Bone Disorders Flashcards

1
Q

4 Inherited Bone Disorder

A
  1. Osteogenesis Imperfecta
  2. Osteopetrosis
  3. Cleidocranial Dysplasia
  4. Cherubism
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2
Q

5 Acquired Bone Disorders

A
  1. Osteitis Deformans (Paget Disease)
  2. Fibro-Osseous Lesions of the Jaw (Fibrous dysplasia and Cemento-osseous dysplasia)
  3. Osteoporotic bone marrow defect
  4. Idiopathic osteosclerosis
  5. Simple bone cyst
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3
Q

“Brittle bone disease”

A

Osteogenesis Imperfecta

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4
Q

Osteogenesis Imperfecta has to do with defective ____

A

Collagen

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5
Q

Defective collagen –> Abnormal bone ____ –> Low bone ____

A

Mineralization

Density (osteopenia)

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6
Q

Most cases of Osteogenesis Imperfecta are autosomal ____

A

Dominant

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7
Q

One of the most heritable bone disorders

A

Osteogenesis Imperfecta

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8
Q

Clinical features of Osteogenesis Imperfecta

A
  1. Bones fragile
  2. Blue sclera
  3. Hearing loss
  4. Bowing deformity
  5. Craniofacial alterations
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9
Q

Dental alterations in Osteogenesis Imperfecta are identical to ____ _____

A

Dentinogenesis Imperfecta

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10
Q

3 dental alterations in Osteogenesis Imperfecta

A
  1. Blue/Yellow/Brown translucence
  2. Opalescent teeth
  3. Severe attrition leading to loss of VDO and potential tooth loss
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11
Q

Dental defects associated with Osteogenesis Imperfecta should be designated as ___ ___

A

Opalescent teeth

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12
Q

Term reserved for alterations isolated to the teeth

A

Dentinogenesis imperfecta

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13
Q

Radiographic features of Osteogenesis Imperfecta

A

“Shell teeth” or premature pulpal obliteration

Narrow or “corn-cob” shaped roots

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14
Q

Treatment for dentition of Osteogenesis Imperfecta patients

A

Crown/bridge, partial/complete dentures/ implants/ orthognathic surgery, orthodontics

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15
Q

Prognosis of Osteogenesis Imperfecta

A

Variable

Minimal bone deformity –> death from passing through birth canal

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16
Q

Is the autosomal dominant form or the autosomal recessive form of Osteopetrosis more severe?

A

Recessive

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17
Q

Osteopetrosis is the failure of ___ to function normally

A

Osteoclasts (bone is not resorbed)

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18
Q

Continued bone formation and ossification in Osteopetrosis leads to

A

Increased bone density

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19
Q

Osteopetrosis involves the loss of ____ ___ cells

A

Hematopoietic precursor cells (pancytopenia)

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20
Q

Because of the loss of hematopoietic precursor cells in Osteopetrosis, there is is an increased susceptibility to ___ and ____

A

Infections and osteomyelitis

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21
Q

If there is CN compression, then patients with Osteopetrosis may experience ___ and ____

A

Blindness and deafness

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22
Q

____ is a frequent complication of tooth extraction in patients with Osteopetrosis

A

Osteomyelitis

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23
Q

____ ____ is often delayed with Osteopetrosis

A

Tooth eruption

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24
Q

Radiographic features of Osteopetrosis

A

Diffuse density of skeleton
Marrow spaces filled by dense bone
Tooth roots difficult to visualize
Failure of tooth eruption

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25
Q

Prognosis for autosomal dominant vs. autosomal recessive Osteopetrosis

A

Poor for recessive

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26
Q

Uncommon bone disorder that affects the skull, jaws, and clavicles primarily

A

Cleidocranial dysplasia

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27
Q

Patients with cleidocranial dysplasia often have a prominent ____, and _____ midface

A

Forehead

Hypoplastic

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28
Q

Describe the neck and shoulders of a patient with Cleidocranial dysplasia

A

Long neck

Shoulders are narrow, drooping, and have hypermobility

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29
Q

Dental findings in a patient with Cleidocranial dysplasia

A

Primary dentition retained

Numerous impacted and supernumerary teeth

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30
Q

Dental treatment for patient with Cleidocranial dysplasia

A

Correct skeletal relations, remove supernumerary teeth, correct alignment of permanent teeth

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31
Q

Prognosis for patient with Cleidocranial dysplasia

A

Good

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32
Q

Bone disorder detected in childhood where patient has painless bilateral expansion of the jaws (esp. mandible)

A

Cherubism

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33
Q

Why might the “eyes be turned to heaven” in a patient with Cherubism

A

Involvement of the inferior and/or lateral orbital walls may tilt the eyeballs upward and retract lower eyelid

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34
Q

Radiographic features of Cherubism

A

Bilateral multilocular radiolucencies in the posterior
Occasionally unilocular
Often significant tooth displacement

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35
Q

You may see ___ ___ in the histology of Cherubism

A

Perivascular hyalinization

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36
Q

Surgical intervention in Cherubism sometimes:

