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Oral Path Exam 3 > Developmental Bone Disorders > Flashcards

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
Q

Incidental Finding in x-ray, very radiopaque w/ no bone expansion

A

Idiopathic Osterosclerosis

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26
Q

Other terms for Idiopathic Osterosclerosis

A

Dense bone island/Enostosis

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27
Q

Most common region for Idiopathic Osterosclerosis

A

Molar/PM

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28
Q

Micro appearance of bone w/ Idiopathic Osterosclerosis

A

Dense/viable

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29
Q

DD for Idiopathic Osterosclerosis

A

Condensing osteitis, hypercementosis, cementoblastoma

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30
Q

Painless, bilateral expansion of the jaws especially he mandible detected in childhood

A

Cherubism

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31
Q

Cause of Cherubism

A

AD or de novo mutation

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32
Q

Result of inf/lat orbital walls being affected by Cherubism

A

Eyeballs tilt upwards and lower eyelids are retracted

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33
Q

Dental radiographic findings w/ Cherubism

A

Bilateral/multilocular radiolocuncies of post. mandible

Displacement of teeth

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34
Q

Microscope apperance of Cherubism

A

Edematous/cellular CT, sparse multinucleated giant cells, perivascular hyalinization

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35
Q

Treatment of Cherubism

A

Surgery may accelerate, involute during puberty

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36
Q

Condition seen in 1st/2nd decade, well-circumscribed radiolucency that can scallop between roots

A

Simple Bone Cyst (Traumatic bone cyst)

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37
Q

Sex predilection for Simple Bone Cyst

A

Male

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38
Q

Location most common for Simple Bone Cyst

A

Posterior mandible

39
Q

Cause of Simple Bone Cyst

A

Idiopathic, relation to trauma

40
Q

Findings upon exploration of Simple Bone Cyst

A

Empty bone cavity, fragments of bone lined by chronically inflamed granulation tissue

41
Q

Why or why not is a Simple Bone Cyst considered a true cyst

A

Doesn’‘t have epithelial lining, not a true cyst

42
Q

Treatment of Simple Bone Cyst

A

Induce bleeding to organize/heal lesion

43
Q

What bones have Simple Bone Cysts been observed

A

Every bone in the body

44
Q

AKA as Peget disease of bone, condition from abnormal resorption and deposition of bone, resulting in bone distortion

A

Osteitis Deformans

45
Q

How is Osteitis Deformans usually discovered

A

Routine blood test or dental radiograph

46
Q

Condition of affected bones w/ Osteitis Deformans

A

Thickened and weakened

47
Q

Age/sex/ancestry associated with Osteitis Deformans

A

Male 2:1, >40, Anglo-Saxon

48
Q

MArkedly elevated serum alkaline phophatase is associated with what condition

A

Osteitis Deformans

49
Q

What perfect of patients have bone pain with Osteitis Deformans

A

40

50
Q

Are most cases of Osteitis Deformans poly or monostotic

A

polystotic

51
Q

Result of femurs being affected with Osteitis Deformans

A

Simian stance

52
Q

Jaws are involved in what percent of patients w/ Osteitis Deformans

A

17%

53
Q

Maxilla or mandible more affected by Osteitis Deformans

A

Maxilla

54
Q

Radiographic appearance of Osteitis Deformans

A

Cotton wool, mosaic pattern (irregular trabeulae), rimmed by osteoclasts/blasts

55
Q

Other dental condition seen with Osteitis Deformans

A

Hypercementosis

56
Q

What is bone marrow replaced by in Osteitis Deformans

A

Vascular fibrous CT

57
Q

Treatment/Prognosis for Osteitis Deformans

A

Not life-threatening

  • No Tx if asymptomatic
  • Bisphosphonates
  • Monitor for other bone tumors
58
Q

5 Potential Dental complications with Osteitis Deformans

A

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
Q

Developmental, tumor like lesion that results in painless unilateral swelling w/ slow growth

A

Fibrous Dysplasia

60
Q

Gene mutated that results in Fibrous Dysplasia

A

GNAS1 (tumor suppressor gene)

