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Oral Pathology > Developmental Bone Disorders > Flashcards

Developmental Bone Disorders Flashcards

1
Q

___ ___ includes several rare disorders of bone characterized by defective ___, which results in abnormal bone mineralization and low bone density (___).

A

Osteogenesis Imperfecta; collagen; osteopenia

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2
Q

Brittle bone disease

A

osteogenesis imperfecta

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3
Q

Osteogenesis imperfecta can be autosomal dominant or recessive. ___% are autosomal dominant and some cases are sporadic.

A

90

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4
Q

Where is the mutation for osteogenesis imperfecta?

A

collagen gene

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5
Q

T/F. Bones are very fragile and fracture easily, but the degree of fragility varies with the type of OI

A

True.

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6
Q

T/F. OI is one of the least common heritable bone diseases.

A

False, it’s one of the most common.

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7
Q

Clinical features of OI include ___ sclera, ___ loss, craniofacial alterations (CLIII occlusion, traingular facies), ___ deformity of long bones, and ___ teeth.

A

blue; hearing; bowing; opalescent

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8
Q

OI’s dental alterations are ___ to dentinogenesis imperfecta. Blue, yellow or brown translucences of teeth (noticed easier in ___ teeth). Severe ___ leading to loss of VDO and potential tooth loss.

A

identical; primary; attrition

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9
Q

Radiographically, OI has “___ teeth” or premature ___ obliteration. Tooth roots ___ or ___ ___ shaped.

A

shell; pulpal; narrow; corn cob

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10
Q

OI and dentinogenesis imperfecta are distinct mutations, different diseases with different tooth alterations and have blue sclera.

A

False, OI and dentinogenesis imperfecta can have SIMILAR tooth alterations and blue sclera but they are distinct mutations, different diseases

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11
Q

How do you designate dental defects associated with OI?

A

opalescent teeth

“Dentinogenesis imperfecta” is reserved for alterations isolated to the teeth.

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12
Q

How is OI treated?

A
  1. physiotherapy, rehabilitation, orthopedic surgery
  2. minimize factors that cause fractures
  3. intravenous bisphosphonates
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13
Q

What is typically administered to children with moderate to severe disease pain?

A

intravenous bisphosphonates

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14
Q

What does the prognosis depend on for OI patients?

A

type of OI

expression of the gene

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15
Q

How does death result in OI patients with severe forms of the disease?

A

passage though the birth canal

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16
Q

What is a rare inherited bone disease caused by lack of osteoclastic activity?

A

osteopetrosis

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17
Q

Osteopetrosis has an ___ in bone density due to failure of ___ to function normally with continued bone formation and ossification.

A

increase; osteoclasts

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18
Q

T/F. Osteopetrosis has autosomal dominant and recessive forms.

A

True. The recessive is much worse

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19
Q

What is seen on CT imaging with osteopetrosis?

A

thickening of bones of skull

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20
Q

What is commonly seen in the autosomal recessive form of osteopetrosis?

A
  1. blindness and deafness (cranial nerve compression)
  2. fractures
  3. osteomyelitis of the jaw
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21
Q

___ spaces are filled in by dense bone, resulting in loss of hematopoietic precursor cells leading to ___

A

Marrow; pancytopenia

  • increased susceptibility to infection
  • osteomyelitis of the jaw
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22
Q

What is a frequent complication of tooth extraction?

A

osteomyelitis

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23
Q

In osteopetrosis, radiographs show diffuse density of the ___. Tooth ___ are difficult to visualize due to density of surrounding bone.

A

skeleton; roots

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24
Q

In osteopetrosis, tooth eruption in common.

A

False, FAILURE OF tooth eruption is common

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25
Q

How is osteopetrosis treated?

A 
supportive measures (transfusions and antibiotics)
bone marrow transplant (limited success)
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26
Q

What are some alternative therapies of osteopetrosis?

A

interferon with calcitriol

restricted Ca++ intake, corticosteroids and erythropoietin

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27
Q

Prognosis of osteopetrosis is ___ for AR form with many patients dying before ___ years of age. ___ can have long term survival.

