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

Developmental Bone Disorders Flashcards

(100 cards)

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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
True or False: the AD osteopetrosis form can have long-term survival.
true
26
Cleidocranial Dysplasia is an uncommon autosomal _____ condition that affects the development of bones and teeth.
dominant
27
Cleidocranial dysplasia primarily affects which bones?
jaw skull clavicles
28
True or False: Patients with cleidocranial dysplasia typically have a retained primary dentition.
True, permanent teeth don't erupt or are impacted ****they HAVE plenty of teeth (sometimes even too many) but they just don't erupt****
29
What are some clinical signs of cleidocranial dysplasia?
prominent forehead hypoplastic midface long neck missing or hypoplastic clavicles = able to approximate shoulders
30
What is the treatment for cleidocranical dysplasia?
combined surgical and orthodontic care to correct skeletal relationships removal of extra teeth- bring permanent into occlusion
31
True or False: the lifespan of cleidocranial dysplasia patients is normal.
true, its more of a cosmetic problem
32
What is an "osteoporotic bone marrow defect?"
Usually it's an incidental finding via radiographic imaging that is asymptomatic and has an unknown pathogenesis (resembles metastatic disease)
33
Where are osteoporotic bone marrow defects usually seen?
in the body of the mandible at an old extraction site in middle age females
34
What are the microscopic findings of Osteoporotic Bone Marrow Defects?
Fatty and hematopoietic marrow
35
How does an Osteoporotic Bone Marrow Defect appear on radiographs?
Panoramic: radiolucent and somewhat circumscribed Periapical: ill-defined borders and fine central trabeculations
36
In addition to "Osteoporotic Bone Marrow Defects," what is another incidental radiographic finding?
Idiopathic Osteosclerosis
37
Other terms for Idiopathic Osteosclerosis include: ________ __________
Enostosis | Dense Bone Island
38
Idiopathic Osteosclerosis is an area of ______ on xray and is most commonly seen in the _____ region.
radiopacity (without expansion) with sharp or blended borders premolar-molar
39
Idiopathic osteosclerosis is commonly mislabeled as which three conditions?
condensing osteitis hypercementosis cementoblastoma
40
How is condensing osteitis differentiated from idiopathic osteosclerosis?
condensing osteitis is an area of decay surrounding the root and has a WIDENED PDL
41
How is cementoblastoma differentiated from idiopathic osteosclerosis?
Cementoblastoma occurs in the cementum and therefore is a radiopacity that is SURROUNDED BY the PDL
42
True or False: Idiopathic Osteosclerosis appears in areas between teeth and shows no alteration in the PDL.
True *also, histiologically the bone is dense and vital*
43
What is cherubism?
autosomal dominant condition (or de novo) that causes painless, bilateral expansion of the jaws- detected in childhood
44
True or False: The mandible is less likely affected by cherubism
false: mandible is common, maxilla is less frequently involved
45
What is the cause for "eyes upturned to heaven" appearance associated with cherubism?
involvement of the inferior/lateral orbit walls may tilt the eyeballs upward and retract the lower lids = exposing the sclera below the iris
46
What is the radiographic appearance of cherubism?
BILATERAL multilocular radiolucencies of posterior mandible and displacement of teeth
47
Microscopic evidence of cherubism includes: ______ connective tissue _______ cells ________ hyalinization
edematous, cellular, fibrous CT multinucleated giant cells perivascular hyalinization
48
True or False: Surgical intervention is the treatment of choice for cherubism.
FALSE! it has been known to accelerate the growth of some lesions
49
Many cases of cherubism have _____ during puberty
involuted
50
Which idiopathic bone lesions has a predilection for the posterior mandible of males?
simple bone cyst (traumatic bone cyst)
51
Simple bone cysts have a questionable relation to _____.
trauma
52
What is the radiographic appearance of simple bone cysts?
well-circumscribed radiolucency that scallops between roots (lamina dura remain in tact/normal)
53
Upon surgical exploration of a simple bone cyst, what is found?
an empty cavity lined by chronically inflamed granulation tissue (no epithelial lining = not a "true cyst")
54
What is the recommended treatment of a traumatic bone cyst?
induce bleeding within the hollow cavity to stimulate bone fill
55
What is Osteitis Deformans?
abnormal resorption and deposition of bone that results in thick/weak bones and distortion (aka Paget Disease of Bone)
56
True or False: Osteitis Deformans is often discovered incidentally before age forty.
False: it is rare in patients <40 years old
57
True or False: Osteitis Deformans is more common in men.
True: men 2-to-1 | **deforMANs**
58
Which enzyme in markedly elevated in osteitis deformans?
serum alkaline phosphatase
59
How often is bone pain associated with osteitis deformans?
40% at presentation
60
If a femur appears moth-eaten radiographically, what is often seen clincally in this osteitis deformans patient?
simian stance (monkey-like) due to bowing of legs
61
How often are the jaws affected by osteitis deformans?
17% of patients
62
