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BONE DISORDERS (ppt) Flashcards

1
Q

T/F

OSTEOGENESIS IMPERFECTA is autosomal dominant

A

T

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2
Q

“Brittle Bones”

A

OSTEOGENESIS IMPERFECTA

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3
Q

Fragilatis Ossium

A

OSTEOGENESIS IMPERFECTA

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4
Q

Osteopsathyrosis

A

OSTEOGENESIS IMPERFECTA

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5
Q

Lobstein’s Disease

A

OSTEOGENESIS IMPERFECTA

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6
Q

abnormality in the type I collagen (bone, ligament, dentin, and sclera)

A

OSTEOGENESIS IMPERFECTA

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7
Q

fragility of bones

A

OSTEOGENESIS IMPERFECTA

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8
Q

OSTEOGENESIS IMPERFECTA has 2 Forms:

A
  1. Vrolik’s type
  2. Lobstein type
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9
Q

T/F

OSTEOGENESIS IMPERFECTA increase bone mass

A

False

(INCREASE bone mass)

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10
Q

Extreme fragility /porosity of bones

A

OSTEOGENESIS IMPERFECTA

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11
Q
  • abnormal electrical response of muscles
  • Triangular-shaped head
  • Pale, blue sclera

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS
Q. CHERUBISM

A

A

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12
Q
  • Deafness
  • Skin: thin and translucent
  • Subcutaneous capillary bleeding

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS
Q. CHERUBISM

A

A

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13
Q

Oral Manifestations / Radiographic Findings

  • teeth: bulbous crowns, with narrow, constricted roots and obliterated canals

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

A

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14
Q

Oral Manifestations / Radiographic Findings

  • blue-gray to yellowish-brown teeth
  • Anterior and posterior cross bites and open bites
    maxillary hypoplasia rather than mandibular hyperplasia

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

A

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15
Q

Histologic Features:
1. bone: thin cortices, immature spongy bone

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

A

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16
Q

*cortical bone replaced with fibrous tissue
* retarded osteoblastic activity

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

A

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17
Q

Treatment and prognosis of OSTEOGENESIS IMPERFECTA

A

Treatment and Prognosis:
> no definite treatment (bisphonates)
> immobilization of fractures
> may improve after puberty

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18
Q

Caffey’s Disease,

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

B

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19
Q

Clinical Features:
1. tender, deeply-placed soft tissue swellings
2. systemic manifestations
3. cortical thickening

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

B

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20
Q

Caffey-Silverman Syndrome

A

Infantile cortical hyperostosis

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21
Q

DOral Manifestations:
1. assymetric deformity of mandible
2. facial swelling

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

B

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22
Q

©Radiographic Manifestations:
1. unilateral / bilateral
thickening of cortex

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

B

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23
Q

@Prognosis:
1. may regress without treatment
2. may recurr
3. conservative treatment

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

B

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24
Q

Marie and Sainton’s Disease

A

. CLEIDOCRANIAL DYSPLASIA

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25
Q

Scheuthauer-Marie-Sainton Syndrome, Mutational Dysostosis

A

. CLEIDOCRANIAL DYSPLASIA

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26
Q

Vertical midline furrow
with frontal bossing

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

C

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27
Q

> Depressed midface
Prominent chin

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

C

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28
Q

Oral Manifestations:

prolonged retention of deciduous teeth and delayed
eruption of succedaneous teeth

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

C

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29
Q

Oral Manifestations:

*high, narrow arched palate
*underdeveloped maxilla and maxillary sinus

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

C

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30
Q

CLEIDOCRANIAL DYSPLASIA treatment

A

Treatment:
1. care of deciduous teeth
2. multidisciplinary
approach

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31
Q

•Clinical Features:
1. affects head, face
2. other areas:
Spina bifida occulta

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

C

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32
Q

T/F

Craniofacial Dysostosis:

•Cranial synostosis with syndactyly

A

False

•Cranial synostosis WITHOUT syndactyly

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33
Q

T/F

Craniofacial Dysostosis is hereditary

A

T

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34
Q

Triangular frontal defect
Steep forehead
Hypertelorism
Exophthalmus
Antimongoloid slope of eyes