A

Accelerates growth of lesion

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37
Q

Osteitis deformans is also called:

A

Paget disease

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38
Q

Abnormal resorption and deposition, resulting in distorted/weaker bone

A

Paget disease

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39
Q

The affected bones in Paget disease become ____

A

Thickened

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40
Q

When is Paget disease typically discovered

A

On routine blood test or dental radiographs

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41
Q

Paget disease has a ____ predilection

A

Male

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42
Q

Paget disease typically affects ____ patients

A

Older

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43
Q

Many Paget disease patients have a ____ stance if their ____ is involved

A

“Simian” (monkey-like)

Femurs (bowing of legs)

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44
Q

There is elevated serum ___ ___ in patients with Paget disease

A

Alkaline phosphatase

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45
Q

What might a patient with Paget disease complain about if there is skull involvement?

A

Their hat won’t fit - the skull has progressive involvement

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46
Q

What might a patient with Paget disease complain about if their jaw is involved?

A

Denture won’t fit - thickened, enlarged alveolar bone

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47
Q

Radiographic feature of Paget disease

A

“Cotton wool” appearance of bone

Extensive hypercementosis

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48
Q

What is the marrow replaced with in Paget disease

A

Vascular fibrous connective tissue

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49
Q

In Paget disease, you will see a ___ pattern of irregular ____ with resting and ___ lines

A

Mosaic
Trabeculae
Reversal

50
Q

Treatment for Paget disease

A

Bisphosphonates (or no treatment if asymptomatic)

51
Q

Patients with Paget disease should be monitored for development of a ___ ___ ___ or ___ ___, especially ___

A

Giant cell tumor
Malignant tumors
Osteosarcoma

52
Q

You may have difficulty extracting teeth in patients with Paget disease due to:

A

Hypercementosis and/or ankylosis

53
Q

2 additional dental complications with Paget disease

A

Hemorrhage during vascular/lytic phase

Poor wound healing, increased susceptibility to osteomyelitis during avascular/sclerotic phase

54
Q

Prognosis for implants in Paget disease

A

Unfavorable (more so with use of bisphosphonates)

55
Q

Etiologies of fibro-osseous lesions

A

Developmental (hamartomatous), Reactive, Dysplastic, Neoplastic

56
Q

2 Fibro-osseous lesions

A
  1. Fibrous dysplasia

2. Cemento-Osseous Dysplasia

57
Q

3 types of Cemento-Osseous Dysplasia

A
  1. Periapical
  2. Focal
  3. Florid
58
Q

3 times of occurrence for fibrous dysplasia post-zygotic mutation

A
  1. Pluripotent stem cell
  2. Skeletal progenitor cell
  3. Post-natal life
59
Q

Are patients with Fibrous dysplasia younger or older than patients with Paget disease

A

Younger

60
Q

Do most cases of fibrous dysplasia involve many or one bone?

A

One bone

61
Q

____ are among the most commonly affected bones for fibrous dysplasia

A

Jaws

62
Q

Is the maxilla or the mandible more involved in fibrous dysplasia

A

Maxilla

63
Q

Why might a patient with craniofacial fibrous dysplasia have facial deformity?

A

Maxillary lesions may involve adjacent facial bones

64
Q

Early radiographic stages of fibrous dysplasia are ___ or ____. They ____ as they grow

A

Radiolucent or mottled

Opacify

65
Q

Obliteration of the ____ ____ can occur in fibrous dysplasia

A

Maxillary sinus

66
Q

Fibrous dysplasia has a classic ___ ____ pattern

A

Ground glass

67
Q

2 types of polyostotic fibrous dysplasia

A
  1. Jaffe type

2. McCune-Albright type

68
Q

Jaffe type

A

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

69
Q

McCune-Albright type

A

2 or more bones affected, cafe-au-lait spots, and endocrine disturbances (precocious puberty)

70
Q

Type of bone associated with fibrous dysplasia

A

Woven

71
Q

Because there is no capsule, abnormal bone in this disorder will fuse to the adjacent normal bone

A

fibrous dysplasia

72
Q

____ is contraindicated for treatment of fibrous dysplasia

A

Radiation

73
Q

Prognosis for fibrous dysplasia

A

Sometimes disease stabilizes or regresses. Rare malignant transformation

74
Q

Most common fibro-osseous lesion encountered in the clinical practice of dentistry

A

Cemento-Osseous dysplasia

75
Q

Cemento-Osseous dysplasia is possibly a ____ process

A

Reactive

76
Q

Cemento-Osseous dysplasia is most commonly seen in ___ ____

A

Black females

77
Q

Focal type Cemento-Osseous dysplasia is more common in ___ ___

A

White females

78
Q

Teeth usually test _____ (vital/non-vital) in Cemento-Osseous dysplasia

A

Vital

79
Q

Florid Cemento-Osseous dysplasia shows multiple ___ ____ type radio____ (pacities/lucities) in at least ____ quadrants of the jaws

A

Cotton wool
Radiopacities
2 quadrants

80
Q

Cemento-Osseous dysplasia may be associated with a ___ ___ cyst

A

Simple bone

81
Q

Types of Cemento-Osseous dysplasia that are mild, moderate, and severe

A

Periapical (mild)
Focal (moderate)
Florid (Severe)

82
Q

Periapical Cemento-Osseous dysplasia is usually in the ____ (anterior/posterior) ____ (maxilla/mandible)

A

Anterior mandible

83
Q

What can periapical Cemento-Osseous dysplasia be confused with?

