IN THE NAME - PowerPoint Presentation

Slide1

IN THE NAME OF GOD

Dr.kheirandish

DDS,MSC

Oral and maxillofacial pathologySlide2

Bone PathologyChapter 14Slide3

Osteogenesis ImperfectaOsteopetrosis

Cleidocranial

Dysplasia

Focal Osteoporotic Marrow Defect

Idiopathic

Osteosclerosis

Massive

Osteolysis

Paget's Disease of BoneSlide4

OSTEOGENESIS IMPERFECTASlide5

Most common type of inherited bone diseaseCollagen maturationType

I

collagen

COL1A1

gene on chromosome 17

COL1A2

gene on chromosome 7

Collagen forms :

Major portion of bone

Dentin

Sclerae

Ligaments

Skin

Autosomal dominant

Autosomal recessive

Sporadic Slide6

Bone Thin cortexFine trabeculationDiffuse osteoporosis

Fracture

Long bone and spine deformities

Blue sclera

Altered teeth

Hypoacusis

(hearing loss)

Joint

hyperextensibilitySlide7

Radiographic hallmarks OsteopeniaBowingAngulation

Deformity of the long bones

Multiple fractures

Wormian

bones in the skull (not specific)

Wormian

bones

10 or more

sutural

bones that are 6 x 4 mm in diameter or larger and arranged in a mosaic pattern. Slide8
Slide9

Oral cavityBoth dentitions Blue to brown translucenceReveal premature pulpal obliteration

Dentinogenesis

imperfecta

Class III malocclusion (maxillary hypoplasia)Slide10

TYPE I OSTEOGENESIS IMPERFECTAMost common and mildest formMild to moderately severe bone fragility

Hearing loss

commonly develops before age 30

Hypermobile joints

and easy bruising

Opalescent dentin

Blue

sclerae

Autosomal

dominant

TYPE II

OSTEOGENESIS IMPERFECTA

Most

severe

form

Extreme

bone fragility

Many patients are

stillborn

, and 90% die before 4 weeks of age

Blue

sclerae

Opalescent teeth

Both autosomal

recessive and dominantSlide11

TYPE III OSTEOGENESIS IMPERFECTAMost severe form noted in individuals beyond the perinatal period

Severe

bone

fragility

Sclerae

(fades

as the child grows

older)

Hearing

loss

Die

during

childhood

(cardiopulmonary

complications

caused by

kyphoscoliosis

)

Opalescent

dentin/

normal

teeth

Both autosomal

dominant

and recessive

TYPE

IV

OSTEOGENESIS IMPERFECTA

Mild

to

moderate

bone

fragility (decreases

after

puberty)

Sclerae

(blue

color

fades

later in

life)

Opalescent

dentin/

normal

teeth

.

Autosomal

dominant Slide12

Osteoblasts are presentBone matrixFailure

of woven bone to become transformed

to lamellar

boneSlide13

No cure Symptomatic improvement

Intravenous

(

IV) or

oral

bisphosphonates : decreased pain, reduced risk of fracture

Opalescent dentin

:

severe

attrition of their teeth, leading to tooth

lossSlide14

OSTEOPETROSISSlide15

MARBLE BONE DISEASEIncrease in bone densityFailure

of normal osteoclast

function

(Number

of

osteoclasts … Bone

is

not

resorbed)

Key

elements necessary for osteoclast

function :

Proton pump

Chloride channel

Carbonic

anhydrase

II

Two major clinical patterns:Infantile osteopetrosisadult osteopetrosisSlide16

INFANTILE OSTEOPETROSISMalignant osteopetrosis

Autosomal recessive

Marrow failure

Frequent fractures

Cranial nerve compression

Initial signs

Normocytic anemia

Hepatosplenomegaly

(resulting from compensatory

extramedullary

hematopoiesis)

Granulocytopenia

:

Increased susceptibility to infection Slide17

Facial deformity Broad faceHypertelorismSnub nose

Frontal bossing

Tooth eruption : delayed

Narrowing skull foramina

Press on the cranial nerves

Optic nerve atrophy and blindness

Deafness

Facial paralysis

Pathologic fractures : common

Osteomyelitis : common complication of tooth extraction

Increase in skeletal density (distinction between cortical and

cancellous

bone is lost)Slide18

Intermediate osteopetrosisLess severe variants

Asymptomatic

at birth

Marrow failure

Hepatosplenomegaly

Transient

osteopetrosis

Radiographic evidence of diffuse sclerosis

Marrow failure

BUT

Resolve

without specific

therapy

Return

to

normalcySlide19

ADULT OSTEOPETROSISBenign osteopetrosisDiscovered later in life

Autosomal dominant

Long bones

Asymptomatic

Marrow failure

Two major variants

Cranial nerve compression … common / Fractures rare

Frequent fractures / Nerve compression … uncommon

Mandible

is involved, fracture and

osteomyelitis

after tooth extractionSlide20

Abnormal endosteal bone formationNumerous osteoclasts

Adult

osteopetrosis

: long-term survival

Infantile

osteopetrosis

: bone marrow transplantation

Interferon gamma-1b +

calcitriol

:

Reduce bone mass

Decrease infections

Lower nerve compression

Corticosteroids

Limiting calcium intake

Antibiotics Slide21
Slide22

CLEIDOCRANIAL DYSPLASIASlide23

Dental and clavicular abnormalitiesCBFA1 gene (RUNX2)

Odontogenesis

Odontoblast

differentiation

Enamel organ formation

Dental lamina proliferationSlide24

Clavicles Absent(unilaterally or bilaterally)…10%Hypoplasia Muscles : underdevelopedSlide25

Neck : longShoulders : narrow / unusual mobility Short statureLarge heads with pronounced frontal and parietal bossing

Hypertelorism

Broad base nose

Depressed nasal bridge

Wormian

bonesSlide26

Dental and jaw manifestationsSmall or absent maxillary sinusesMandibular prognathism

Narrow, high-arched palate

Cleft palate

Prolonged retention of deciduous teeth

Delay or complete failure of eruption of permanent teeth

Numerous

unerupted

permanent and supernumerary teeth (more than 60)Slide27
Slide28

Unerupted permanent teeth Lack secondary cementum Insufficient alveolar bone

resorption

is the reason

for impaired tooth eruptionSlide29

FOCAL OSTEOPOROTIC

MARROW DEFECTSlide30

Area of hematopoietic marrow that is sufficient in size May be confused with an intraosseous neoplasm

Aberrant bone regeneration after tooth extraction

Persistence of fetal marrow

Marrow hyperplasia in response to increased demand for erythrocytesSlide31

Asymptomatic Radiographic examinationRadiolucent lesionVarying in size

More than

75%

: adult women

70%

: posterior mandible (edentulous areas)

No expansion Slide32

Hematopoietic and/or fatty marrow

Incisional biopsy

Treatment : noSlide33

IDIOPATHIC OSTEOSCLEROSIS

Dense bone island

Bone scar

Enostosis

Focal

periapical

osteopetrosisSlide34

Focal area of increased radiodensity Unknown cause

Other bones

Nonvital

teeth or significantly inflamed pulps :

Condensing

osteitis

or focal chronic

sclerosing

osteomyelitis Slide35

Blacks and asiansLate first or early second decade

Slow increase in size

Asymptomatic

Cortical expansion : no

Routine radiographic examination

Mandible : 90% (first molar / second premolar / second molar )Slide36

Well-definedRoundedRadiodense mass (uniformly radiopaque OR nonhomogeneous)

Radiolucent rim : no

Associated with a root apexSlide37

MASSIVE OSTEOLYSIS

GORHAM DISEASE

VANISHING BONE DISEASE

PHANTOM

BONE

DISEASESlide38

Rare diseaseSpontaneous and usually progressive destruction of one or more bonesReplaced by a vascular proliferation…dense fibrous tissue

Cause … unknown

Proliferation of blood or lymphatic vessels that is occasionally

multicentricSlide39

Children and young adults (50% report of trauma) Maxillofacial : 30% Mandible Mobile teeth

Pain

Malocclusion

Deviation of the mandible

Deformity

Obstructive sleep apnea syndromeSlide40

Radiolucent foci of varying size with indistinct margins…become larger Loss of the lamina dura Thinning of the cortical plates

Mimc

periodontitis or

periapical

inflammatory diseaseSlide41
Slide42

Early stages : Nonspecific vascular proliferation intermixed with fibrous connective and a chronic inflammatory infiltrate Later stages : More

collagenized

Repair by new bone formation is not seenSlide43

Spontaneous arrest Mortality : UncommonBone graf

Radiation therapy is the most successful

Postirradiation

sarcomaSlide44

PAGET'S DISEASE OF BONESlide45

Abnormal resorption and deposition of boneDistortion and weakening of the affected bonesCause … unknown

Inflammatory

Genetic

Endocrine factors

Mutations

sqstml

(p62) / VCP gene

Virus infectionSlide46

CommonGeographic varianceBritainMen : whites

Older than 45 y/o

Asymptomatic disease :

Radiography

Elevation in serum alkaline phosphatase

Most cases :

polyostotic

Bone pain : common complaint

Most commonly affected bones : lumbar vertebrae, pelvis, skull, and femur

Simian (monkeylike) stanceSlide47

SkullIncrease in the circumference of the headMaxilla : more common / enlargement of the 1/3 faceLionlike

facial deformity

Alveolar ridges : symmetrical enlarged

Spacing of the teeth

Dentures no longer fit Slide48

RadiographicallyOsteolytic : radiolucentOsteoblastic

: radiopaque

(patchy /"cotton wool")

Hypercementosis

Bone

scintigraphy

:

entire mandible from condyle to condyle

,

a

black

beard or Lincoln's signSlide49

Resorption and formation of boneOsteoclasts activityOsteoblastic activity

Characteristic microscopic feature :

Basophilic

reversal lines

"jigsaw puzzle" or "mosaic" appearanceSlide50

Serum alkaline phosphataseBlood calcium and phosphorusUrinary hydroxyproline

Alkaline phosphatase is more than 25% to 50%

OsteosarcomaSlide51