A

poor; 20; AD

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28
Q

What is an uncommon autosomal dominant disorder that affects skull, jaws, and clavicles?

A

cleidocranial dysplasia

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29
Q

How do patients with cleidocranial dysplasia present?

A

prominent forehead; hypoplastic midface

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30
Q

Why are primary dentition retained in patients with cleidocranial dyplasia?

A

because permanent teeth do not erupt

numerous impacted permanent and supernumerary teeth (plenty of teeth just not erupting in the correct space or not erupting at all)

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31
Q

What is today’s treatment for cleidocranial dysplasia?

A

combined surgical and orthodontic care to correct skeletal relations, remove supernumerary teeth and bring permanent teeth into proper relation

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32
Q

T/F. The prognosis is poor for cleidocranial dysplasia.

A

False, it is good. the life span of these patients is essentially normal

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33
Q

Where is osteoporotic bone marrow defect found?

A

usually seen in the body of mandible, usually at old extraction sites

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34
Q

T/F. Osteoporotic bone marrow defect is an incidental finding in middle aged, asymptomatic females.

A

True.

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35
Q

What is the pathogenesis of osteoporotic bone marrow defect?

A

unknown, may resemble metastatic disease (biopsy)

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36
Q

Microscopically, ___ and ___ marrow is seen in osteoporotic bone marrow defects.

A

fatty; hematopoietic

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37
Q

What is seen on pans of osteoporotic bone marrow defect? What about periapical radiographs?

A

pan: radiolucent, circumscribed
PA: ill-defined borders and fine central trabeculations

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38
Q

What are several other terms for idiopathic osteosclerosis?

A

dense bone island

enostosis

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39
Q

Idiopathic osteosclerosis is very ___ with no expansion. What region is most common?

A

radiopaque (dense viable bone microscopically)

premolar-molar

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40
Q

Describe the margins of idiopathic osteosclerosis.

A

margins may be sharp or blend with adjacent bone.

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41
Q

With idiopathic osteosclerosis, past studies did not distinguish the idiopathic lesions from those of inflammatory origin and confusion in terminology has resulted. What 3 disorders can it be confused with?

A
  1. condensing osteitis
  2. hypercementosis
  3. cemetoblastoma
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42
Q

T/F. Cherubism is an autosomal recessive condition detected in childhood.

A

False, it is autosomal DOMINANT or de novo mutation detected in childhood

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43
Q

Cherubism is a ___, bilateral expansion of the jaws, especially in the ___ that results in chubby cheeks.

A

painless; mandible

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44
Q

What causes the “eyes upturned to heaven” appearance seen in Cherubism?

A

involvement of the inferior and/or lateral orbital walls may tilt the eyeballs upward and retract the lower eyelid, thereby exposing the sclera below the iris to produce an “eyes upturned to heaven” appearance.

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45
Q

How does the radiograph look for patients with Cherubism?

A

bilateral multilocular radiolucencies of posterior mandible with often significant displacement of teeth.

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46
Q

In Cherubism, microscopically, there is edematous cellular ___ connective tissue, relatively sparse benign ___ ___ cells, and sometimes ___ ___.

A

fibrous connective; multinucleated giant; perivascular hyalinization

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47
Q

What is perivascular hyalinization?

A

eosinophilic cuffing

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48
Q

T/F. In Cherubism, optimal treatment has not been determined and surgical intervention has been known to accelerate the growth of some lesions.

A

True.

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49
Q

T/F. In Cherubism, many cases seem to progress into adulthood.

A

False, many cases seem to involute during puberty

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50
Q

What is another name for simple bone cyst?

A

traumatic bone cyst

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51
Q

What is an idiopathic condition seen in the 1st and 2nd decade with a questionable relation to trauma?

A

simple bone cyst

52
Q

Simple bone cyst has a ___ predilection and is found in the ___ ___.

A

male; posterior mandible

53
Q

Describe the radiograph seen with simple bone cyst.

