Bone Pathology Oral Pathology I Disease Def’n/Mode ofInheritance (KNOW!) Clinical Features Radiographic Features Oral Features Histo- pathological Features Treatment and Prognosis Extra Information Osteogenesis Imperfecta -AD, AR, spontaneous mutations -all affect collagen maturation -fragile bones, blue sclera, hypoacusis, bone deformities, joint hyperextensibility -osteopenia -bowing, angulation & deformity of long bones -multiple fx -Wormian bones in skull -clinically & radiographically identical to dentinogenesis imperfecta; termed opalescent teeth (1&2 dentitions involved; blue to brown translucence, pulpal obliteration) -often class III malocclusion -florid osseous dysplasia -attenuated bone -minimal matrix -immature woven bone -bone fracture management -teeth often show attrition -overall disability depends on severity of disease -there are 4 types ofOsteogenesis Imperfecta (we don’t need to know specifics for each type) Osteopetrosis -rare disease ofosteoclastic defect -Infantile osteopetrosis has AR inheritance -really is a BONE disease, not a tooth disease Infantile: -increased skeletal density (delayed tooth eruption) -Infantile ranges from malignant to intermediate to transient -Adult usually is benign -Osteosclerosis is NOT = osteopetrosis! sclerosis is more of an inflammatory dz Cleidocranial Dysplasia -AD and spontaneous mutation -defect ofosteoblastic differentiation -hypoplasia, malformation orabsence of clavicles -short stature, large heads, frontal bossing, ocularhypertelorism, depressed nasal bridge -open skull sutures, Wormian bones -increased prevalence of cleft palate -retained deciduous teeth -unerupted permanent & supernumerary teeth (lack secondary cementum) -narrow ascending mandibular ramus (may develop class III malocclusion) -IS significant dentally
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-Infantile ranges frommalignant tointermediate totransient-Adult usually is benign-Osteosclerosis is NOT= osteopetrosis!sclerosis is more of aninflammatory dz
Cleidocranial Dysplasia -AD andspontaneousmutation-defect of osteoblasticdifferentiation
-hypoplasia,malformation or absence of clavicles-short stature, largeheads, frontalbossing, ocular hypertelorism,depressed nasalbridge
-open skullsutures,Wormian bones
-increasedprevalence of cleftpalate-retained deciduousteeth-uneruptedpermanent &supernumerary teeth(lack secondarycementum)-narrow ascendingmandibular ramus
Focal Osteoporotic Marrow Defect Not a trueneoplasm!-may be aberrantbone regenerationfollowing EXT-persistent fetalmarrow-marrowhyperplasia tomeet increaseddemand for RBC
-incidentalfinding-panoramic viewmay appear radiolucent andwell defined-periapical viewshows ill definedborders and finetrabeculations-usually several
mm-cm
-normocellular hematopoieticmarrow
-Bx-dx (notreatment)
Idiopathic Osteosclerosis -Focal increasedradiodensity of unknown cause. Itcan’t be attributedto anyinflammatory,dysplastic,neoplastic or systemic disorder
-5% prevalence-slightly higher inBlacks, Asians-M = F-arises in late 1st,early 2nd decade-static or slowincrease in size-may have slowincrease in density
to 4th
decade-ALWAYSasymptomatic-90% mandible (1st M area, PM, 2nd M)
Aka: “bone scar”,“enostosis” -well defined,rounded or ellipticalradiodense area-3mm-2cm-no radiolucentrim-often at root
apex (typicallyplace of inflammation)
-may be multiple!
-dense lamellar bone
-no need tobiopsy, don’tusually changeover time.
-VERY VERY common
Condensing Osteitis -inflammation of bone; responds bylaying down morebone
-looks similar toidiopathicOsteosclerosisbut has aradiolucent rimaround the lesion
-characterized byabnormalresorption anddeposition of bone
-common dz inBritain, US. Rare in Africa & Asia-M>F, over 40-usually polyostotic-bone pain,osteoarthriticchanges-lumbar vert, pelvis,
skull, femur -thickened,enlarged, weakbones (bowing of long bones,enlargement of skull- hats don’t fit !!!)-max> mand(diastema form,dentures don’t fit!)
