Developmental Bone Disorders Flashcards

1
Q

Several Rare disorders characterized by defective collagen

A

Osteogenesis Imperfecta (Brittle Bone Disase)

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2
Q

Bone conditions with Osteogenesis Imperfecta

A

Low bone density, fragile

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3
Q

Symptoms of Osteogenesis Imperfecta (none dental)

A

Blue sclera, hearing loss, craniofacial alterations, bowing of large bones

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4
Q

What are the dental symptoms of Osteogenesis Imperfecta identical to?

A

Dentinogenesis Imperfecta

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5
Q

What are the Dental symptoms w/ Osteogenesis Imperfecta

A

Translucent teeth (more in primary), loss of VDO, attrition
Shell teeth in radiographs, narrow roots
* Opalescent teeth not DI

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6
Q

What percent of Osteogenesis Imperfecta is caused by AD mutation

A

90

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7
Q

Treatment for Osteogenesis Imperfecta

A

Physiotherapy, rehab, IV bisphophantes in children w/ moderate/severe disease

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8
Q

Inherited Bone disease caused by lack of osteoclastic activity

A

Osteopetrosis

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9
Q

Symptoms of Osteopetrosis

A
  • Thickening of bones
  • Deafness/blindness (CN compression)
  • Pancytopenia(Increase in infection)
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10
Q

Dental Symptoms with Osteopetrosis

A

Osteomyelitis after extraction
Failure of tooth eruption
Difficult to discern roots from x-ray

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11
Q

Symptoms in recessive type of Osteopetrosis

A

Fractures and osteomyeltis

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12
Q

Cause/Frequency of Osteopetrosis

A

Rare, AR and AD

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13
Q

Treatment for Osteopetrosis

A

Supportive measures

  • Limited success w/ BM transplant
  • Alt therapies (IF, Ca, EPO, corticosteroids)
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14
Q

Prognosis for Osteopetrosis

A

AR- Poor (die before 20)

AD- Long term survival

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15
Q

Condition affecing skull, jaws, and clavicles w/ a prominent forehead and hypoplastic midface

A

Cleidocranial Dysplasia

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16
Q

Dental conditions w/ Cleidocranial Dysplasia

A

Primary dentition retained because permanent teeth don’t erupt, impacted permanent/supernumerary teeth

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17
Q

What is the Cause/ Frequency of Cleidocranial Dysplasia

A

Unoommon, AD

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18
Q

Treatment for Cleidocranial Dysplasia?

A

Surgical and ortho to remove supernumerary/impacted teeth and move into proper rrelation

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19
Q

Prognosis for Cleidocranial Dysplasia

A

Good, normal lifespan

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20
Q

Incidental finding in body of mandible in old extraction site that appears radiolucent/circumscribed on pan and ill-defined w/ trabeculations on PA

