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AU'18 Oral Path II > Bone Disorders - part I > Flashcards

Bone Disorders - part I Flashcards

1
Q

inherited bone disorders

A
  1. osteogenesis imperfecta
  2. osteopetrosis
  3. cleidocranial dysplasia
  4. cherubism
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2
Q

acquired bone disorders

A
  1. osteitis deformans (Paget disease)
  2. fibro-osseous lesions of the jaw: fibrous dysplasia
  3. fibro-osseous lesions of the jaw: cemento-osseous dysplasia (COD)
  4. osteoporotic bone marrow defect
  5. idiopathic osteosclerosis
  6. simple bone cyst
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3
Q

osteogenesis imperfecta

A

“brittle bone disease”

several rare disorders of bone

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4
Q

what does defective collagen in osteogenesis imperfecta cause?

A

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

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5
Q

etioloigy of osteogenesis imperfecta

A

mutation in collagen gene

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6
Q

T/F: osteogenesis imperfecta can be autosomal dominant or recessive

A

true

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7
Q

T/F: a majority of osteogenesis imperfecta cases are autosomal recessive

A

false, 90% are autosomal dominant

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8
Q

T/F: some of the osteogenesis imperfecta are sporadic

A

true

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9
Q

T/F: osteogenesis imperfecta is one of the most common heritable bone diseases

A

true

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10
Q

why does the severity of osteogenesis imperfecta widely varies?

A

it depends on mutation

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11
Q

clinical features of osteogenesis imperfecta

A
  1. bones fragile, fracture easily; varies widely with the type of mutation
  2. blue sclerae
  3. hearing loss
  4. Bowing deformity long bones
  5. craniofacial alterations (CL III occlusion, triangular facies)
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12
Q

dental alterations of OI that are identical to dentinogenesis imperfecta

A
  1. blue, yellow or brown translucence (more noticeable in primary teeth)
  2. opalescent teeth
  3. severe attrition leading to loss of VDO and potential tooth loss
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13
Q

T/F: OI and dentinogenesis imperfecta can have similar tooth alterations and blue sclerae, but they are distinct mutations, different diseases

A

true

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14
Q

dental defects associated with OI should be designated as what?

A

“opalescent teeth”

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15
Q

T/F: “dentinogenesis imperfecta” is reserved for alterations isolated to the teeth

A

true

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16
Q

T/F: OI can appear identical to dentinogenesis imperfecta radiographically

A

true

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17
Q

radiographic features of osteogenesis imperfecta

A
  1. “shell teeth”, or premature pulpal obliteration

2. narrow or “corn cob” shaped roots

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18
Q

treatment for osteogenesis imperfecta

A
  1. physiotherapy/rehabilitation
  2. orthopedic surgery
  3. minimize factors leading to fracture
  4. IV bisphosphonates for children with moderate-severe pain
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19
Q

why are IV visphosphonates indicated for children with moderate-severe pain?

A

reduce fracture rates

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20
Q

treatment for dentition of osteogenesis imperfecta

A
  1. crown/bridge
  2. partial or complete dentures
  3. ± implants
  4. orthognathic surgery
  5. orthodontics
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21
Q

prognosis of osteogenesis imperfecta depends on what?

A
  1. mutation

2. expression of the gene

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22
Q

what is the range of prognosis for osteogenesis imperfecta?

A

ranges from minimal bone deformity with essentially normal growth to severe forms leading to death from passage through the birth canal

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23
Q

T/F: osteopetrosis have several forms, both autosomal dominant and recessive

A

true

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24
Q

T/F: autosomal dominant form of osteopetrosis is more severe

A

false, recessive more severe

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25
Q

what happens when osteoclasts fail to fxn normally?

A

bone is not resorbed

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26
Q

what will happen if bone continued to form and ossify in osteopetrosis?

A

increased bone density

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27
Q

pancytopenia

A

loss of hematopoietic precursor cells

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28
Q

what happens when pancytopenia occurs in osteopetrosis?

A

increased susceptibility to infections and osteomyelitis

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29
Q

T/F: osteopetrosis is rare

A

true

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30
Q

clinical features of osteopetrosis

A
  1. blindness and deafness due to CN compression
  2. fractures
  3. osteomyelitis
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31
Q

what is the cause of frequent complication of tooth extraction in osteopetrosis pts?