A

Hypercementosis, Idiopathic osteosclerodid, benign cementoblastoma

84
Q

Focal Cemento-Osseous dysplasia is more common in ____ (older/younger) patients

A

Younger

85
Q

Focal Cemento-Osseous dysplasia can be confused with _____ _____. Which is more common?

A

Ossifying fibroma

Focal Cemento-Osseous dysplasia is more common

86
Q

Overlying mucosa can ulcerate and result in ___ ____ with florid Cemento-Osseous dysplasia

A

Bony sequestration

87
Q

Lesions in florid Cemento-Osseous dysplasia tend to be hypovascular, resulting in:

A

Reduced healing ability

Prone to necrosis, infection, osteomyelitis

88
Q

What does the mineralized product in Cemento-Osseous dysplasia resemble?

A

Ginger root

89
Q

How do you diagnose Cemento-Osseous dysplasia

A

Radiographs and confirm with biopsy if indicated

90
Q

Treatment for periapical Cemento-Osseous dysplasia

A

None

91
Q

Why might a biopsy be indicated for focal Cemento-Osseous dysplasia

A

To rule out another disease

92
Q

Biopsy is generally not necesssary for ____ Cemento-Osseous dysplasia

A

Florid

93
Q

Appearance of focal Cemento-Osseous dysplasia may be the first sign of ___ ___ ___ ___

A

Florid Cemento-Osseous dysplasia

94
Q

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

A

Osteoporotic bone marrow defect

95
Q

Osteoporotic bone marrow defect is usually seen in the ____ (anterior/posterior) _____ (maxilla/mandible)

A

Posterior mandible

96
Q

Usually seen at an old extraction site

A

Osteoporotic bone marrow defect

97
Q

Osteoporotic bone marrow defect has a ____ predilection

A

Female

98
Q

In a radiograph, Osteoporotic bone marrow defect is a ____ (radiopaque/radiolucent) lesion with ___ (well/ill) defined borders and a fine ___ pattern

A

Radiolucent
Ill
Trabecular

99
Q

Osteoporotic bone marrow defect shows fatty and ____ marrow

A

Hematopoietic

100
Q

True/False: The osteoblast and osteoclast activity is abnormal in Osteoporotic bone marrow defect

A

False - no abnormal activity

101
Q

Treatment for Osteoporotic bone marrow defect

A

Biopsy often indicated to establish diagnosis

102
Q

Prognosis for Osteoporotic bone marrow defect

A

Excellent

103
Q

Other terms for Idiopathic Osteosclerosis

A

Dense bone island, Enostosis, Bone whorl, Focal periapical osteopetrosis, bone scar

104
Q

Focally increased area of dense bone

A

Idiopathic Osteosclerosis

105
Q

Is there expansion with Idiopathic Osteosclerosis

A

No

106
Q

Peak prevalence of Idiopathic Osteosclerosis is in the ____ decade

A

3rd

107
Q

Most common site for Idiopathic Osteosclerosis

A

Mandibular pre-molar/molar area

108
Q

For Idiopathic Osteosclerosis, are the borders sharp or do they blend?

A

They blend, but occasionally may be sharp

109
Q

Does Idiopathic Osteosclerosis cause tooth displacement?

A

Rarely

110
Q

Idiopathic Osteosclerosis may be confused with

A
  1. Condensing osteitis
  2. Hypercementosis
  3. Cementoblastoma
111
Q

In Idiopathic Osteosclerosis, you see dense ___ bone

A

Vital

112
Q

Other terms for simple bone cyst

A

Traumatic bone cyst, Hemorrhagic bone cyst

113
Q

Empty or fluid-filled bone cavity can be seen in a ___ ___ ___

A

Simple bone cyst

114
Q

Why is a simple bone cyst not a true cyst?

A

It lacks an epithelial lining

115
Q

Trauma-hemorrhage theory

A

Trauma causing hematoma but not fracture - without subsequent organization and repair of hematoma - instead liquifies (possible theory for simple bone cyst)

116
Q

Does a simple bone cyst have a gender predilection?

A

None in the jaws, but male in other bones

117
Q

Simple bone cyst is mostly seen in the ____ (anterior/posterior) _____ (mandible/maxilla) and _____

A

Posterior mandible and symphysis

118
Q

Simple bone cyst often _____ between roots. Is this diagnostic?

A

Scallops - not diagnostic, but is suggestive

119
Q

Bone fragments in a simple bone cyst are lined by ___ ___ tissue

A

Inflamed granulation

120
Q

Why is the treatment for a simple bone cyst surgical exploration and curettage?

A

Induce bleeding –> causes hemorrhage –> organizes and heals lesion