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oral path 2 > 3 - bone disorders > Flashcards

3 - bone disorders Flashcards

1
Q

4 inherited bone disorders

A

osteogenesis imperfecta

osteopetrosis (denser)

cleidocranial dysplasia

cherubism

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2
Q

5 acquired bone disorders

A 
osteitis deformans (pagets)
fibro-osseous lesions of the jaw (fibrous dysplasia, cemento-osseous dysplasia COD)
osteroporotic bone marrow defect
idiopathic psteosclerosis 
simple bone cyst
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3
Q

90% autosomal dominant inherited “brittle bone disease”

several RARE disorders of bone

defective collagen > abnormal bone mineralization > low bone density(osteopenia)

A

osteogenesis imperfecta

big spectrum, defective collagen gene

some sporadic cases
most common binherited bone disease

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4
Q

most common bone disorder and severity is on a spectrum

A

osteogenesis imperfecta

90% AD

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5
Q

blue sclerae
hearing loss
bones fragile and fracture easily - varies widely with the type of mutation

A

osteogenesis imperfecta

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6
Q

craniofacial alterations Class 3 occlusion, triangular face

bowing deformity long bones

A

osteogenesis imperfecta

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7
Q

osteogenesis imperfecta dental alterations identical to _

3 things

A

dentinogenesis imperfecta

blue, yellow, brown TRANSLUCENCE (more noticeable in primary teeth)

opalescent teeth

severe attrition leading to loss of VDO and potential tooth loss

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8
Q

osteogenesis imperfecta and dentinogenesis imperfecta can have similar tooth alterations and blue sclerae, but they are

the same or different mutations

A

they are distinct mutations, different diseases

dentinogenesis imperfecta is reserved for alterations isolated to the teeth

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9
Q

dental defects associated with OI should be designated as _ teeth

A

opalescent teeth

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10
Q

radiographic features

“shell teeth” or premature pulpal obliteration

narrow or “corn cob” shaped roots

A

Osteogenesis Imperfecta or dentinogenesis imperfecta

shell teeth - normal enamel thickness, thin dentin

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11
Q

tx for osteogenesis imperfecta

A

physiotherapy/rehab
orthopedic surgery
minimize factors leading to fracture
IV bisphosonates for children with mod/severe pain, reduce fracture rates

crown/bridge
full/partial dentures
implants(possibly IV bisphos prob not)

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12
Q

osteogenesis impefecta Px

depends on the mutation and expression of the gene
fractures bones heal - usually with _ (weaker)

A

abnormal callus formation

ranges from minimal bone deformity to essesntially normal growth to severe forms leading to death from passage thru the birth canal

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13
Q

Several forms - autosomal dominant and recessive (recessive more severe)
failure of osteoclasts to fxn normally - bone is not resorbed

continued bone formation and ossification - increased bone density

A

osteopetrosis

loss of hematopoietic precursor cells (pancytopenia)

increased susceptibility to infections and osteomyelitis

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14
Q

RARE
blindness and deafness due to cranial nerve compression
fractures
osteomyelitis (frequent complication of tooth extraction)

A

osteopetrosis - fucked up fxn of osteoclasts - densebone

tooth eruption is often delayed

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15
Q

radiograhic findings

diffuse density of skeleton
marrow spaces filled in by dense bone
tooth roots diffiuclt to visualize due to surrounding dense bone
failure of tooth eruption

A

osteopetrosis

uniformly sclerotic bone, failure of tooth eruption

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16
Q

TX: supportive measures (transfusions antibiotics)
bone marrow transplant - limit success
alternative therapies - interferon with calcitriol, restriction of Ca++ intake, steroids

A

osteopetrosis

AD can have longterm survival

poor Px for autosomal recessive form - usually die before 20years old

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17
Q

AD
spontaneous mutations up to 40%
also autorecessive as well

uncommon, affects skull, jaws and clavicles primarily

prominent forehead, hypo plastic face

A

cleidocranial dysplasia

long neck

shoulders narrow and droppings (hypoplastic or missing clavicles)

hypermobility of shoulders

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18
Q

primary dentition retained because permanent teeth do not erupt

numerous impacted and supernumerary teeth

plenty of teeth; not erupting in the correcting space or not erupting at all

A

cleidocranial dysplasia

prominent forehead, hypoplastic midface, long neck, dropping shoulders, missing clavicles

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19
Q

combined surgical and orthodontic care

correct skeletal relations, remover supernumerary teeth, correct alignment of permanent teeth

Px - good, life span is essentially normal

A

cleidocranial dysplasia

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20
Q

AD
spontaneous mutations occur

variable expressivity - lesions may cause tooth displacement, tooth mobility, failure of tooth eruption, impaired mastication, speech difficulties, upper airway obstruction, and vision or hearing loss