A

osteomyelitis

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32
Q

radiographic findings of osteopetrosis

A
  1. diffuse density of skeleton
  2. marrow spaces filled in by dense bone
  3. tooth roots difficult to visualize due to surrounding dense bone
  4. failure of tooth eruption
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33
Q

tx of osteopetrosis

A
  1. supportive measures
  2. bone marrow transplant
  3. alternative therapies
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34
Q

supportive measures for osteopetrosis

A

transfusions and abx when necessary

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35
Q

alternative therapies for osteopetrosis

A
  1. interferon with calcitriol
  2. restriction of Ca2+ intake
  3. steroids
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36
Q

T/F: autosomal dominant form of osteopetrosis can have long-term survival

A

true

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37
Q

what is the prognosis for autosomal recessive form of osteopetrosis?

A

poor

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38
Q

T/F: patients with the autosomal recessive form of osteopetrosis usually die before 20 y.o.

A

true

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39
Q

T/F: cleidocranial dysplasia is autosomal recessive

A

false, autosomal dominant

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40
Q

T/F: spontaneous mutations have also been reported for cleidocranial dysplasia

A

true

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41
Q

T/F: investigators have proposed an autosomal recessive form of cleoidcranial dysplasia

A

true

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42
Q

T/F: cleidocranial dysplasia is common

A

false, uncommon

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43
Q

where does the cleidocranial dysplasia primarily affect?

A
  1. skull
  2. jaws
  3. clavicles
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44
Q

clinical features of cleidocranial dysplasia

A
  1. prominent forehead, hypoplastic midface
  2. long neck
  3. shoulders narrow and drooping (hypoplastic or missing clavicles)
  4. hypermobility of shoulders
45
Q

dental findings of cleidocranial dysplasia

A
  1. primary dentition retained because permanent teeth don’t erupt
  2. numerous impacted and supernumerary teeth (i.e. plenty of teeth; not erupting in the correct space, or, not erupting at all)
46
Q

tx for cleidocranial dysplasia

A

combined surgical and orthodontic care to

  1. correct skeletal relations
  2. remove supernumerary teeth
  3. correct alignment of permanent teeth
47
Q

prognosis of cleidocranial dysplasia

A

good

48
Q

T/F: life span of ppl with cleidocranial dysplasia is essentially normal

A

true

49
Q

T/F: cherubism is autosomal recessive

A

false, autsomal dominant

50
Q

T/F: cherubism has limited expressivity

A

false, variable

51
Q

when is cherubism detected?

A

in childhood

52
Q

clinical features of cherubism

A
  1. painless bilateral expansion of jaws, especially mandible
  2. chubby cheeks
  3. involvement of inferior and/or lateral orbital walls may tilt the eyeballs upward and retract lower eyelid “eyes upturned to heaven”
53
Q

T/F: cherubism involves the maxilla more frequently

A

false, less frequently

54
Q

radiographic features of cherubism

A
  1. bilateral multilocular radiolucencies posterior mandible, maxilla
  2. often significant displacement of teeth
55
Q

T/F: cherubism is occasionally appears unilocular in radiographs

A

true

56
Q

histopathologic features of cherubism

A
  1. cellular fibrous CT
  2. sparse benign-appearing multinucleated giant cells
  3. may see perivascular hyalinization
57
Q

tx for cherubism

A
  1. optimal tx has not been determined

2. surgical intervention sometimes accelerates growth of lesions

58
Q

T/F: many cherubism cases involute during puberty

A

true

59
Q

T/F: pts with cherubism will have persistent facial deformity or continued disease progression

A

true

60
Q

etiology of osteitis deformans (Paget disease)

A

unknown

61
Q

osteitis deformans (Paget disease)

A

abnormal resorption and deposition, resulting distored, weaker bone

62
Q

what happens to the affected bones in Paget disease?

A

becomes thickened

63
Q

T/F: Paget disease is often discovered incidentally on routine blood test or dental radiographs

A

true

64
Q

Paget disease is primarily of what ancestry?

A

Anglo-Saxon

65
Q

T/F: Paget disease has a 2:1 female predilection

A

false, 2:1 MALE predilection

66
Q

who is affected by Paget disease?

A

older pts, rare <40 y.o.

67
Q

40% of Paget disease cases have what in common?

A

bone pain

68
Q

clinical features of Paget disease

A
  1. “Simian” (monkey-like) stance if femurs involved
  2. elevated serum alkaline phosphatase
  3. skull involvement - progressive enlargement, “hate won’t fit”
69
Q

what happens if femur is involved in Paget disease?

A

bowing of legs

70
Q

T/F: most Paget disease cases are polyostotic

A

true

71
Q

what happens to the jaw if involved in Paget disease?

