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AU 16- Oral Path MT 3 > Developmental Bone Disorders > Flashcards

Developmental Bone Disorders Flashcards

1
Q

Osteogenesis Imperfecta is characterized by defective _______ which results in abnormal bone _______ and low bone density (osteopenia).

A

collagen

mineralization

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2
Q

True or False: Osteogenesis Imperfecta is also known as “Brittle Bone Disease”

A

True

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3
Q

True of False: OI is a common disorder of bone.

A

False: it is a rare disorder of bone (overall)

*however, it is one of the most common HERITABLE bone diseases

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4
Q

OI is the mutation of a collagen gene. What is the inheritance type?

A

autosomal dominant or recessive

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5
Q

Is OI more commonly recessive or dominant?

A

90% are autosomal DOMINANT (“DOI”-dominant.O.I)

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6
Q

True or False: If you have OI, the degree of fragility is always rather severe.

A

False: Severity varies widely depending on the mutation

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7
Q

What are the clinical features of osteogenesis imperfecta?

A

blue sclera
hearing loss
craniofacial (class III occlusion/triangular facies)
bowing of long bones
translucent teeth (yellow, blue, or brown)
severe attrition leading to loss of VDO
radiographic “shell teeth” - Pulpal obliteration
Narrow roots/Corn Cob chaped

(“a B.A.T.C.H of corn shells”)
Blue/Bowing, Attrition, Translucence, Cranio, Hearing
corn cob roots
shell teeth

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8
Q

True or False: OI and DI have similar tooth alterations because they are similar mutations and the diseases are related.

A

False: Distinct mutations, Different Diseases, with similar tooth alterations

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9
Q

Dental defects associated with OI should be designated as _______. However, alterations that are limited only to the teeth (not bone) would be considered “______”

A

opalescent teeth

dentinogenesis imperfecta

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10
Q

What are the mainstays of treatment for OI?

A 
physiotherapy
rehabilitation
orthopedic surgery
minimize factors that cause fractures
IV bisphosphonates (children with mod-severe pain)
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11
Q

True or False: Some OI patients have very minimal bone deformity and have normal growth.

A

True

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12
Q

In severe forms of OI, how could an infant die?

A

crushing during passage through the birth canal :(

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13
Q

Osteopetrosis is a (common or rare?) bone disease that is caused by a lack of _________ activity.

A

rare

osteoclastic

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14
Q

What is worse, the autosomal dominant or recessive form of osteopetrosis?

A

recessive

fractures and osteomyelitis are common in the AR form due to decreased vascularity/healing ability

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15
Q

What is the results of osteoclastic inactivity?

A

increased bone density

decreased osteoclast + continued bone formation

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16
Q

What is seen on the CT scan of a patient with osteopetrosis?

A

thickening of bones of the skull

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17
Q

Why might someone with osteopetrosis have blindness or deafness?

A

cranial nerve compression

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18
Q

True or False: Osteopetrosis leads to pancytopenia

A

True: Marrow spaces are filled with dense bone = loss of hematopoietic precursor cells

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19
Q

What are the risks associated with pancytopenia?

A

increased susceptibility to infection

osteomyelitis of the jaws and extraction complications

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20
Q

True or False: A patient with osteopetrosis could be seen clinically with draining sinus tracts or crowded teeth.

A

True:
infections in bone that has nowhere else to spread
bone is too dense, teeth erupt irregularly or not at all

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21
Q

Why is it difficult to see an osteopetrosis patient’s tooth roots in a radiograph?

A

difficult to discern due to the density of surrounding bone

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22
Q

True or False: The treatment of choice for osteopetrosis is a bone marrow transplant.

A

False: limited success of transplants

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23
Q

What is the treatment for osteopetrosis?

A

Supportive measures
transfusions
antibiotics when needed
alternative therapies (interferon + calcitriol + corticosteroids + erythropoietin + dec. calclium intake)

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24
Q

The AR osteopetrosis form has a ____ prognosis.

A

Poor

many patients die before age 20 years

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25
Q

True or False: the AD osteopetrosis form can have long-term survival.

A

true

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26
Q

Cleidocranial Dysplasia is an uncommon autosomal _____ condition that affects the development of bones and teeth.

A

dominant

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27
Q

Cleidocranial dysplasia primarily affects which bones?

A

jaw
skull
clavicles

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28
Q

True or False: Patients with cleidocranial dysplasia typically have a retained primary dentition.

A

True, permanent teeth don’t erupt or are impacted

they HAVE plenty of teeth (sometimes even too many) but they just don’t erupt

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29
Q

What are some clinical signs of cleidocranial dysplasia?

A

prominent forehead
hypoplastic midface
long neck
missing or hypoplastic clavicles = able to approximate shoulders

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30
Q

What is the treatment for cleidocranical dysplasia?

A

combined surgical and orthodontic care to correct skeletal relationships
removal of extra teeth- bring permanent into occlusion

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31
Q

True or False: the lifespan of cleidocranial dysplasia patients is normal.

A

true, its more of a cosmetic problem

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32
Q

What is an “osteoporotic bone marrow defect?”