A

Cherubism

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21
Q

detected in childhood (2-7)
painless bilateral expansion of jaws, especially mandible

chubby cheeks

A

cherubism

chubby cheeks - cherubs depicted in Renaissance etchings

maxilla involved less frequently

involvement of inferior and/or lateral orbital wals may tilt the eyeballs upward and retract lower eyelid - “eyes upturned to heaven”

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22
Q

radiographic features

bilateral multiocular radiolucencies posterior mandible, maxilla

occasionally unilocular

often significant displacement of teeth

A

Cherubism

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23
Q

histopathologic features

cellular fibrous CT
sparse benign-appearing multinucleated giant cells

may see perivascular hyalinization 9 eosinophilic cuffing)

A

cherubism

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24
Q

Cherubism tx

A

optimal tx has not been determined

surgical intervention sometimes accelerates growth of lesions

many cases involute during puberty

px - difficult to predict for any given case
persistent facial deformity or continued disease progression possible

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25
Q

unknown etiology (genetic? envirconmental? virus?)
abnormal resorption and deposition, resulting distorted, weaker bone
affected bones become thickened

A

Osteitis deformans (paget disease)

acquired bone disorders

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26
Q

often discovered incidentally on routine blood test or dental radiographs, elevated serum alkaline phosphatase

primarily Anglo-Saxon ancestry

2:1 male or female predilection

A

Osteitis deformans (paget)

2;1 male predilection, older patients

rare <40

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27
Q

“Simian” monkey like stance if femurs involved - bowing of legs

most cases polyostotic

bone pain in up to 40% of cases

A

Osteitis deformans - paget

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28
Q

skull involvement - progressive enlargement, “hat won’t fit”

jaws involved 17% of cases - thickened, enlarged alveolar bone “dentures don’t fit”

A

acquired Osteitis deformans - pagets

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29
Q

radiographic features

“cotton wool” appearance of bone - “moth-eaten”

may have extensive hypercementosis

A

Osteitis deformans - pagets

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30
Q

histopathologic features:
“Mosaic” pattern of irregular trabeculae with resting and reversal lines (from abnormal resorption and deposition)

osteoblastic rimming

marrow replaced by vascular fibrous CT

A

osteitis deformans (paget)

osteitis deformans

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31
Q

tx for osteitis deformans - pagets

A

if asymptomatic - no tx

bisphosphonates

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32
Q

Osteitis deformans - pagets

Px - chronic and progressive, but usually no life-threatening

patients should be monitored for development of a giant cell tumor or malignant tumors, especially _

A

especially osteosarcoma

33
Q

difficulty extracting teeth with hypercementosis and/or ankylosis
Hemorrhage during vascular/lytic phase

poor wounding healing, increased susceptibility to osteomyelitis during avascular/sclerotic phase

A

osteitis deformans - pagets

new dentures may be required periodically

although successful implant placement reported in a few cases, Px generally unfavorable and more so with use of bisphosphonates

34
Q

diverse group varying etiologies

developmental(harmartomatous)
reactive
dysplastic
neoplastic

A

fibro-osseous lesions

fibrous dysplasia

cemento-osseous dysplasia COD (periapical, focal, florid)

35
Q

Fibro-osseous lesions

post-zygotic mutation: pluripotent stem cell, skeletal progenitor cells, post-natal life

time of occurrence of mutation determines extent

A

Fibrous dysplasia

36
Q

Fibro-Osseous lesions

presents ~1st or 2nd decade (younger than Paget disease)
no gender predilection

polyostotic or monostotic (many or one bone)

75-80% monostotic

A

Fibrous Dysplasia

37
Q

Fibrous-osseous lesions

painless, unilateral swelling

slow growth

jaws among most commonly affected bones

MAXILLA more than mandible

A

fibrous dysplasia

craniofacial lesions may involve adjacent facial bones
results in marked facial deformity

38
Q

radiographic features

early stages radiolucent or mottled
opacify as they grow
obliteration of maxillary sinus 
class "ground glass" pattern 
poorly defined, blending margins
A

fibrous dysplasia of fibro-osseous lesions

39
Q

2 types of polyostotic fibrous dysplasia type

2 or more bones affected, and cafe-au-lait spots with jagged borders (coast of Maine)

A

Jaffe type Fibrous Dysplasia

40
Q

Fibrous dysplasia type

2 or more bones affected by fibrous dysplasia, cafe-au-lait spots and endocrine disturbances - precocious puberty