A

well-circumscribed radiolucency, that can scallop between roots

54
Q

What is found at surgical exploration in patients with simple bone cyst?

A

an empty cavity is found within the bone making it difficult to obtain lesional tissue.

55
Q

In simple bone cyst, ___ of bone are lined by chronically inflamed granulation tissue.

A

fragments

56
Q

T/F. Simple bone cyst is a true cyst.

A

False, it has no epithelial lining and therefore, it is not a true “cyst”.

57
Q

What is recommended for simple bone cyst treatment?

A

to enter the lesion, establish the diagnosis, then induce bleeding (supposedly the hemorrhage organizes and the lesion heals)

58
Q

T/F. Simple bone cyst are ONLY found in the mandible.

A

False, they have been reported in almost every bone of the body.

59
Q

What disease is also known as Paget disease of bone?

A

Osteitis deformans

60
Q

Osteitis deformans has abnormal ___ and deposition, resulting in distortion of bone. The affected bones become ___ and ___.

A

resorption; thickened; weak

61
Q

How is osteitis deformans discovered?

A

incidentally on routine blood test or dental radiographs

62
Q

T/F. Osteitis deformans is seen in young, black female patients under 40

A

False, it is seen in older patients of Anglo-Saxon decent, it is rare in patients under 40 and it is more common in males (2:1)

63
Q

Although the etiology is unknown, what markers are elevated in osteitis deformans?

A

markedly elevated serum alkaline phosphatase

64
Q

Although symptoms vary in osteitis deformans, at presentation, bone pain is present in up to ___%

A

40

65
Q

Most cases of osteitis deformans are ___, with involvement of ___ causing a “___” (monkeylike) stance due to bowing of the legs.

A

polyostotic; femurs; simian

66
Q

Jaws are involved in ___% of patients affected with Osteitis deformans.

A

17

67
Q

T/F. The mandible is involved more than the maxilla in osteitis deformans.

A

False, the MAXILLA is affected more.

68
Q

What is seen radiographically in osteitis deformans?

A

cotton-wool appearance

hypercementosis of teeth

69
Q

How is the “mosaic” pattern produced in osteitis deformans?

A

irregular trabeculae with resting reversal lines rimmed by osteoclasts and osteoblasts

70
Q

What is the marrow replaced by in osteitis deformans?

A

vascular fibrous connective tissue

71
Q

T/F. Osteitis deformans is chronic and progressive, but usually not life threatening.

A

True

72
Q

What is the treatment for Osteitis deformans?

A

if asymptomatic, no tx
bisphosphonates
patients should be monitored for the development of giant cell tumor or bone as well as malignant bone tumors, especially osteosarcoma.

73
Q

What are some potential dental complications in patients with osteitis deformans?

A
  1. difficulties in extracting teeth with hypercementosis and/or ankyloses
  2. extensive hemorrhage from oral surgical procedures performed during vascular lytic phase
  3. poor wound healing with increases susceptibility to osteomyelitis during the avascular sclerotic phase
  4. edentulous patients may require new dentures periodically to compensate for progressive alveolar enlargement
  5. pagetic bone and a history of bisphosphonate therapy generally considered unfavorable factor for osseointegration of dental implants
74
Q

What are the two types of fibro-osseous lesions of the jaws?

A
  1. fibrous dysplasia

2. cement-osseous dysplasia

75
Q

What are the three types of cement-osseous dysplasias?

A
  1. focal cement-osseous dysplasia
  2. periapical cement-osseous dysplasia
  3. florid cement-osseous dysplasia
76
Q

Fibrous dysplasia is a ___, tumor-like lesion due to post-zygotic mutation of a ___ ___ gene (GNAS1) usually present in the first or second decade?

A

developmental; tumor suppressor

77
Q

What percentage of fibrous dysplasia are monstotic (affecting one bone)?

A

70-85%

78
Q

T/F. Fibrous dysplasia is a painful bilateral fast-growing swelling.