A

Usually it’s an incidental finding via radiographic imaging that is asymptomatic and has an unknown pathogenesis (resembles metastatic disease)

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33
Q

Where are osteoporotic bone marrow defects usually seen?

A

in the body of the mandible
at an old extraction site
in middle age females

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34
Q

What are the microscopic findings of Osteoporotic Bone Marrow Defects?

A

Fatty and hematopoietic marrow

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35
Q

How does an Osteoporotic Bone Marrow Defect appear on radiographs?

A

Panoramic: radiolucent and somewhat circumscribed
Periapical: ill-defined borders and fine central trabeculations

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36
Q

In addition to “Osteoporotic Bone Marrow Defects,” what is another incidental radiographic finding?

A

Idiopathic Osteosclerosis

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37
Q

Other terms for Idiopathic Osteosclerosis include:
________
__________

A

Enostosis

Dense Bone Island

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38
Q

Idiopathic Osteosclerosis is an area of ______ on xray and is most commonly seen in the _____ region.

A

radiopacity (without expansion) with sharp or blended borders
premolar-molar

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39
Q

Idiopathic osteosclerosis is commonly mislabeled as which three conditions?

A

condensing osteitis
hypercementosis
cementoblastoma

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40
Q

How is condensing osteitis differentiated from idiopathic osteosclerosis?

A

condensing osteitis is an area of decay surrounding the root and has a WIDENED PDL

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41
Q

How is cementoblastoma differentiated from idiopathic osteosclerosis?

A

Cementoblastoma occurs in the cementum and therefore is a radiopacity that is SURROUNDED BY the PDL

42
Q

True or False: Idiopathic Osteosclerosis appears in areas between teeth and shows no alteration in the PDL.

A

True

also, histiologically the bone is dense and vital

43
Q

What is cherubism?

A

autosomal dominant condition (or de novo) that causes painless, bilateral expansion of the jaws- detected in childhood

44
Q

True or False: The mandible is less likely affected by cherubism

A

false: mandible is common, maxilla is less frequently involved

45
Q

What is the cause for “eyes upturned to heaven” appearance associated with cherubism?

A

involvement of the inferior/lateral orbit walls may tilt the eyeballs upward and retract the lower lids = exposing the sclera below the iris

46
Q

What is the radiographic appearance of cherubism?

A

BILATERAL multilocular radiolucencies of posterior mandible and displacement of teeth

47
Q

Microscopic evidence of cherubism includes:
______ connective tissue
_______ cells
________ hyalinization

A

edematous, cellular, fibrous CT
multinucleated giant cells
perivascular hyalinization

48
Q

True or False: Surgical intervention is the treatment of choice for cherubism.

A

FALSE! it has been known to accelerate the growth of some lesions

49
Q

Many cases of cherubism have _____ during puberty

A

involuted

50
Q

Which idiopathic bone lesions has a predilection for the posterior mandible of males?

A

simple bone cyst (traumatic bone cyst)

51
Q

Simple bone cysts have a questionable relation to _____.

A

trauma

52
Q

What is the radiographic appearance of simple bone cysts?

A

well-circumscribed radiolucency that scallops between roots (lamina dura remain in tact/normal)

53
Q

Upon surgical exploration of a simple bone cyst, what is found?

A

an empty cavity lined by chronically inflamed granulation tissue (no epithelial lining = not a “true cyst”)

54
Q

What is the recommended treatment of a traumatic bone cyst?

A

induce bleeding within the hollow cavity to stimulate bone fill

55
Q

What is Osteitis Deformans?

A

abnormal resorption and deposition of bone that results in thick/weak bones and distortion
(aka Paget Disease of Bone)

56
Q

True or False: Osteitis Deformans is often discovered incidentally before age forty.

A

False: it is rare in patients <40 years old

57
Q

True or False: Osteitis Deformans is more common in men.

A

True: men 2-to-1

deforMANs

58
Q

Which enzyme in markedly elevated in osteitis deformans?

A

serum alkaline phosphatase

59
Q

How often is bone pain associated with osteitis deformans?

A

40% at presentation

60
Q

If a femur appears moth-eaten radiographically, what is often seen clincally in this osteitis deformans patient?

A

simian stance (monkey-like) due to bowing of legs

61
Q

How often are the jaws affected by osteitis deformans?

A

17% of patients

62
Q

Which is affected more often, maxilla or mandible? How does the bone look, radiographically?

A

maxilla

cotton-wool or moth-eaten

63
Q

True of False: Osteitis deformans often presents with generalized hypercementosis of teeth

A

True

64
Q

If a patient reports that their “hat won’t fit” or “denture stopped fitting,” what condition would you expect?

A

Paget Disease of Bone (osteitis deformans)

*remember, appears >40years old

65
Q

How does osteitis deformans appears microscopically?

A

-irregular trebeculae with resting and reversal lines
“mosaic pattern”
-marrow replaced by vascular fibrous CT
-rimmed by osteoclasts/osteoblasts

66
Q

What is the treatment for osteitis deformans?

A

asymptomatic = no tx
bisphosphonates
monitor for development of giant cell tumor of bone and osteosarcoma

67
Q

What are the potential dental complications associated with osteitis deformans?