A

McCune-Albright type

41
Q

Histopathologic features

irregular-shaped trabeculae of immature (woven) bone in cellular fibrous stroma “Chinese characters”

no capsule - abnormal bone fuses to adjacent normal bone

A

fibrous dysplasia

42
Q

TX Fibrous Dysplasia
none if lesion is small

delay surgery until disease _

radiation CONTRAINDICATED

A

delay surgery until disease quiescent

surgical reduction if cosmetic or fxnal problem

En-bloc resection

43
Q

Px
sometimes disease stabilizes with skeletal maturation
spontaneous regression has been reported
25%-50% surgically treated lesions show regrowth, especially younger pts

malignant transformation rare, usually in lesions which have received radiation

A

fibrous dysplasia

44
Q

Proteus syndrome is _ man disease

A

elephant man disease

45
Q

Fibro-osseous lesions

benign, possibly reactive process
may originate from fibroblasts of PDL vs defect in bone remodeling

most common fibro-osseous lesion encountered in clinical practice of dentistry

A

Cemento-osseous dysplasia COD

acquired bone disorder

periapical COD
Focal COD (most common white female)
Florid COD

term may be changing to “osseous dysplasia” instead of COD

46
Q

Fibrous-osseous dysplasia

Biopsy multiple small gritty fragments obtained
most common in females but can affect both genders and any ethnic groups

A

cemento osseous dyspalsia - more common in female

black> east asian>white

focal type reported to be more common in white females

47
Q 
usually found incidentally on x-ray
tooth-bearing areas of jaws
asymptomatic 
swelling, discomfort unusual
TEETH TEST VITAL
A

COD - cemento-osseous dysplasa

48
Q

radiographic features

ranges from completely radiolucent to densely radiopaque with a thin radiolucent rim (PDL remains intact)

may be associated with simple bone cyst

A

Cemento-osseous dysplasia C)D

49
Q

_ COD shows multiple cotton wool type radiopacities in at least 2 quads of the jaws
may

A

Florid COD

Focal - white women

50
Q

Cemento-osseous dysplasia - COD spectrum of severity

A

Periapical - mild

focal - moderate (white women)

Florid - severe - cotton wool in 2 quads

51
Q

_ COD

mandibular anterior region usually, maxillary anterior as well

middle-aged black females especially

initially unilocular radiolucencies at apices, central opacity develops gradually

asymptomatic

A

Periapical COD - mild

periapical COD can be confused with hypercementosis(radiodensity is all within the PDL),

idiopathic osteosclerosis (no radiolucent rim, borders blend with surrounding trabeculae)

, benign cementoblastoma (radiolucent rim is contigous with PDL and PDL is not intact at he involved portion of the root)

52
Q

_ COD

more often affects white females; may be biased by studies
body of mandible
most common 20-40 younger demographic
unilocular radioluceny or radiopacity with thin radiolucent rim
asymptomatic

A

focal COD

can be confused with an ossifying fibroma, a true neoplasm, but focal COD is seen more often (similar demographic - younger adult women)

53
Q

_ COD

middle-age or older black females
multiple quads of jaws affected
generally asymptomatic, unless overlying mucosa ulcerates, resulting in bony sequestration (eg from ill-fitting denture)

A

Florid COD - most severe

yellowish, avascular cementum-like material is beginning to exfoliate thru the oral mucosa

DENTAL IMPLANT PLACEMENT NOT RECOMMENDED

lesions tend to be hypovascular (prone to necrosis, infection, osteomuelitis with minimal provocation)
recuded ability to heal

54
Q

Histopathologic features:

cellular fibrous CT with embedded mineralize tissue resembling either immature(woven) bone or cellular cementum
fragmented specimen
mineralized product resembles GINGER ROOT

A

cemento-osseous dysplasia

mature lesions have more mineralized product than cellular stroma

FLORID COD can show densely mineralized tissue with necrotic debris and inflammation

55
Q

Cemnto-osseous dysplasia dx based on clinical and radiographic feactures

can be confirmed by Bx if indicated

A

ginger root

vital teeth

spicules of bone and cementum like hard tissue within moderately cellular fibrous CT

56
Q

TX for COD

none indicated for _ COD

regular vists for dental prophy and OHI to prevent periodontal disease and need for extractions

good fit of dentures

A

none indicated for periapical COD

Bx for focal to ruleout other disease, Bx not necessary for Florid COD

encourage patients to retian their teeth
ideally avoid surgical procedures - onset of symptoms asssociated with exposure to sclrotic bone to oral cavity

57
Q

tx for COD symptomatic patient with secondary osteomyelitis

A

is difficult

debridement
antibiotics - often not effective
CHX rinse

58
Q

fibro-osseous lesions COD Px
excellent for _ and _, although initial appearance of _ COD may be first sign of _ COD

generally good fo _ COD

COD malginant transformation common or rare?