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

D

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35
Q

Parrot-beak nose

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

D

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36
Q

Frog-like features

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

D

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37
Q

Oral Features:
1. hypoplasia of maxilla
2. mandibular prognathism
3. high-arched palate

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

D

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38
Q

Craniofacial Dysostosis treatment

A

Treatment and Prognosis:
1. craniectomy; cosmetic surgery
2. may lead normal lives
3. may or may not be retarted

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39
Q

coloboma of the outer portion of the lower eyelids

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E

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40
Q

*deficiency of eyelashes
*malformation of middle and inner ear
*ear: underdeveloped;
malformed; or prominent

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E

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41
Q

(Treacher-Collin Syndrome)

A

MANDIBULOFACIAL DYSOSTOSIS

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42
Q

*blind fistulas between angles of the ear and angle of mandible
* atypical hair growth
*hypoplasia of malar bones and mandible

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E

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43
Q

*increased anterior facial height
*. anterior open bite
* small chin

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E

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44
Q

bird-like or fish like features

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E. MANDIBULOFACIAL DYSOSTOSIS

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45
Q

Other Features:
1. grossly underdeveloped paranasal sinuses
2. absent / deficient auditory ossicles, cochlea, and vestibular apparatus

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E. MANDIBULOFACIAL DYSOSTOSIS

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46
Q

normal cranial vault

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

E. MANDIBULOFACIAL DYSOSTOSIS

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47
Q

MANDIBULOFACIAL DYSOSTOSIS treatment

A

©Can live a normal life span;
reconstruction of deformities

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48
Q

Anomalad

A

Pierre Robin Syndrome

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49
Q

Primary defect of Pierre Robin Syndrome

A

Micrognathia

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50
Q

Secondary defect of Pierre Robin Syndrome

A

Cleft palate

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51
Q

Tertiary defect of Pierre Robin Syndrome

A

*respiratory difficulty
*glossoptosis

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52
Q

is a malformation together with its subsequently derived structural changes, the primary defect setting off a series of secondary or even tertiary events resulting in multiple anomalies.

A

anomalad

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53
Q

Arachnodactyly

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

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54
Q

autosomal dominant; defect on chromosome 15 codes for the connective tissue protein, fibrillin

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

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55
Q

musculoskeletal, cardiac, and ocular problems predominate.

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

56
Q
  1. disproportionately long thin extremities

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

57
Q

funnel chest pigeon breast

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

58
Q

kyphosis / scoliosis

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

59
Q

*dislocation of lens of eyes; nearsightedness
*pale blue sclera
*cardiovascular complications

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

60
Q

Oral Manifestations:
1. high-arched palate
2. bifid uvula
3. malocclusions
4. odontogenic cysts

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

61
Q

TMJ dysarthrosis

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

62
Q

cardiovascular compromise: most common cause of patient death.

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

G. MARFAN SYNDROME

63
Q

Txt of Marfan Syndrome

A
  • no specific treatment
    *management of the cardiovascular manifestations
    *Patient longevity now approaches that of persons without Marfan syndrome
64
Q

T/F

Etiology of Down Syndrome is advanced maternal age

65
Q

*uterine and placental abnormalities
* chromosomal aberrations

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

H. DOWN SYNDROME

66
Q

subnormal mentality with cranial and facial deformities

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

H. DOWN SYNDROME

67
Q

Features:
1. sexual underdevelopment
2. cardiac abnormalities
3. hypermobility of joints

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

H. DOWN SYNDROME

68
Q

*short stature with short hands and fingers
*may have acute leukemia

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

H. DOWN SYNDROME

69
Q

Oral Features:
1. high-arched palate
2. microdontia
3. enamel hypoplasia
4. teeth with abnormal shape and form
5. abnormal eruption sequence
6. periodontal disease