A
  • difficulty extracting teeth
  • extensive hemorrhage from oral surgery during “vascular lytic stage”
  • poor wound healing during “avascular sclerotic phase”
  • edentulous patients require new dentures regularly
  • unfavorable situation for osseointegration of implants
68
Q

Fibrous Dysplasia is a fibro-osseous, tumor-like lesion of the jaw. Recent work suggests post-zygotic mutation of the tumor suppressor gene _______.

A

GNAS1

69
Q

When does fibrous dysplasia present?

A

first or second decade

70
Q

Does fibrous displasis have a sex predilection?

A

no

71
Q

How often is Fibrous Dysplasia monostotic (one bone)?

A

70-85%

72
Q

True or False: The jaws are among the most commonly affected bones of Fibrous Dysplasia.

A

True, maxilla> mandible

73
Q

True or False: Fibrous Dysplasia is painless

A

True, slow-growing, painless unilateral swelling

74
Q

What is a more severe presentation of Fibrous dysplasia?

A

Craniofacial Fibrous Dysplasia

-may involve facial bones including the sphenoid, zygoma, and occiput

75
Q

What is the classic radiographic presentation of fibrous dysplasia?

A

“Ground Glass”

  • irregularly shaped trabeculae of immature woven bone
  • abnormal bone fuses to adjacent normal bone
  • cellular intertrabecular connective tissue
76
Q

True or False: Maxillary sinus obliteration is a common finding of fibrous dysplasia

A

True

77
Q

Fibrous Dysplasia is commonly (70-85%) monostotic. What are the two polyostotic presentations?

A
  1. Jaffe Type

2. McCune Albright

78
Q

Jaffe-Type Fibrous dysplasia includes two or more bones in conjunction with _______.

A

cafe-au-lait spots with jagged (coast of maine) borders

79
Q

McCune-Albright type fibrous dysplasia includes two or more bones in conjunction with ______.

A 
cafe-au-lait pigmentation
endocrine disturbances (manifest as precocious "early" puberty)
80
Q

What is the treatment for Fibrous Dysplasia?

A

en bloc resection for small lesions
surgical reduction for cosmetic
stabilization sometimes occurs with maturation

81
Q

______ % of surgically treated Fibrous Dysplasia lesions show regrowth

A

25-50

82
Q

True or False: Malignant transformation of fibrous dysplasia lesions is rare

A

True

83
Q

Cemento-Osseous Dysplasias are _____, possibly reactive, processes that may originate from the _______.

A

benign

periodontal ligament fibroblasts

84
Q

Cemento Osseous Dysplasia is most commonly seen in _____

A

african american females

then asian females, then white females

85
Q

True or False: Cemento Osseous Dysplasias occur in tooth-bearing areas of the jaws and teeth are not vital.

A

False: yes tooth bearing areas are affected but the teeth test VITAL

86
Q

What is the spectrum of severity for Cemento-Osseous Dysplasias?

A

Mild- Periapical
Moderate- Focal
Severe- Florid

87
Q

Which area of the jaw is most commonly affected by cemento-osseous dysplasia?

A

anterior mandible

88
Q

How does cemento-osseous dysplasia appear radiographically?

A

radiolucencies at apices with gradual central opacity developing (looks like it needs endo but the teeth are vital)

89
Q

Differentiate between Cemento-osseous dysplasia and: hypercementosis, idiopathic osteosclerosis, cementoblastoma.

A

hypercementosis: radiodensity remains within the PDL
idiopathic osteosclerosis: not at apex, no lucent border
cementoblastoma: radiopaque mass around one root

90
Q

True or False: No treatment is necessary for Periapical Cemento-Osseous Dysplasia.

A

True

91
Q

Focal (moderate) Cemento-Osseous Dysplasia usually occurs in the _____ and has a _____ predilection.

A

mandible

female

92
Q

Histological appearance of trabeculae in Focal Cemento-Osseous Dysplasia has a ______ appearance

A

ginger root

93
Q

True or False: Treatment of Focal Cemento-osseous dysplasia is unnecessary.

A

True but biopsy is often warranted

94
Q

What is the most severe presentation of Cemento-Osseous dysplasia? Which population group is most affected?

A

Florid

middle-age african american females

95
Q

Florid-COD is generally asymptomatic but it affects _______ of the jaws.

A

multiple quadrants of the jaws

asymptomatic as long as there is no ulceration/sequestration

96
Q

How does florid-COD appear radiographically?

A

cotton-wool radiopacities in multiple quads

97
Q

true or false: biopsy is needed to diagnose florid-COD.

A

false, typical radiographic presentation

98
Q

Lesions of florid-COD tend to be ______vascular and prone to _____ or secondary infection with minimal provocation.

A

hypovascular

necrosis

99
Q

What is the treatment/management of cemento-osseous dysplasias?

A

regular recalls/OHI/prophylaxis
prevent tooth loss which could expose sclerotic mass
debridement/antibiotics if symptomatic

100
Q

Development of ______ within an area of cemento-osseous dysplasia has been reported but is extremely rare.

A

sarcoma

AU 16- Oral Path MT 3 (7 decks)

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