-mandible> maxilla(may cross midline in mandible, usuallyanterior)-may beasymptomatic or expand, pain,parasthesia,ulceration-nonagressivelesions (nosymptoms, slow
growth, noperforation or rootresorption)-aggressive lesions(pain, rapid growth,cortical perforation,root resorptioni,recurrence)
-can thin outcortex, resorb
the root, moveteeth out of position-radiolucent-unilocular or multilocular -well definedborders, butnoncorticated
-may beassociated with
aneurismal bonecyst, centralodontogenicfibroma*identical to browntumors of hyperparathyroidism*multifocal lesionssuggest cherubism(different clinically)-multinucleated
giant cells in abackground of spindle cells toovoid cells (mayshow hemosiderindeposits)-presence of giantcells doesn’tindicate GCG b/cthey’re present inmany other lesionsas well
variety of appearances, you canalmost always put it inyour differentialdiagnosis-Central Giant CellGranuloma, rule outhyperparathyroidism with blood tests(elevated serumcalcium in blood, or measure parathyroid
hormone)Giant Cell Tumor (different fromgranuloma!) is a:-long bone lesion-more aggressive-may becomemalignant
Cherubism -AD with highpenetrance,variableexpressivity
-usually manifests 2-5 yrs old-progresses tilpuberty, stabilized,regresses (limitsitself in adulthood)-cherublike face-upturned eyes-usually bilateralsymmetricalenlargement of post
mandible includingangle & ascendingrami
-multilocular,expansile,bilateralradiolucent areas-RARELYunilocular -RARELY other bones
-affects teeth-tooth displacement,failure to erupt
-multinucleatedgiant cells (likeGCG, browntumors) in avascular, fibrousstoma
-natural history isunpredictable-may regress after puberty-optimal therapyunknown (surgeryvs. observation)
-ALWAYS multifocal!How you distinguishcherubism from giantcell granuloma(unifocal unless it’s abrown tumor)-Syndrome Association: Ramon,Jaffe-Campanacci,Noonan-like
Simple Bone Cyst -benign, empty or fluid filled cavityw/in bone, w/oepithelial lining-theory that it’strauma induced(“traumatic bonecyst”)
-occurs in almostEVERY bone-common in jaw;mandible >> maxilla-10-20yrs of age-rare under age 5 or over 35-usually
asymptomatic (20%painless swelling)
-well delineatedradiolucent area(may be focally illdefined)-1cm-10cm-may scallop b/wteeth-vital teeth
-less commonlyexpansile-may occur inassociation withcemento-osseousdysplasia & other BFOL
-lined by thinfibrovascular CT(not lined byepithelium)
-bx-dx (no tx)-scant tissue frombiopsy-requires clinical/pathological/radiographiccorrelation“NO RAY NO
SAY”
Aneurysmal Bone Cyst -intraosseousaccumulation of blood filled spaces(hemorrhagic
extravasation)-may have giantcell granuloma-likeareas
-occurs in longbones andvertebrate-rare in jaws
-wide age range,mean ~20yrs-mandible> maxilla;posterior> anterior -rapidly growingswelling-may move teeth
-unilocular or multilocular radiolucent area-cortical
expansion &thinning-borders may bewell or poorlydefined-may have smallradiopaque foci
-teeth have normal enamel, thicker dentin, no pulp! Why they look translucent (The Tindle effect- how light is refracted as it passes through another material)
-lower x-rays show a different type of osteogenesis imperfecta where the teeth just have shells
Mesenchyme forms the dentin (how dentinogenesis imperfecta is related to osteogenesis imperfecta)
Osteopetrosis: text figures FIGURE 4-20Osteopetrosis. A, Ribs, humerus, and clavicles exhibit increased bone density. B, Increase in bone density of cranial and base-of-skull bones.
FIGURE 4-21Osteopetrosis. Panoramic radiograph demonstrating unerupted teeth and areas of increased bone density of the mandible and maxilla.
Massive Osteolysis: (aka Gorham's disease) Gorham's disease or vanishing bone disease can be severely disabling and 16% of cases are fatal. Gorham's disease is usuallymonocentric but locally aggressive, with resorption of the affected bone extending into the adjacent soft tissues
described the criteria for the diagnosis of massive osteolysis as:
1. Evidence of local progressive osseous resorption
2. Minimal or no osteoblastic response and an absence of dystrophic calcification
3. Non-expansile, non-ulcerative lesion
4. Absence of visceral involvement
5. Osteolytic radiographic pattern
6. Negative findings for a hereditary, metabolic, neoplastic, immunologic or infectious origin
Figure 1
Panoramic radiograph showing missing teeth in the right quadrant,resorption and decreased vertical height of the right mandibular
body; bone resorption extending to the mesial aspect of the leftcanine
Figure 2
Panoramic radiograph taken 3 months after initial panoramic radiographshowing progressive resorption of the right mandibular body and loss of
bone, encroaching on the mandibular canal space
Figure 3
Panoramic radiograph taken 4 months after the initial panoramic radiograph,with an arrow pointing towards a fracture in the right mandibular body withoverriding fragments, osteolysis extending onto the angle and the ramus with
Look up Differentials in Back of Textbook: supernumerary teeth! (distractor = something else that also has supernumerary teeth and give a reason to choose one over the other)