A

Osteoporotic Bone Marrow Defect

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21
Q

Age/sex more common w/ Osteoporotic Bone Marrow Defect

A

Middle aged female

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22
Q

Symptoms of Osteoporotic Bone Marrow Defect

A

None, resembles metastatic disease

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23
Q

Micro appearance of Osteoporotic Bone Marrow Defect

A

Fatty and hematopoietic marrow

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24
Q

Pathogenesis of Osteoporotic Bone Marrow Defect

A

Unknown

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25
Incidental Finding in x-ray, very radiopaque w/ no bone expansion
Idiopathic Osterosclerosis
26
Other terms for Idiopathic Osterosclerosis
Dense bone island/Enostosis
27
Most common region for Idiopathic Osterosclerosis
Molar/PM
28
Micro appearance of bone w/ Idiopathic Osterosclerosis
Dense/viable
29
DD for Idiopathic Osterosclerosis
Condensing osteitis, hypercementosis, cementoblastoma
30
Painless, bilateral expansion of the jaws especially he mandible detected in childhood
Cherubism
31
Cause of Cherubism
AD or de novo mutation
32
Result of inf/lat orbital walls being affected by Cherubism
Eyeballs tilt upwards and lower eyelids are retracted
33
Dental radiographic findings w/ Cherubism
Bilateral/multilocular radiolocuncies of post. mandible | Displacement of teeth
34
Microscope apperance of Cherubism
Edematous/cellular CT, sparse multinucleated giant cells, perivascular hyalinization
35
Treatment of Cherubism
Surgery may accelerate, involute during puberty
36
Condition seen in 1st/2nd decade, well-circumscribed radiolucency that can scallop between roots
Simple Bone Cyst (Traumatic bone cyst)
37
Sex predilection for Simple Bone Cyst
Male
38
Location most common for Simple Bone Cyst
Posterior mandible
39
Cause of Simple Bone Cyst
Idiopathic, relation to trauma
40
Findings upon exploration of Simple Bone Cyst
Empty bone cavity, fragments of bone lined by chronically inflamed granulation tissue
41
Why or why not is a Simple Bone Cyst considered a true cyst
Doesn''t have epithelial lining, not a true cyst
42
Treatment of Simple Bone Cyst
Induce bleeding to organize/heal lesion
43
What bones have Simple Bone Cysts been observed
Every bone in the body
44
AKA as Peget disease of bone, condition from abnormal resorption and deposition of bone, resulting in bone distortion
Osteitis Deformans
45
How is Osteitis Deformans usually discovered
Routine blood test or dental radiograph
46
Condition of affected bones w/ Osteitis Deformans
Thickened and weakened
47
Age/sex/ancestry associated with Osteitis Deformans
Male 2:1, >40, Anglo-Saxon
48
MArkedly elevated serum alkaline phophatase is associated with what condition
Osteitis Deformans
49
What perfect of patients have bone pain with Osteitis Deformans
40
50
Are most cases of Osteitis Deformans poly or monostotic
polystotic
51
Result of femurs being affected with Osteitis Deformans
Simian stance
52
Jaws are involved in what percent of patients w/ Osteitis Deformans
17%
53
Maxilla or mandible more affected by Osteitis Deformans
Maxilla
54
Radiographic appearance of Osteitis Deformans
Cotton wool, mosaic pattern (irregular trabeulae), rimmed by osteoclasts/blasts
55
Other dental condition seen with Osteitis Deformans
Hypercementosis
56
What is bone marrow replaced by in Osteitis Deformans
Vascular fibrous CT
57
Treatment/Prognosis for Osteitis Deformans
Not life-threatening - No Tx if asymptomatic - Bisphosphonates - Monitor for other bone tumors
58
5 Potential Dental complications with Osteitis Deformans
1) Difficulty extracting teeth (hypercemntosis) 2) Extensive hemorrhage for procedures during vascular lytic phase 3) Poor wound healing and increased chance of osteomyelitis during avascular sclerotic phase 4) Progressive alveolar enlargement in denture patients 5) Unfavorable conditions for osteointegration
59
Developmental, tumor like lesion that results in painless unilateral swelling w/ slow growth
Fibrous Dysplasia
60
Gene mutated that results in Fibrous Dysplasia
GNAS1 (tumor suppressor gene)
61
Age Fibrous Dysplasia usually presents
1st or 2nd decade
62
What percent of Fibrous Dysplasias are monostotic
70-85%
63
Are jaws commonly affect, and max or mand more
Yes, Max more
64
More severe presentation of Fibrous Dysplasia
Craniofacial Fibrous Dysplasia
65
Cause of facial deformity with Craniofacial Fibrous Dysplasia
Maxillary lesions involve adjacent bones
66
Radiographic appearance of Fibrous Dysplasia
Ground glass, blended margins, radiolucent in early stages, obliteration of max sinus , irrregular shape of trabeculae of immature bone, no capsule, cellular intertrabecular CT
67
2 Types of Polyostotic Fibrous Dysplasia and their symptoms
1) Jaffe Type- 2+ bones, and cafe au lait spots w/ jagged border 2) McCune Albright- Same as Jaffe w/ addition of endocrine disturbances which manifest as precocious puberty
68
Tx for Fibrous Dysplasia
- Small- None or en bloc resection - Large- Surgical - Can stabalize with skeletal maturation
69
What percent of surgically treated lesions show regrowth in Fibrous Dysplasia
25-50%
70
Is malignant transformation common for Fibrous Dysplasia
No, reported if lesion treat w/ radiation
71
Benign reactive process that may originate from PDL of fibroblast, most often seen in African American Females
Cemento-Osseous Dysplasia, can affect any sex or ethnic group
72
3 forms based on severity
1) Mild, Periapical Cemento-Osseous Dysplasia 2) Moderate- Focal Cemento-Osseous Dysplasia 3) Florid Cemento-Osseous Dysplasia, severe
73
Areas, radiographic appearance and vitality of teeth in Cemento-Osseous Dysplasia
Any tooth-bearing area, PDL remains intact (radiolucent/opaque) ** Teeth test vital
74
Area and ethnicity Periapical Cemento-Osseous Dysplasia is found
Mandibular ant. of AA women
75
Radiographic appearance of Periapical Cemento-Osseous Dysplasia
Radiolucencies at apices, developing central opacity
76
Diagnosis and DD for Periapical Cemento-Osseous Dysplasia
Hypercementosis (radiodensity only in PDL), Idiopathic ostersclerosis( not necessarily at apex), benign cementoblastoma - Factor: Clinical/radiographic findings
77
Tx/Prognosis for Periapical Cemento-Osseous Dysplasia
None, excellent
78
Ethnic group for Focal Cemento-Osseous Dysplasia
All, whites more common
79
Condition Focal Cemento-Osseous Dysplasia is confused with and which is more common
FCOD more common, central-ossifying fibroma | Both seen in younger adult women
80
Age, location, sex common in Focal Cemento-Osseous Dysplasia
Body of mandible, female 20-40
81
Signs/Symptoms of Focal Cemento-Osseous Dysplasia
Unilocular radiolucent, pain and swelling is unusual
82
Condition of bone obtained in surgery, and micro apperance
- Small, gritty fragments obtained (poorly defined from surrounding bone) - CT w/ mineralized tissue, resembling woven bone or cellular cementum
83
Tx and Prog for Focal Cemento-Osseous Dysplasia
Biopsy, none, good unless first sign of Florid Cemento-Osseous Dysplasia
84
Age and ethnicity for Florid Cemento-Osseous Dysplasia
Middle age and older AA women
85
How many areas affected by Florid Cemento-Osseous Dysplasia
Multiple quadrants
86
Symptoms of Florid Cemento-Osseous Dysplasia
None, unless overlying area ulcerates and results in sequestration
87
Radiographic apperance of Florid Cemento-Osseous Dysplasia
cotton wool, become more radiodense overtime
88
What condition may be associated with Florid Cemento-Osseous Dysplasia
Simple bone cyst
89
Can an implant be placed in area of Cemento-Osseous Dysplasia
No, lesions are hypovasular and prone to necrosis and secondary infection
90
Is a biopsy always necessary if Cemento-Osseous Dysplasia is suspected
No, diagnose off clinical features
91
Why should patients with Cemento-Osseous Dysplasia be encouraged to retain teeth
Symptoms can present after lesion exposure
92
Treatment for asymptomatic patient with Cemento-Osseous Dysplasia Symptmatic
Asymptomatic- Prophy and OHI | Symptomatic- Debridgement and antibiotics w/ secondary myelitis
93
Overall prognosis for Cemento-Osseous Dysplasia
Good, risk for secondary infection, rare malignant transformation