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Dent 356 Laboratory

Inherited, Developmental,Inflammatory, & MetabolicDisorders of Bone

Dr. Huda Hammad

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Osteogenesis Imperfecta:Clinical Features

Sclerae may appearblue because they are

so thin that the choroidshows through.

Joint hypermobility withlax ligaments.

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Osteogenesis Imperfecta:Clinical Features

Dentinogenesisimperfecta may beassociated with some

cases.

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Osteopetrosis (Marble Bone Disease):Clinical Features

Two basic patterns:

1. Malignant type:ProgressiveAutosomal recessive

Occurs early in lifeSevere bone fragility andmalformationsDeath usually before puberty.

2. Benign type:Autosomal dominantLess severeDiagnosis may not be made untillate in life and incidentally.Repeated fractures following minortrauma

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Osteopetrosis (Marble Bone Disease)Radiographic Features

Increased density of skeleton.

Lack of distinction betweencortical and medullar bone.

Marked density of base ofskull.

Mandible more involved thanmaxilla.

Roots of teeth may beinvisible.

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Osteopetrosis (Marble Bone Disease):Histopathologic Features

Thickened cortices.

Reduced marrow

cavities.

Persistence of wovenbone.

Marked lack of maturelamellar bone.

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Cleidocranial Dysplasia (Cleidocranial Dysostosis):Clinical Features

Abnormalities of skull:Fontanelles and suturestend to remain open.Skull appears flat withprominent frontal, parietal,and occipital bones.Nasal bridge is depressed.

Maxilla may beunderdeveloped with a high,narrow arched palate.

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Cleidocranial Dysplasia (Cleidocranial Dysostosis):Clinical Features

Partial or complete

absence of claviclesallows shoulders to beapproximated until theymeet.

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Cleidocranial Dysplasia (Cleidocranial Dysostosis):Clinical Features

Dental abnormalities:Deciduous dentition tendsto be retained with delayedor non-eruption ofpermanent dentitionbecause of multipleimpactions.Supernumerary teeth anddentigerous cysts arecommon.

Roots tend to be thinnerthan normal.Secondary cementum issparse or absent on bothdentitions.

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Monostotic Fibrous Dysplasia of Bone:Clinical Features

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Monostotic Fibrous Dysplasia of Bone:Radiographic Features

Jaw lesions are variable inappearance, reflecting differingamounts of metaplastic boneformed within fibrous tissue.

Borders are difficult to definebecause of gradual transitionto normal.

Initially resemble cyst-likeradiolucencies containing faintbony trabeculae.

With increasing trabeculation,they become mottled andeventually opaque.

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Monostotic Fibrous Dysplasia of Bone:Radiographic Features

The many delicate trabeculae give aground-glass or orange-peel-stippling effect.

In some lesions, coarse mottling ofsmoke-screen pattern produced byirregular radiopaque masses lying ina radiolucent background.

In the maxilla, lesions may extendup to and distort, but do not crosssuture lines.

Roots of teeth in involved areas maybe separated and teeth may bedisplaced, but root resorption isexceptional.

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Polyostotic Fibrous Dysplasia of Bone:Clinical Features

May present as part of Albrightsyndrome:Very uncommon.Café-au-lait melanotic spots onskin.Precocious puberty in females.Occasionally other endocrineabnormalities.Premature skeletal maturation.Pigmentation of oral mucosareported.Occurs in males withoutprecocious puberty.

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Fibrous Dysplasia of Bone:Histopathologic Features

Replacement of normal bone byfibrous tissue containing islands andtrabeculae of metaplastic bone.

Appearances of jaw lesions aremore variable than in other bones.

Fibrous tissue may be richly cellularand show a whorled pattern, or mayconsist of thick, interlacing collagenbundles.

Newly formed bony trabeculae aredelicate and irregular (likened toChinese characters).

They consist of immature, coarse-fibered woven bone.

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Fibrous Dysplasia of Bone:Histopathologic Features

In jaw lesions, trabeculae may be thickerand blunter than in long bones.

Spherical areas of calcification resemblingcemetum may be present.

Osteoblastic and osteoclastic activity may

be seen in relationship to some trabeculae.

At the margins, lesional bone fuses withnormal bone and this particular featuresdistinguishes FD from ossifying fibroma.

Usually with increasing age, the amount andcellularity of fibrous tissue decreases andthe amount of bone increases.as the lesionmatures, there is progressive remodelling ofwoven bone to lamellar bone.

Occasionally, the lesion may be associatedwith development of aneurysmal bone cyst.