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

H. DOWN SYNDROME

70
Q

T/ F

Oral features of DOWN SYNDROME, is high-arched palate and periodontal disease

71
Q

• Macroglossia
• open-mouthed appearance
• Bifid uvula

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

H. DOWN SYNDROME

72
Q

2 Main Types:
1. Benign Dominant
2. Malignant Recessive

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

I. OSTEOPETROSIS

73
Q

T/f

DOWN SYNDROME has Teeth with short roots

74
Q

Marble Bone Disease

A

OSTEOPETROSIS

75
Q

Albers-Schonberg Disease

A

OSTEOPETROSIS

76
Q

Osteosclerosis Fragilis Generalista

A

OSTEOPETROSIS

77
Q

Radiographic Features:

  • dense bone
  • obliterated sinuses
    *. hypoplastic maxilla

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

I. OSTEOPETROSIS

78
Q

OSTEOPETROSIS:

multiple pathologic fractures

A. Benign Dominant Form
B. Malignant Recessive Form

79
Q

OSTEOPETROSIS:

bone pain

A. Benign Dominant Form
B. Malignant Recessive Form

80
Q

OSTEOPETROSIS:

cranial nerve palsy

A. Benign Dominant Form
B. Malignant Recessive Form

81
Q

OSTEOPETROSIS:

osteomyelitis

A. Benign Dominant Form
B. Malignant Recessive Form

82
Q

OSTEOPETROSIS:

  1. optic atrophy
  2. hepatosplenomegaly

A. Benign Dominant Form
B. Malignant Recessive Form

83
Q

OSTEOPETROSIS:

*poor growth
*frontal bossing

A. Benign Dominant Form
B. Malignant Recessive Form

84
Q

OSTEOPETROSIS:

loss of hearing

A. Benign Dominant Form
B. Malignant Recessive Form

85
Q

OSTEOPETROSIS:

pathologic fractures

A. Benign Dominant Form
B. Malignant Recessive Form

86
Q

OSTEOPETROSIS:

*facial palsy
*genu valgum

A. Benign Dominant Form
B. Malignant Recessive Form

87
Q

T/ F

OSTEOPETROSIS’ Oral Manifestation are

  1. enamel hypoplasia
  2. slow root development
  3. caries - prone
  4. delayed tooth eruption
88
Q

Radiographic Features:
roots of teeth “invisible”

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

I. OSTEOPETROSIS

89
Q

T/ F

Osteopetrosis develop osteomyelitis

90
Q

T/ F
Osteopetrosis develop genu valgum

91
Q

Treatment for osteopetrosis:

Adult and infantile

A

Infantile osteopetrosis:
Calcitriol, Erythropoietin’ Corticosteroids,
Gamma interferon

Adult osteopetrosis:
requires no treatment by itself complications might require intervention.

92
Q

Features:
1. short stature with thickened musculature
2. brachycephalic skull
3. small hands and stubby fingers

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

J. ACHONDROPLASIA

93
Q

T/ F

osteopetrosis:

Prognosis:

remarkable change after bone marrow transplantation.

Adult osteopetrosis: good long-term survival rates

94
Q

T/ F

ACHONDROPLASIA is an Autosomal Dominant

95
Q

*lumbar lordosis
* limited joint movement
*. unusual physical strength and ability
*normal intelligence

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

J. ACHONDROPLASIA

96
Q

Oral Features:
1. mandibular prognathism / maxillary retrusion

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

J. ACHONDROPLASIA

97
Q

Histologic Features:
1. aplasia of the zone of calcification of endochondral growth
2. disruption of the longitudinal growth of bone

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

J. ACHONDROPLASIA

98
Q

T/F

Achondroplasia’s treatment and prognosis is none and they lives a normal lives

99
Q

T/ F

OSTEITIS DEFORMANS has specific etiology

A

False

(Non-specific etiology)

100
Q

T/ F

OSTEITIS DEFORMANS Clinical features:
1. pathologic fractures
2. bone pain
3. severe headache
4. dizziness
5. deafness and blindness
5. facial paralysis
6. weakness

