and (2) psammomatoid.

Clinical Features
Age: 6 months to over 70 years of age
( trabecular form -II years / psammomatoid variant - 22
years)
Sex : equal
Site: maxillary predominance
S/S: slow growing
Well – circumscribed
discovered upon routine radiographic examination,
cortical expansion result in facial enlargement.
 Psammomatoid frequently appears outside of the jaws

Orbital / frontal bones / paranasal sinuses.

Nasal obstruction, exophthalmos, or proptosis may be seen.

Rarely, temporary or permanent blindness occurs.

Intracranial extension has been discovered in neoplasms arising

adjacent to the cribriform plates

X-ray features:

The lesions are circumscribed radiolucencies with central

radiopacities

Lesion in sinus may appear radiodense and often create a

clouding that may be confused with sinusitis

Histopathologic Features
Both patterns are -

nonencapsulated - well demarcated

The tumor consists of

fibrous connective tissue that are loose and cellular

Myxomatous foci with pseudocystic degeneration.

Mitotic figures can be found but are not numerous.

Areas of hemorrhage and small clusters of multinucleated giant

cells are usually seen.

The mineralized component in the two patterns is very different.

trabecular variant : irregular strands of highly cellular osteoid encasing

osteocytes and lined by plump osteoblasts /multinucleated osteoclasts.

the psammomatoid pattern: concentric lamellated and spherical ossicles

with basophilic centers with peripheral eosinophilic osteoid rims

A peripheral brush border blending into the surrounding stroma

is noted in many of the ossicles. Occasionally, individual ossicles

Treatment and Prognosis
smaller lesions - complete local excision or thorough curettage

appears adequate.

rapidly growing lesions - wider resection may be required.

recurrence rates - 30% to 58

Malignant transformation – not documented.

 Cherubism
(Familial fibrous dysplasia of the jaws,
disseminated juvenile fibrous dysplasia,
familial multilobular cystic disease of the jaws,
familial fibrous swelling of the jaws)
“An autosomal dominant fibro-osseous lesion of the jaws involving
more than one quadrant that stabilizes alter the growth period,
usually leaving some facial deformity and malocclusion.”
“Cherubism, a non-neoplastic hereditary bone lesion that is
histologically similar to central giant cell granuloma affects the jaws of
children bilaterally and symmetrically; usually producing the so- called
cherubic look “
The disease was first described in 1933 by Jones, who called it
familial mutilocular disease of the jaws.
 It is a rare, benign condition with autosomal
dominant inheritance
it is genetically determined osteoclastic lesion.
Sex: male – 100% penetrance
Female - 50-70%
usually occurs bilaterally / unilateral involvement
Clinical Features
Children are normal at birth
Disease manifests at 14 months -3 yrs
Symmetric enlargement of the jaws begins.
The earlier the lesion appears, the more rapidly it progresses.
Self- limited bone growth - slows down at 5 yrs of age
stops by 12-15 years.
regress - puberty .
Jaw remodeling continues - third decade of life.
S/S:
Innocent to grosteque deformity of mandible and maxilla
- respiratory obstruction
impairment of vision/ hearing.
painless and symmetric florid involvement.
firm to palpation / nontender, condyles always spared, cervical
lymphadenopathy.
LN enlarge - 6 years
decrease - eight years

palate - alveolar ridge - V shape. rarely enlarged - 12 years.

A rim of sclera visible beneath the iris, giving the classic 'eye to
heaven' appearance.
 Oral Manifestations.
agenesis of the mandibular second and third molars
displacement of the teeth,
premature exfoliation of the primary teeth,
delayed eruption of the permanent teeth,
transpositions and rotation of the teeth.
In severe cases, tooth resorption occurs.
The oral mucosa is usually intact and of normal color.
Noonan's syndrome,
a lesion in the humerus,
gingival fibromatosis,
psychomotor retardation,
orbital involvement
obstructive sleep apnea.
Grading System. Arnott (1978)
grade I - involvement of both mandibular
ascending rami,
grade II - involvement of both maxillary tuberosities,
mandibular ascending rami
grade III - McCune-Albright syndrome
whole maxilla and mandible
except the coronoid process and condyles.
 Roentgenographic Features.
bilateral multilocular cystic expansion of the jaws.
Early lesions - posterior body of the mandible, ascending rami.
Maxillary lesions may escape early radiographic detection because of overlap of the

sinus and nasal cavities.