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Florid Cemento-osseous Dysplasia:Radiographic Features

Poorly demarcated,dense, lobular,

radiopaque masses.

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Cherubism

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Cherubism: Clinical FeaturesProgressive reduction in deformityas the patient passes from pubertyinto adult life.

Cosmetic surgery is often neededto deal with residual deformity.

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Cherubism: Radiographic Features

Sharply defined, multilocularradiolucencies.

Expansion and thinning ofcortical plates, evenperforation.

Mandibular lesions appear tobegin near the angle andspread to involve the body andramus.

Maxillary lesions are oftenconfined to tuberosities, butsinus may be obliterated.

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Cherubism: Histopathologic Features

Cellular and vascular fibroustissue containing varyingamounts of multinucleatedgiant cells.

The appearance is similar toother giant cell lesions of the jaws, and differentiationbetween them requires clinicaland radiographic information.

As the activity of the lesiondecreases, it becomes morefibrous, giant cell numberdiminishes, metaplastic boneis deposited.

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Focal Sclerosing (Condensing) Osteitis

Generally seen at rootapex, most commonly1 st permanent molar.

May remain afterextraction.

Usually asymptomatic.

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Suppurative Osteomyelitis:Clinical Features

Chronic:Discharge of pusthrough one or more

intraoral or extraoralsinuses.

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Suppurative Osteomyelitis:Radiographic Features

Normal in early stages.

In 10-14 days, sufficientbone resorption occursto produce irregular,moth-eaten areas ofradiolucency.

Sequestra may beseen.

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Suppurative Osteomyelitis:Histopathologic Features

Suppurative osteomyelitis,note the devitalized lamellarbone sequestrum withscalloped edges and absence

of stainable osteocytes andosteoblasts. An osteoclast in aresorption area is seen.

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Chronic Sclerosing OsteomyelitisA controversial condition:

- localized lesions are identicalto focal sclerosing osteitis.

- some previously reporteddiffuse types probably

represent infected floridcemento-osseous dysplasia.

However, diffuse sclerosinglesions of the mandible as acomplication of spread from

contiguous focus of low-gradeinfection/inflammation such asperiapical granuloma havebeen reported.

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Chronic Osteomyelitis with Proliferative Periostitis(Garré’s Osteomyelitis, Periostitis Ossificans)

Radigraphs show focalsubperiosteal overgrowth of bonewith smooth surface on outercortical plate.

The subperiosteal mass consistsof irregular trabeculae of activelyforming woven bone withscattered chronic inflammatorycells in fibrous marrow.

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Chronic Osteomyelitis with Proliferative Periostitis(Garré’s Osteomyelitis, Periostitis Ossificans)

Periosteal new boneformation (periostealreaction) / Onion-peelappearance.

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Radiation Injury and Osteoradionecrosis

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Radiation Injury and Osteoradionecrosis

Extensive osteomyelitiswith painful necrosis ofbone, often associated

with sloughing ofoverlying oral andsometimes facial softtissues may occur,

even years afterradiotherapy.

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Primary Hyperparathyroidism:Clinical Features*

Severe cases may variably be associated with:1. Bone pain.2. Bone cysts (osteitis fibrosa cystica).3.

Pathologic fractures.4. Brown tumors.5. Renal colics due to stones.6. Mental changes including depression, emotional

liability, poor mentation, and memory defects.7. Increased incidence of peptic ulcer.8. Chronic pancreatitis.9. Hypertension.

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Primary Hyperparathyroidism:Clinical Features*

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Primary Hyperparathyroidism:Clinical Features

Biochemical changes have to be demonstrated toconfirm diagnosis. They are:

1. Elevated parathomone level.2. Increased serum calcium level.3. Reduced serum phosphate level.

4. Increased urinary excretion of calcium andphosphate5. There may be elevated alkaline phosphatase.

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Primary Hyperparathyroidism:Histopathologic Features

Increased osteoclastic activitythroughout the skeleton.

Fibrosis of marrow (osteitisfibrosa).

Occasionally, focal areas ofbone resorption result information of lesions calledbrown tumors.

They are identical to othergiant cell lesions of the jaws.

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Primary Hyperparathyroidism:Radiographic Features

May show no detectablechanges or generalizedosteoporosis.

Brown tumors present assharply defined, round or ovalradiolucent areas.

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Primary Hyperparathyroidism:Radiographic Features

Partial loss of lamina dura around roots of teeth may occur.

Brown tumors present as sharply defined, round or oval radiolucentareas.

They may be multilocular.

They occur more frequently in the mandible than in the maxilla.

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Rickets and Osteomalacia

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Acromegaly