A

thickened, enlarged alveolar bone “dentures don’t fit”

72
Q

radiographic features of Paget disease

A
  1. “cotton wool” appearance of bone

2. may have extensive hypercementosis

73
Q

histopathologic features of Paget disease

A
  1. “Mosaic” pattern of irregular trabeculae with resting and reversal lines
  2. marrow replaced by vascular fibrous CT
74
Q

tx for Paget disease if asymptomatic

A

no tx

75
Q

Paget disease if symptomatic

A

bisphosphonates

76
Q

T/F: Paget disease is chronic and progressive, but usually not life-threatening

A

true

77
Q

what should pts with Paget disease be monitored for?

A

development of a giant cell tumor or malignant tumors esp osteosarcoma

78
Q

why would an extraction be difficult on pts with Paget disease?

A

difficulty extracting teeth w hypercementosis and/or ankylosis

79
Q

what is a dental complication during the vascular/lytic phase for pts with Paget disease?

A

hemorrhage

80
Q

poor wound healing in pts with Paget disease increases susceptibility to what during avascular/sclerotic phase?

A

osteomyelitits

81
Q

T/F: new dentures may be required periodically for pts with Paget disease

A

true

82
Q

T/F: implant placement is generally favorable for pts with Paget disease

A

false, unfavorable and more so with use of bisphosphonates

83
Q

etiologies of fibro-osseous lesions

A
  1. developmental (hamartomatous)
  2. reactive
  3. dysplastic
  4. neoplastic
84
Q

fibro-osseous lesions

A
  1. fibrous dysplasia

2. cemento-osseous dysplasia (COD)

85
Q

T/F: fibrous dysplasia is a post-zygotic mutation

A

true

86
Q

post-zygotic mutation of fibrous dysplasia

A
  1. pluripotent stem cell
  2. skeletal progenitor cell
  3. post-natal life
87
Q

what determines the extent of fibrous dysplasia

A

time of occurrence of mutation

88
Q

when does fibrous dysplasia presents?

A

~1st-2nd decade (younger than Paget disease)

89
Q

T/F fibrous dysplasia has a female predilection

A

false, NO gender predilection

90
Q

T/F: fibrous dysplasia can be polyostotic or monostotic, but a majority of the cases are polyostotic

A

false, 75-80% monostotic

91
Q

clinical features of fibrous dysplasia

A
  1. painless, unilateral swelling
  2. slow growth
  3. craniofacial fibrous dysplasia
92
Q

which bone is most commonly affected by fibrous dysplasia?

A

jaws

93
Q

T/F: mandible is more often affected by fibrous dysplasia than maxilla

A

false, maxilla > mandible

94
Q

craniofacial fibrous dysplasia lesions may involve what?

A

adjacent facial bones

95
Q

what may craniofacial fibrous dysplasia result in?

A

marked facial deformity

96
Q

how are the early stages of fibrous dysplasia seen radiographically?

A

radiolucent or mottled

97
Q

radiographic features of fibrous dysplasia

A
  1. opacify as they grow
  2. obliteration of maxillary sinus
  3. classic “ground glass” pattern
  4. poorly defined, blending margins
98
Q

what are the 2 types of polyostotic fibrous dysplasia?

A
  1. Jaffe type

2. McCune-Albright type

99
Q

Jaffe type of polyostotic fibrous dysplasia

A

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

100
Q

McCune-Albright type of polyostotic fibrous dysplasia

A

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

101
Q

what is the result of endocrine disturbances in McCune-Albright type of polyostotic fibrous dysplasia

A

precocious puberty

102
Q

histopathologic features of fibrous dysplasia

A
  1. irregular-shaped trabeculae of immature (woven) bone in cellular fibrous stroma
  2. no capsule - abnormal bone fuses to adjacent normal bone
103
Q

tx for fibrous dysplasia if lesion(s) are small

A

none

104
Q

when should surgery be delayed for fibrous dysplasia?

A

until disease quiescent

105
Q

tx for fibrous dysplasia

A
  1. surgical reduction if cosmetic or functional problem

2. en-bloc resection

106
Q

what is contraindicated in txing fibrous dysplasia?

A

radiation

107
Q

T/F: sometimes fibrous dysplasia stabilizes with skeletal maturation

A

true

108
Q

T/F: surgically treated fibrous dysplasia lesions show regrowth esp in younger pts

A

true

109
Q

T/F: malignant transformation of fibrous dysplasia is rare ans is usually in lesions which have received radiation

A

true

AU'18 Oral Path II (31 decks)

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