Displacement of the inferior alveolar canal.

numerous unerupted teeth
destruction of the alveolar bone → displaces teeth →
floating tooth syndrome
Adulthood - the cystic areas → re-ossified → ground glass
appearance
Treatment:
surgery - esthetic considerations or severe functional
 Histologic Features
numerous multinucleated giant cells - osteoclasts.
collagenous stroma - spindle-shaped fibroblasts
water-logged, granular nature
Numerous small vessels are present
large endothelial cells
perivascular cuffing.
Older, resolving lesions - increase in fibrous tissue
decrease in giant cells
formation of new bone
D/D:
giant cell granuloma, osteoclastoma
aneurysmal bone cyst, fibrous dysplasia
hyperparathyroidism.
 Mucopolysaccharidoses
(Lysosomal storage disease)

Mucopolysaccharidoses (MPS) are a group of lysosomal storage

diseases, each of which is produced by an inherited deficiency of an
enzyme involved in the degradation of acid mucopolysaccharides
[GAG]).

These diseases are autosomal recessive, except tor MPS type II,
which is X-linked.

Etiology.
Proteoglyans (GAGS)  ECM  Endocytosis Lysosomal
enzymes  Degradation
Enzyme deficiency -> lysosomal storage disease
Sno Disease Enzyme deficiency
1 Hurler syndrome Alpha-L- iduronidase deficiency

2 Scheie syndrome Alpha-L- iduronidase deficiency

3 Sanfilippo syndrome Heparan sulfate sulfamidase ,

N-acetyl-alpha-D-glucosaminidase,
Acetyl-CoA

4 Morquio syndrome N-acetylgalactosamine-6-sulfatase

5 Marteaux – lamy syndrome N-acetylgalactosamine-4-
sulfatase

6 Hunter syndrome L-sulfoiduronate sulfatase

Clinical Features
Manifests - early childhood.
Skeletal findings - dwarfism
Treatment:
stiff articulations
No cure for
coarse facies
MPS exists,
Death occurs –
symptomatic
Hurler - 5-10 years. and
Scheie syndrome - normal. supportive
Hunter and Sanfilippo syndrome - puberty.
Morquio syndrome - 20-40 years.
Maroteaux- Lamy syndrome - early adulthood.
D/D:
Gaucher disease, Niemann-Pick disease,
syphilis, osteogenesis imperfecta,
vitamin D-resistant rickets, nephrogenic osteopathy,
Hyperparathyroidism /↑PTH

PTH gland
↓regulate
serum calcium and phosphorous levels
Hyperparathyroidism are discovered accidentally when hypercalcemia is noted
during a routine serum chemistry examination.

Etiology:
Primary – hyperplasia / adenoma of PTH gland – MEN typeI / II
Secondary – gland hyperplasia – chronic low ca++
chronic renal failure, rickets,
malabsorption syndome
Tertiary – autonomous hyperstimulation of gland -
hypercalcemia
Clinical Features

Age: any age- fifth and sixth decades

Sex: females
S/S: Asymptomatic - hypercalcemic parathyroid crisis

Skeletal And Muscular Changes

profound
bone pain tenderness, muscle fatigue dehydration
weakness and spontaneous fractures , coma
non­specific myalgias, osteoporosis, osteopenia
cystic bone lesions, vertebral collapse
chondrocalcinosis and pseudogout
Abdomen :
Pancreatitis / pancreatic calcification
Peptic ulcer, abdominal distress, constipation,
vomiting, anorexia and weight loss.

Neuropsychiatric illness, altered mental status -

anxiety, depression, psychosis and apathy
Hypertension and congestive heart failure.
Laboratory Findings

↑PTH, ↑ serum calcium.

↑ serum chloride levels,

↓serum phosphate level

↓ serum carbon dioxide,

↑ urine cyclic adenosine monophosphate (cAMP)

Treatment and Prognosis.

symptoms are mild at the time of presentation and resolve with

surgical correction of the disorder.

Hypoparathyroidism

Primary hypoparathyroidism is caused by a group of heterogeneous conditions in

which hypocalcemia and hyperphosphatemia occur as a result of deficient
parathyroid hormone (PTH) secretion.

