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
Prognosis for autosomal dominant vs. autosomal recessive Osteopetrosis
Poor for recessive
26
Uncommon bone disorder that affects the skull, jaws, and clavicles primarily
Cleidocranial dysplasia
27
Patients with cleidocranial dysplasia often have a prominent ____, and _____ midface
Forehead | Hypoplastic
28
Describe the neck and shoulders of a patient with Cleidocranial dysplasia
Long neck | Shoulders are narrow, drooping, and have hypermobility
29
Dental findings in a patient with Cleidocranial dysplasia
Primary dentition retained | Numerous impacted and supernumerary teeth
30
Dental treatment for patient with Cleidocranial dysplasia
Correct skeletal relations, remove supernumerary teeth, correct alignment of permanent teeth
31
Prognosis for patient with Cleidocranial dysplasia
Good
32
Bone disorder detected in childhood where patient has painless bilateral expansion of the jaws (esp. mandible)
Cherubism
33
Why might the "eyes be turned to heaven" in a patient with Cherubism
Involvement of the inferior and/or lateral orbital walls may tilt the eyeballs upward and retract lower eyelid
34
Radiographic features of Cherubism
Bilateral multilocular radiolucencies in the posterior Occasionally unilocular Often significant tooth displacement
35
You may see ___ ___ in the histology of Cherubism
Perivascular hyalinization
36
Surgical intervention in Cherubism sometimes:
Accelerates growth of lesion
37
Osteitis deformans is also called:
Paget disease
38
Abnormal resorption and deposition, resulting in distorted/weaker bone
Paget disease
39
The affected bones in Paget disease become ____
Thickened
40
When is Paget disease typically discovered
On routine blood test or dental radiographs
41
Paget disease has a ____ predilection
Male
42
Paget disease typically affects ____ patients
Older
43
Many Paget disease patients have a ____ stance if their ____ is involved
"Simian" (monkey-like) | Femurs (bowing of legs)
44
There is elevated serum ___ ___ in patients with Paget disease
Alkaline phosphatase
45
What might a patient with Paget disease complain about if there is skull involvement?
Their hat won't fit - the skull has progressive involvement
46
What might a patient with Paget disease complain about if their jaw is involved?
Denture won't fit - thickened, enlarged alveolar bone
47
Radiographic feature of Paget disease
"Cotton wool" appearance of bone | Extensive hypercementosis
48
What is the marrow replaced with in Paget disease
Vascular fibrous connective tissue
49
In Paget disease, you will see a ___ pattern of irregular ____ with resting and ___ lines
Mosaic Trabeculae Reversal
50
Treatment for Paget disease
Bisphosphonates (or no treatment if asymptomatic)
51
Patients with Paget disease should be monitored for development of a ___ ___ ___ or ___ ___, especially ___
Giant cell tumor Malignant tumors Osteosarcoma
52
You may have difficulty extracting teeth in patients with Paget disease due to:
Hypercementosis and/or ankylosis
53
2 additional dental complications with Paget disease
Hemorrhage during vascular/lytic phase | Poor wound healing, increased susceptibility to osteomyelitis during avascular/sclerotic phase
54
Prognosis for implants in Paget disease
Unfavorable (more so with use of bisphosphonates)
55
Etiologies of fibro-osseous lesions
Developmental (hamartomatous), Reactive, Dysplastic, Neoplastic
56
2 Fibro-osseous lesions
1. Fibrous dysplasia | 2. Cemento-Osseous Dysplasia
57
3 types of Cemento-Osseous Dysplasia
1. Periapical 2. Focal 3. Florid
58
3 times of occurrence for fibrous dysplasia post-zygotic mutation
1. Pluripotent stem cell 2. Skeletal progenitor cell 3. Post-natal life
59
Are patients with Fibrous dysplasia younger or older than patients with Paget disease
Younger
60
Do most cases of fibrous dysplasia involve many or one bone?
One bone
61
____ are among the most commonly affected bones for fibrous dysplasia
Jaws
62
Is the maxilla or the mandible more involved in fibrous dysplasia
Maxilla
63
Why might a patient with craniofacial fibrous dysplasia have facial deformity?
Maxillary lesions may involve adjacent facial bones
64
Early radiographic stages of fibrous dysplasia are ___ or ____. They ____ as they grow
Radiolucent or mottled | Opacify
65
Obliteration of the ____ ____ can occur in fibrous dysplasia
Maxillary sinus
66
Fibrous dysplasia has a classic ___ ____ pattern
Ground glass
67
2 types of polyostotic fibrous dysplasia
1. Jaffe type | 2. McCune-Albright type
68
Jaffe type
2 or more bones affected and cafe-au-lait spots with jagged borders (coast of Maine)
69
McCune-Albright type
2 or more bones affected, cafe-au-lait spots, and endocrine disturbances (precocious puberty)
70
Type of bone associated with fibrous dysplasia
Woven
71
Because there is no capsule, abnormal bone in this disorder will fuse to the adjacent normal bone
fibrous dysplasia
72
____ is contraindicated for treatment of fibrous dysplasia
Radiation
73
Prognosis for fibrous dysplasia
Sometimes disease stabilizes or regresses. Rare malignant transformation
74
Most common fibro-osseous lesion encountered in the clinical practice of dentistry
Cemento-Osseous dysplasia
75
Cemento-Osseous dysplasia is possibly a ____ process
Reactive
76
Cemento-Osseous dysplasia is most commonly seen in ___ ____
Black females
77
Focal type Cemento-Osseous dysplasia is more common in ___ ___
White females
78
Teeth usually test _____ (vital/non-vital) in Cemento-Osseous dysplasia
Vital
79
Florid Cemento-Osseous dysplasia shows multiple ___ ____ type radio____ (pacities/lucities) in at least ____ quadrants of the jaws
Cotton wool Radiopacities 2 quadrants
80
Cemento-Osseous dysplasia may be associated with a ___ ___ cyst
Simple bone
81
Types of Cemento-Osseous dysplasia that are mild, moderate, and severe
Periapical (mild) Focal (moderate) Florid (Severe)
82
Periapical Cemento-Osseous dysplasia is usually in the ____ (anterior/posterior) ____ (maxilla/mandible)
Anterior mandible
83
What can periapical Cemento-Osseous dysplasia be confused with?