A

excellent Px for periapical and focal

focal might be first sign of florid

generally good for florid

guared if secondarily infected requireing debridement and ATB

reported, but RARE

59
Q

area of hematopoietic bone marrow of sufficient size to cause a radiographic radiolucency

pathogenesis unknown
may resemble metastatic disease

A

acquired

osteoporotic bone marrow defect

60
Q 
usually posterior body of mandible 
often at old extraction site
usually middle age FEMALE
incidentally on radiograph 
asymptomatic radiolucency
A

osteoporotic bone marrow defect

radiolucency can appear circumscribed, but on closer inspection may show ill-defined borders and a fine trabecular pattern

61
Q

radiolucency can appear circumscribed, but on closer inspection may show ill-defined borders and a fine trabecular pattern, old extraction site, females,

A

osteoporotic bone marrow defect

62
Q

histopathologic features

fatty and hematopoietic marrow
no abnormal osteoblastic or osteoclastic activity
maybe megakaryocytes

A

osteoporotic bone marrow defect

63
Q

Osteoporotic bone marrow defect Tx and Px

A

Bx often indicated to establish Dx

Px - excellent once Dx’d, no further tx needed

64
Q

dense bone island, Enostosis, bone whorl, focal periapical osteopretrosis, bone scar

A

Idiopathic osteosclerosis

65
Q

focally increased area of dense bone
unknown cause
usually found incidentally on radiograph
asympotmatic

remain static or slowly enlarge - usually stabilize at skeletal maturity (occasionally regress)

A

acquired
Idiopathic osteosclerosis

no gender predilection
no expansion
3rd decade!!

66
Q

radiographic features

radiopaque!
mandibular premolar/molar area most common site

borders blend with surround trabeculae, BUT occasionally may be sharp

A

Idiopathic osteosclerosis

borders will be blended for the most part with surround trabecular bone

67
Q

does Idiopathic sclerosis cause tooth displacement

A

rarely does

usually does not

lamina dura and PDL fine - vital teeth

68
Q

idiopathic osteosclerosis and fibrous dysplasia are well or poorly defined on CT

A

IO - well defined on CT usually, blended on xray

fibrous dysplasia is poorly defined on CT

69
Q

in the past this acquired bone disorder was not distinguished from inflammatory or other lesions; so it may be confused with: condensing osteitis, hypercementosis, cementoblastoma

A

idiopathic osteosclerosis

condensing osteitis - big lesion appearing pulp and widen PDL change inflammation bone at apex

hypercementosis - within PDL

cementoblastoma - PDL around root of tooth and surround lesion - classic circle dome of endo

70
Q

Histopatholgoic features

dense vital bone
may see fibrofatty marrow

A

Idiopathic osteosclerosis

71
Q

tx for idiopathic osteosclerosis

none indicated unless _ or _

Px?

A

none indicated unless symptoms or cortical expansion

if noted in childhood, periodic radiographs until lesion stabilizes, Bx if confirmation needed to establish Dx

Px = excellent, not likely to progress or change in adulthood

72
Q

empty or fluid filled bone cavity
lacks an epithelial lining, therefore a pseudocyst
usually an incidental findings

A

simple bone cyst

other terms traumatic bone cyst, hemorrhagic bone cyst

73
Q

etiology of simple bone cyst

A

unknown - many theories

trauma-hemorrhage theory - trauma causing hematoma but not fracture - without subsequent organization and repair of hematoma - instead liquefies

trauma not a consistent finding

74
Q

seen in 1st or 2nd decade

no gender predilection in jaws but in other bones, male
posterior mandible and symphysis
typically asymptomatic and no expansion

A

simple bone cyst

75
Q

radiographic features

well delineated
usually unilocular, can be multilocular
often scallops betwen roots( sugggestive but not dx)

A

simple boen cyst

76
Q

histopatholgical features

difficult to obtain specimen; usually just fragments of bone
microscopically these bone fragments are lined by inflamed granulation tissue, NO EPITHELIAL lining

loose CT and Bone

A

simple bone cyst

“teenage male with radiolucenyc right mandible”

77
Q

simple bone cyst tx and Px

A

surgical exploration and curetage to induce bleeding (empty cavity within bone is found at time of surgery)
causes hemorrhage which organizes and lesions heals

Px = normal radiographs ~12-17 months postop
periodic radiographs warranted until complete resolution
recurrence rate low - up to 27% though

78
Q

patients with _ disease have an increased risk of malignant tumors, especially osteosarcoma

A

Paget disease

oral path 2 (7 decks)

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