101
Q

T/F

OSTEITIS DEFORMANS progressive skull enlargement, deformed spine, femur, tibia

102
Q

T/F

OSTEITIS DEFORMANS broadening and flattening of chest, areas affected feels warm

103
Q

Affected bones (sacral and lumbar vertebrae, pelvis, tibia, femur) soften and bend

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

K. OSTEITIS DEFORMANS

104
Q

Oral Manifestations:
1. progressive enlargement of maxilla with widening and flattening of the palate
2. teeth: loose, migrating
3. open-mouth appearance

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

K. OSTEITIS DEFORMANS

105
Q

Hypercementosis

Loss of lamina dura

mosaic-bone appearance

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

K. OSTEITIS DEFORMANS

106
Q

Histologic Feature:
1. mosaic-bone appearance or jigsaw puzzle appearance of bone resorption and deposition

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

K. OSTEITIS DEFORMANS

107
Q

Osteosarcoma due
to Paget’s disease

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

K. OSTEITIS DEFORMANS

108
Q

Paget’s disease of bone

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

K. OSTEITIS DEFORMANS

109
Q

O Features:
1. swelling at the angles of the mandible and bridge of nose
2. loss of vision and facial sensation
3. facial paralysis
4. deafness
5. overgrowth of alveolar process

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

L. GENERALIZED CORTICAL HYPEROSTOSIS

110
Q

T/F

GENERALIZED CORTICAL HYPEROSTOSIS has no treatment and patient can live normal lives

111
Q

Features:
1. pathologic fractures after minor trauma
2. destruction of mandible
3. facial asymmetry

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

M. MASSIVE OSTEOLYSIS

112
Q

polyostotic: clavicle, ilium, ishium, humerus, ribs, sacrum

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

M. MASSIVE OSTEOLYSIS

113
Q

Cortical and cancellous bone showing osteoporosis with an increased number of capillaries and small arterioles

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

M. MASSIVE OSTEOLYSIS

114
Q

T/F

. MASSIVE OSTEOLYSIS has No specific treatment

115
Q

osteolytic process involving the whole of the femur and vital teeth with ill-defined radiolucency

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

A

M. MASSIVE OSTEOLYSIS

116
Q

Lesions affecting multiple bones

A. McCune-Albright Syndrome
B. Jaffe type

117
Q

(Polyostotic Fibrous
Dyplasia)

A. McCune-Albright Syndrome
B. Jaffe type

118
Q

Café-au-lait pigmentation

A. McCune-Albright Syndrome
B. Jaffe type

119
Q

Endocrine disturbances

A. McCune-Albright Syndrome
B. Jaffe type

120
Q

> Pathologic fractures with pain and deformity
Teeth: disturbed eruption pattern; jaw expansion

A. McCune-Albright Syndrome
B. Jaffe type

121
Q

Histologic feature: Chinese script-writing appearance

A. McCune-Albright Syndrome
B. Jaffe type

122
Q

Leontiasis ossea

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

123
Q

massive jaw expansion

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

124
Q

ground glass or orange peel appearance

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

125
Q

Localized intramedullary fibrous lesion with curved woven bone Chinese letters (thick material)

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

126
Q

-osteoid material surround by fibrous stroma

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

127
Q

Fibroblasts in woven bone, Oral hyperpigmentation

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

128
Q

cafe-au-lait

A. Monostotic Fibrous Dysplasia
B. Polyostotic Fibrous Dyplasia

129
Q

Treatment of Fibrous Dysplasia:

A

> Surgery: mainly to alleviate functional disturbances
radiation therapy is contraindicated

130
Q

T/ f

CHERUBISm is an autosomal dominant

131
Q

Slow-growing expansion of posterior portion of
maxilla and mandible

132
Q

Radiographic appearance:
soap-bubble appearance

133
Q

Tx: self-limiting ; conservative curettage of lesion with bone recontouring

134
Q

Oral Manifestations:
> Premature loss of primary teeth
> Displacement of follicles of permanent teeth

135
Q

> Ectopic eruption
Permanent teeth may be missing or malformed

136
Q

bilateral expansion of rami

ORAL PATH (15 decks)

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