Hypercementosis, Idiopathic osteosclerodid, benign cementoblastoma
84
Focal Cemento-Osseous dysplasia is more common in ____ (older/younger) patients
Younger
85
Focal Cemento-Osseous dysplasia can be confused with _____ _____. Which is more common?
Ossifying fibroma | Focal Cemento-Osseous dysplasia is more common
86
Overlying mucosa can ulcerate and result in ___ ____ with florid Cemento-Osseous dysplasia
Bony sequestration
87
Lesions in florid Cemento-Osseous dysplasia tend to be hypovascular, resulting in:
Reduced healing ability | Prone to necrosis, infection, osteomyelitis
88
What does the mineralized product in Cemento-Osseous dysplasia resemble?
Ginger root
89
How do you diagnose Cemento-Osseous dysplasia
Radiographs and confirm with biopsy if indicated
90
Treatment for periapical Cemento-Osseous dysplasia
None
91
Why might a biopsy be indicated for focal Cemento-Osseous dysplasia
To rule out another disease
92
Biopsy is generally not necesssary for ____ Cemento-Osseous dysplasia
Florid
93
Appearance of focal Cemento-Osseous dysplasia may be the first sign of ___ ___ ___ ___
Florid Cemento-Osseous dysplasia
94
Area of hematopoietic bone marrow of sufficient size to cause a radiographic radiolucency
Osteoporotic bone marrow defect
95
Osteoporotic bone marrow defect is usually seen in the ____ (anterior/posterior) _____ (maxilla/mandible)
Posterior mandible
96
Usually seen at an old extraction site
Osteoporotic bone marrow defect
97
Osteoporotic bone marrow defect has a ____ predilection
Female
98
In a radiograph, Osteoporotic bone marrow defect is a ____ (radiopaque/radiolucent) lesion with ___ (well/ill) defined borders and a fine ___ pattern
Radiolucent Ill Trabecular
99
Osteoporotic bone marrow defect shows fatty and ____ marrow
Hematopoietic
100
True/False: The osteoblast and osteoclast activity is abnormal in Osteoporotic bone marrow defect
False - no abnormal activity
101
Treatment for Osteoporotic bone marrow defect
Biopsy often indicated to establish diagnosis
102
Prognosis for Osteoporotic bone marrow defect
Excellent
103
Other terms for Idiopathic Osteosclerosis
Dense bone island, Enostosis, Bone whorl, Focal periapical osteopetrosis, bone scar
104
Focally increased area of dense bone
Idiopathic Osteosclerosis
105
Is there expansion with Idiopathic Osteosclerosis
No
106
Peak prevalence of Idiopathic Osteosclerosis is in the ____ decade
3rd
107
Most common site for Idiopathic Osteosclerosis
Mandibular pre-molar/molar area
108
For Idiopathic Osteosclerosis, are the borders sharp or do they blend?
They blend, but occasionally may be sharp
109
Does Idiopathic Osteosclerosis cause tooth displacement?
Rarely
110
Idiopathic Osteosclerosis may be confused with
1. Condensing osteitis 2. Hypercementosis 3. Cementoblastoma
111
In Idiopathic Osteosclerosis, you see dense ___ bone
Vital
112
Other terms for simple bone cyst
Traumatic bone cyst, Hemorrhagic bone cyst
113
Empty or fluid-filled bone cavity can be seen in a ___ ___ ___
Simple bone cyst
114
Why is a simple bone cyst not a true cyst?
It lacks an epithelial lining
115
Trauma-hemorrhage theory
Trauma causing hematoma but not fracture - without subsequent organization and repair of hematoma - instead liquifies (possible theory for simple bone cyst)
116
Does a simple bone cyst have a gender predilection?
None in the jaws, but male in other bones
117
Simple bone cyst is mostly seen in the ____ (anterior/posterior) _____ (mandible/maxilla) and _____
Posterior mandible and symphysis
118
Simple bone cyst often _____ between roots. Is this diagnostic?
Scallops - not diagnostic, but is suggestive
119
Bone fragments in a simple bone cyst are lined by ___ ___ tissue
Inflamed granulation
120
Why is the treatment for a simple bone cyst surgical exploration and curettage?
Induce bleeding --> causes hemorrhage --> organizes and heals lesion