61
Q

Age Fibrous Dysplasia usually presents

A

1st or 2nd decade

62
Q

What percent of Fibrous Dysplasias are monostotic

A

70-85%

63
Q

Are jaws commonly affect, and max or mand more

A

Yes, Max more

64
Q

More severe presentation of Fibrous Dysplasia

A

Craniofacial Fibrous Dysplasia

65
Q

Cause of facial deformity with Craniofacial Fibrous Dysplasia

A

Maxillary lesions involve adjacent bones

66
Q

Radiographic appearance of Fibrous Dysplasia

A

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
Q

2 Types of Polyostotic Fibrous Dysplasia and their symptoms

A

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
Q

Tx for Fibrous Dysplasia

A
  • Small- None or en bloc resection
  • Large- Surgical
  • Can stabalize with skeletal maturation
69
Q

What percent of surgically treated lesions show regrowth in Fibrous Dysplasia

A

25-50%

70
Q

Is malignant transformation common for Fibrous Dysplasia

A

No, reported if lesion treat w/ radiation

71
Q

Benign reactive process that may originate from PDL of fibroblast, most often seen in African American Females

A

Cemento-Osseous Dysplasia, can affect any sex or ethnic group

72
Q

3 forms based on severity

A

1) Mild, Periapical Cemento-Osseous Dysplasia
2) Moderate- Focal Cemento-Osseous Dysplasia
3) Florid Cemento-Osseous Dysplasia, severe

73
Q

Areas, radiographic appearance and vitality of teeth in Cemento-Osseous Dysplasia

A

Any tooth-bearing area, PDL remains intact (radiolucent/opaque)
** Teeth test vital

74
Q

Area and ethnicity Periapical Cemento-Osseous Dysplasia is found

A

Mandibular ant. of AA women

75
Q

Radiographic appearance of Periapical Cemento-Osseous Dysplasia

A

Radiolucencies at apices, developing central opacity

76
Q

Diagnosis and DD for Periapical Cemento-Osseous Dysplasia

A

Hypercementosis (radiodensity only in PDL), Idiopathic ostersclerosis( not necessarily at apex), benign cementoblastoma
- Factor: Clinical/radiographic findings

77
Q

Tx/Prognosis for Periapical Cemento-Osseous Dysplasia

A

None, excellent

78
Q

Ethnic group for Focal Cemento-Osseous Dysplasia

A

All, whites more common

79
Q

Condition Focal Cemento-Osseous Dysplasia is confused with and which is more common

A

FCOD more common, central-ossifying fibroma

Both seen in younger adult women

80
Q

Age, location, sex common in Focal Cemento-Osseous Dysplasia

A

Body of mandible, female 20-40

81
Q

Signs/Symptoms of Focal Cemento-Osseous Dysplasia

A

Unilocular radiolucent, pain and swelling is unusual

82
Q

Condition of bone obtained in surgery, and micro apperance

A
  • Small, gritty fragments obtained (poorly defined from surrounding bone)
  • CT w/ mineralized tissue, resembling woven bone or cellular cementum
83
Q

Tx and Prog for Focal Cemento-Osseous Dysplasia

A

Biopsy, none, good unless first sign of Florid Cemento-Osseous Dysplasia

84
Q

Age and ethnicity for Florid Cemento-Osseous Dysplasia

A

Middle age and older AA women

85
Q

How many areas affected by Florid Cemento-Osseous Dysplasia

A

Multiple quadrants

86
Q

Symptoms of Florid Cemento-Osseous Dysplasia

A

None, unless overlying area ulcerates and results in sequestration

87
Q

Radiographic apperance of Florid Cemento-Osseous Dysplasia

A

cotton wool, become more radiodense overtime

88
Q

What condition may be associated with Florid Cemento-Osseous Dysplasia

A

Simple bone cyst

89
Q

Can an implant be placed in area of Cemento-Osseous Dysplasia

A

No, lesions are hypovasular and prone to necrosis and secondary infection

90
Q

Is a biopsy always necessary if Cemento-Osseous Dysplasia is suspected

A

No, diagnose off clinical features

91
Q

Why should patients with Cemento-Osseous Dysplasia be encouraged to retain teeth

A

Symptoms can present after lesion exposure

92
Q

Treatment for asymptomatic patient with Cemento-Osseous Dysplasia
Symptmatic

A

Asymptomatic- Prophy and OHI

Symptomatic- Debridgement and antibiotics w/ secondary myelitis

93
Q

Overall prognosis for Cemento-Osseous Dysplasia

A

Good, risk for secondary infection, rare malignant transformation

Oral Path Exam 3 (4 decks)

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