Etiology:
Surgical excision / damage to parathyroid glands / Genetic
Autoimmune / pseudohypoparathyroidism
Clinical features:
hypocalcemia
↓hypomagnesemia/hyperkalemia
Neuromuscular hyper-excitability
 S/S:
circumoral numbness
paresthesia of the distal extremities
muscle cramping – carpopedal spasm/tetany
Laryngospasm or bronchospasm and seizures
less specific manifestations - fatigue, irritability, and personality
disturbance.
calcification of the basal ganglia / intracranial calcification
Extrapyramidal neurological symptoms, subcapsular cattracts, band
keratopathy, and abnormal dentition
Lab findings:
Serum -
↓Ca
↑Po4
Vit D urine - ↓Ca, ↑Po4

Treatment:
raise the serum calcium
alleviate acute symptoms
 Cleidocranial Dysplasia
(Marie and Sainton's disease,
Scheuthauer-Marie- Sainton syndrome,
mutational dysostosis)
A congenital disorder of bone formation
clavicular hypoplasia or agenesis with a
narrow thorax, which allows approximation
of the shoulders in front of the chest.
Familial / autosomal dominant
core- binding factor alpha-1 (CBFA1) gene - 6p21
 General features:
Delayed ossification of the skull, excessively large fontanelles - until
adulthood, delayed closing of the sutures - interposition of wormian bones
Bossing of the frontal, parietal, and occipital regions give the skull a large
globular shape with small face “Arnold head”

More than 100 additional anomalies may be

associated –
wide pubic symphysis,
dental abnormalities,
short middle phalanges of the fifth finger
delayed skeletal maturation, defects of vertebral column/ long bones
 abnormalities of the skull, teeth, jaws and shoulder girdle
stunting of the long bones
paranasal sinuses are underdeveloped
calvarial thickening - supraorbital part of the frontal bone
squamous part of the temporal bone
occipital bone;
occasional absence of the parietal bones
faulty development of the foramen magnum,
Clavicle- complete / partial absence
thinning of bone
Oral Manifestations.

Palate - high, narrow arched , cleft palate

Maxilla – underdeveloped

enlarged mandibles rather than small maxillae

lacrimal and zygomatic bones - underdeveloped.

prolonged retention of the deciduous teeth 

delay in eruption of the succedaneous teeth

Tooth roots - short and thinner

Absence of cementum
Roentgenographic Examination.

widely patent anterior fontanel and sutures with wormian

bones in cranium

clavicles – fragmented on each side with middle part being deficient

Delayed ossification of pelvic bones

Spina bifida occulata – cervical and thoracic level

Hands and feet – small carpals, metacarpal, tarsal

 Treatment and Prognosis.

no specific treatment

The retained deciduous teeth - restored if they become carious

since their extraction does not
necessarily induce eruption of the
permanent teeth.
Life expectancy is normal
 Infantile Cortical Hyperostosis
(Caffey's disease,
Caffey-Silverman syndrome,
familial infantile cortical hyperostosis,
sporadic infantile cortical hyperostosis)
Caffey and Silverman described
Smyth and his coworkers - syndrome of unknown etiology
cortical thickening in certain bones of infants
It is self- limited disorder that affects infants and causes – triad
bone changes, soft tissue swelling, and irritability.

familial and sporadic forms appear to exist.

Etiology.
1. inflammatory process –
Early lesion - inflammation of the periosteum and adjacent soft tissues is
observed.
As this resolves - the periosteum remains thickened and subperiosteal immature
lamellar bone is observed.
2 . genetic transmission exists.
3. infectious agent with a long latency period.
4. primary arterial abnormality and allergic reaction.
Clinical Features.
self-limited condition.
Sex: No predilection
two forms –
familial - earlier onset, lower extremities, 6-8wks
Sporadic - late, mandible, 9-11 wks

triad - Irritability
swelling,
bone lesions
,
 Site:
mandible and the clavicles
jaw involvement - facial swelling
Other bones - calvarium, scapula, ribs
tubular bones of the extremities-metatarsals.
S/S:
Swelling appears – suddenly / deep / firm / tender.
Fever may occur.
Babies may refuse to eat, - mandibular involvement,
soft- tissue swellings - associated with deep muscles
scalp, face, neck, thorax and extremities.

pseudoparalysis, dysphagia, pleurisy, anemia, leukocytosis, monocytosis, elevated

sedimentation rate and increased serum alkaline phosphatase .
 Oral Manifestations.
asymmetric deformity of the mandible - angle and ramus area,
severe malocclusion

Roentgenographic Features.
Florid – periosteal new bone formation
Cortical thicknening
Distribution - patchy / asymmetric / multifocal
periosteal new bone or periosteal 'cloaking' is confined to the
diaphyses of the long bones, sparing the metaphvses and
epiphyses.
Histologic Findings.
In early stages - inflammation of the periosteum
adjacent soft tissues is observed.
As this resolves - periosteum remains thickened
subperiosteal immature lamellar bone
Bone marrow spaces - vascular fibrous tissue.
Mature lesion - hyperplasia of lamellar cortical bone
without inflammation or subperiosteal changes.
Treatment. No specific treatment
resolves without sequelae in six to nine months
 Paget's Disease - Sir James Paget
(osteitis deformans)
It is characterized by excessive and abnormal remodelling of bone, results in
bones that are extensively vascularized, weak, enlarged, and deformed with
subsequent complications.