Which is affected more often, maxilla or mandible? How does the bone look, radiographically?
maxilla | cotton-wool or moth-eaten
63
True of False: Osteitis deformans often presents with generalized hypercementosis of teeth
True
64
If a patient reports that their "hat won't fit" or "denture stopped fitting," what condition would you expect?
Paget Disease of Bone (osteitis deformans) *remember, appears >40years old
65
How does osteitis deformans appears microscopically?
-irregular trebeculae with resting and reversal lines "mosaic pattern" -marrow replaced by vascular fibrous CT -rimmed by osteoclasts/osteoblasts
66
What is the treatment for osteitis deformans?
asymptomatic = no tx bisphosphonates monitor for development of giant cell tumor of bone and osteosarcoma
67
What are the potential dental complications associated with osteitis deformans?
- 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
Fibrous Dysplasia is a fibro-osseous, tumor-like lesion of the jaw. Recent work suggests post-zygotic mutation of the tumor suppressor gene _______.
GNAS1
69
When does fibrous dysplasia present?
first or second decade
70
Does fibrous displasis have a sex predilection?
no
71
How often is Fibrous Dysplasia monostotic (one bone)?
70-85%
72
True or False: The jaws are among the most commonly affected bones of Fibrous Dysplasia.
True, maxilla> mandible
73
True or False: Fibrous Dysplasia is painless
True, slow-growing, painless unilateral swelling
74
What is a more severe presentation of Fibrous dysplasia?
Craniofacial Fibrous Dysplasia | -may involve facial bones including the sphenoid, zygoma, and occiput
75
What is the classic radiographic presentation of fibrous dysplasia?
"Ground Glass" - irregularly shaped trabeculae of immature woven bone - abnormal bone fuses to adjacent normal bone - cellular intertrabecular connective tissue
76
True or False: Maxillary sinus obliteration is a common finding of fibrous dysplasia
True
77
Fibrous Dysplasia is commonly (70-85%) monostotic. What are the two polyostotic presentations?
1. Jaffe Type | 2. McCune Albright
78
Jaffe-Type Fibrous dysplasia includes two or more bones in conjunction with _______.
cafe-au-lait spots with jagged (coast of maine) borders
79
McCune-Albright type fibrous dysplasia includes two or more bones in conjunction with ______.
``` cafe-au-lait pigmentation endocrine disturbances (manifest as precocious "early" puberty) ```
80
What is the treatment for Fibrous Dysplasia?
en bloc resection for small lesions surgical reduction for cosmetic stabilization sometimes occurs with maturation
81
______ % of surgically treated Fibrous Dysplasia lesions show regrowth
25-50
82
True or False: Malignant transformation of fibrous dysplasia lesions is rare
True
83
Cemento-Osseous Dysplasias are _____, possibly reactive, processes that may originate from the _______.
benign | periodontal ligament fibroblasts
84
Cemento Osseous Dysplasia is most commonly seen in _____
african american females | then asian females, then white females
85
True or False: Cemento Osseous Dysplasias occur in tooth-bearing areas of the jaws and teeth are not vital.
False: yes tooth bearing areas are affected but the teeth test VITAL
86
What is the spectrum of severity for Cemento-Osseous Dysplasias?
Mild- Periapical Moderate- Focal Severe- Florid
87
Which area of the jaw is most commonly affected by cemento-osseous dysplasia?
anterior mandible
88
How does cemento-osseous dysplasia appear radiographically?
radiolucencies at apices with gradual central opacity developing (looks like it needs endo but the teeth are vital)
89
Differentiate between Cemento-osseous dysplasia and: hypercementosis, idiopathic osteosclerosis, cementoblastoma.
hypercementosis: radiodensity remains within the PDL idiopathic osteosclerosis: not at apex, no lucent border cementoblastoma: radiopaque mass around one root
90
True or False: No treatment is necessary for Periapical Cemento-Osseous Dysplasia.
True
91
Focal (moderate) Cemento-Osseous Dysplasia usually occurs in the _____ and has a _____ predilection.
mandible | female
92
Histological appearance of trabeculae in Focal Cemento-Osseous Dysplasia has a ______ appearance
ginger root
93
True or False: Treatment of Focal Cemento-osseous dysplasia is unnecessary.
True but biopsy is often warranted
94
What is the most severe presentation of Cemento-Osseous dysplasia? Which population group is most affected?
Florid | middle-age african american females
95
Florid-COD is generally asymptomatic but it affects _______ of the jaws.
multiple quadrants of the jaws | asymptomatic as long as there is no ulceration/sequestration
96
How does florid-COD appear radiographically?
cotton-wool radiopacities in multiple quads
97
true or false: biopsy is needed to diagnose florid-COD.
false, typical radiographic presentation
98
Lesions of florid-COD tend to be ______vascular and prone to _____ or secondary infection with minimal provocation.
hypovascular | necrosis
99
What is the treatment/management of cemento-osseous dysplasias?
regular recalls/OHI/prophylaxis prevent tooth loss which could expose sclerotic mass debridement/antibiotics if symptomatic
100
Development of ______ within an area of cemento-osseous dysplasia has been reported but is extremely rare.
sarcoma

AU 16- Oral Path MT 3 (7 decks)

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