A

False, it is a painLESS, UNILATERAL, SLOW-growing swelling

79
Q

The ___ are among the most commonly affected bones in fibrous dysplasia. The ___ is affected more than the ___.

A

jaws; maxilla; mandible

80
Q

What is a more severe form of fibrous dysplasia?

A

“craniofacial fibrous dysplasia”

81
Q

“Craniofacial fibrous dysplasia” has ___ lesions may involve the adjacent facial bones, including the ___, ___, and ___, which results in marked facial deformity.

A

maxillary; sphenoid; zygoma; occiput

82
Q

What is the classic radiographic description for fibrous dysplasia?
Describe the radiograph in these patients.

A

ground glass pattern

poorly defined, blending margins
early stages - radiolucent or mottled

83
Q

T/F. With maxillary involvement, obliteration of the maxillary sinus is common in fibrous dysplasia patients.

A

True.

84
Q

In fibrous dysplasia, microscopically there is irregularly shaped ___ of immature (woven) bone. The abnormal bone fuses to adjacent normal bone but no ___ is formed. The is also moderate cellular ___ connective tissue.

A

trabeculae; capsule; intertrabecular

85
Q

What are the two presentations of polyostotic fibrous dysplasia?

A
  1. jaffe type

2. McCune Albright type

86
Q

The jaffe type has two or more bones affected in conjuction with ___-__-___ spots that have ___ borders (like the coast of ___)

A

café-au-lait; jagged; Maine

87
Q

In McCune-Albright, patients have two or more bones affected in addition to café-au-lait pigmentation and ___ disturbances which manifest as precocious puberty.

A

endocrine

88
Q

In fibrous dysplasia, ___ lesions may not need treatment or may be removed by ___ ___ resection.

A

small; en bloc

89
Q

T/F. Significant cosmetic or functional deformity may require an attempt at surgical reduction in fibrous dysplasia. Sometimes the disease stabilizes with skeletal maturation.

A

True.

90
Q

What percentage of surgically treated fibrous dysplasia lesions show regrowth, particularly in younger patients?

A

25-50%

91
Q

In fibrous dysplasia, malignant transformation to a mesenchymal malignancy is ___ and usually is reported in lesions that have received ___ therapy

A

rare; radiation

92
Q

What is a benign, possibly reactive process that may originate from the periodontal ligament fibroblast?

A

Cemento-ossesous dysplasia

93
Q

What groups is cement-ossesous dysplasis seen in?

A

most commonly African-American females then
East Asian females
White females
but it can affect either sex and any ethinic group

94
Q

Place the different types of cement-osseous dysplasias in order of severity:

  1. Periapical cemento-osseous dysplasia
  2. Focal cemento-osseous dysplasia
  3. Florid cemento-osseous dysplasia

A. Mild
B. Moderate
C. Severe

A

1 - A
2 - B
3 - C

95
Q

Describe a radiograph of a patient with cemento-osseous dysplasia.

A
  1. found incidentally
  2. tooth bearing areas of the jaw (above IAN)
  3. varies from completely radiolucent to densely radiopaque with a thin peripheral radiolucent rim (PDL remains in tact)
  4. TEETH TEST VITAL!!!
96
Q

Periapical cemento-osseous dysplasia is found in the ___ anterior region or ___-aged ___-American women.

A

mandibular; middle; African

97
Q

T/F. Periapical cemento-osseous dysplasia has radiolucencies at apices of teeth with gradual central opacity developing.

A

True.

98
Q

What can be confusing with hypercementosis?

A

radiodensity is all within the PDL

99
Q

What is confusing with idiopathic osteosclerosis?

A

not necessarily at apex; no lucent periphery

100
Q

What is confusing about benign cementoblastoma?

A

resorption of root and fusion with radiopaque mass, usually affecting one posterior tooth

101
Q

How is Periapical Cemento-osseous dysplasia diagnoses, treated and what is its prognosis?

A

diagnosis: based on clincal and radiographic features
treatment: none necessary
prognosis: excellent

102
Q

In the past, what true neoplasm was focal cemento-osseous dysplasia confused with?