Etiology
Unknown
Genetic – autosomal dominant
viral infection - viral inclusion particles in pagetic osteoclasts.
measles virus
inflammatory cause - clinical improvement after anti-inflammatory drugs
Elevated parathyroid hormone
Autoimmune, connective tissue, and vascular disorders are other possible etiologies.
 Paget s disease of bone is characterized by enhanced
resorption of bone by giant multinucleated osteoclasts with
formation of disorganized woven bone by osteoblasts
This process evolves through various phases
•lytic
•mixed lytic and blastic, and
•sclerotic or burned out.

Clinical Features.
Age – prevalence increases with age / after age 50 years
rarely in 20 years.
Sex: equal
Race: whites
musculoskeletal impairments
Neurologic – nerve palsy
cardiovascular complications
Bone - the axial skeleton -
pelvis and spine skull, shoulder girdle and proximal humerus
dull constant aching pain - persist or exacerbate during the night,
pathologic fractures - due to weak bone
warm to the touch because of the increased vascularity
Skull involvement - nonspecific headaches, impaired hearing, and tinnitus
change in hat size / Platybasia

Face - leontiasis ossea (lion-like facies)

Progressive painT paresthesias,

limb paresis, gait difficulties (waddling gait)

platybasia or vertebral fractures – compression of the spinal cord or spinal nerve
bowel and bladder incontinence
hydrocephalus -
Nausea, dizziness,
syncope, ataxia,
dementia can be observed with,
basilar invagination, and cerebellar or brainstem compressive syndromes.
Oral Manifestations.
Involvement of the jaws - common occurrence.
maxilla > mandible
↓
progressive ,enlargement
the alveolar ridge – widened
palate is flattened
Teeth - loose and displaced producing some spacing
Advanced stage : mouth – unable to close
Edentulous pts – denture – tight – remade - frequently
Roentgenographs Features.
Varies depend upon the stage of the disease
initial phase - deossification and softening
Intemediate - dysplastic type of reossification
Initial - osteolytic areas of the skeleton – associated with osteoblastic activity.
These destructive lesions may be multiple and diffuse or isolated.
Skull - large isolated lesion – “Osteoporosis circumscripta”
The osteoblastic phase with pre-existing osteolytic lesions – “ cotton –wool appearance”

Teeth – hypercementosis
Loss of lamina dura
Root resorption -rare
 Lab findings:

Serum-
Calcium / phosphorus – normal
Alkaline phosphataes – elevated

Urine-
Hydroxyproline - elevated – osteoclastic activity
Histopathology:
3 phase
Osteolytic phase:
disordered areas of resorption – large osteoclasts (100 nuclei)
Osteoblastic phase:
New bone matrix and woven bone
Repeated episode of bone removal and formation – jigsaw / mosaic
pattern
Predominantly osteoblastic phase:
Compact and dense bone – pagetic bone
Marrow space – loose, highly vascular
Hypercementosis – obliteration of periodontal ligament
Treatment and Prognosis.

no specific treatment

Vitamin therapy

hormone therapy – calcitonin

radiation therapy

Biphosphonates

Cytotoxic antibiotics- mithramycin

Complication : fracture / Sarcomatous degeneration

 Massive Osteolysis
(Vanishing bone, disappearing bone, phantom bone,
progressive osteolysis, Gorham syndrome)

It is characterized by spontaneous, progressive resorption of

bone with ultimate total disappearance of the bone

Etiology : episode of trauma

Clinical Features.

Age : older children and young and middle- aged adults

Sex: equal
S/S: Usually only one bone is affected sometimes polyostotic
clavicle, scapula, humerus, ribs, ilium, ischium, and sacrum.
Painless but begins suddenly and advance rapidly – bone is replaced by a
thin layer of fibrous tissue surrounding a cavity.
Oral Manifestations.
mandible and other facial bones - pain or facial asymmetry or both.
pathologic fracture following minor trauma - consistent findings
Histologic Features.
replacement of bone by connective tissue
Many thin-walled blood vessels
anastomosing vascular spaces lined by endothelial cells
increased osteoclastic activity- absence in areas of active resorption is often quite striking.
 All laboratory values are usually normal.
Treatment and Prognosis.

There is no specific treatment.

Radiation therapy / surgical resection

when Left untreated, the disease commonly progresses to total destruction of

the involved bone.