A

the central cemento-ossifying fibroma

103
Q

FCOD is seen across all ethnic groups but may affect a greater proportion of ___.

A

whites (that may be a study population bias)

104
Q

T/F. FCOD is less common than central cemento-ossifying fibroma, but they are seen in a similar demographic group - older adult men.

A

False, FCOD is MORE common than central cemento-ossifying fibroma, but they are seen in a similar demographic group - YOUNGER adult WOMEN.

105
Q

How is FCOD detected? What is seen?

A

Radiographic examination

unilocular radiolucency, with or without radiopaque central component

106
Q

FCOD is found in the ___ of the ___, mostly in ___. The most common age range is ___-___. Swelling or discomfort is ___.

A

body; mandible; females; 20-40; unusal

107
Q

With FCOD, at surgery the lesion is usually ___ defined from the surrounding bone and multiple, small gritty ___ are obtained.

A

poorly; fragments

108
Q

In FCOD, connective tissue with embedded mineralized tissue that resembles either ___ bone or cellular ___.

A

woven; cementum

109
Q

How do the trabeculae appear in FCOD?

A

ginger root shaped

110
Q

T/F. FCOD treatment is unnecessary, however biopsy is often warranted to rule out other diseases.

A

True

111
Q

FCOD prognosis is generally ___, although a lesion that initially appears as a focal process may in fact represent the first sign of what disease?

A

good; florid cemento-osseous dysplasia

112
Q

What is the most severe expression of the cemento-osseous dysplasias?

A

Florid cemento-osseous dysplasia

113
Q

Florid is seen in middle-aged or older ___-American ___.

A

African; women

114
Q

Florid affects multiple ___ of the jaws. It is generally asymptomatic unless overlying mucosa ___, resulting in ___.

A

quadrants; ulcerates; sequestration

115
Q

Florid has radiolucencies with multiple “___ ___” radiopacities in at least ___ quadrants of the jaws.

A

cotton wool; two

116
Q

Over time, Florid lesions become more ___. They may also be associated with what disease?

A

radiodense; simple (traumatic) bone cysts

117
Q

With cemento-osseous dysplasia, lesions tend to be ___ and prone to ___ and secondary ___ with minimal provocation.

A

hypovascular; necrosis; infection

118
Q

T/F. Dental implant placement is not recommended in cemento-ossesous dysplasia patients.

A

True

119
Q

In Florid, ___ is not necessary and submission of sequestrating fragments shows densely ___ tissue with ___ debris and inflammation.

A

biopsy; mineralized; necrotic

120
Q

T/F. Patients with cemento-osseous dysplasia should be encouraged to remove their teeth. Why?

A

False, Patients with cemento-osseous dysplasia should be encouraged to RETAIN their teeth.
Why? symptoms begin after lesion exposure resulting from progressive alveolar atrophy under a denture.

121
Q

When does the onset of symptoms occur in cemento-osseous dysplasia?

A

associated with exposure to sclerotic masses to the oral cavity (biopsy, elective tooth extraction). Therefore, these should be avoided

122
Q

If bone is exposed in cemento-osseous dysplasia, ___, ___ ___ -like material begins to exfoliate through the oral mucosa?

A

yellowish, avascular cementum

123
Q

What is recommended for asymptomatic patients with Cemento-osseous dysplasia?

A

regular recall examinations with prophylaxis and oral hygiene reinforcement to control periodontal disease and prevent tooth loss

124
Q

Management of symptomatic Cemento patients who develop secondary ___ is difficult. Treatment consist of ___ and ___ (which are indicated but not effective because they can’t get to the infection)

A

osteomyelitis; debridement; antibiotics

125
Q

What is overall prognosis of Cemento-osseous dysplasia? What causes secondary infection?

A

good

sequestration, requiring debridement and antibiotics

126
Q

T/F. Development of sarcoma within an area of cemento-osseous dysplasia is common.

A

False, it has been reported but is extremely rare.

Oral Pathology (5 decks)

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