Developmental Anatomy/Disturbances/Disorders 26% 17Q Flashcards

1
Q

which fontanelle joins frontal and parietal bones

A

Anterior (Bregma, Coronal suture)

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2
Q

which fontanelle joins the 2 parietal and occipital bones

A

Posterior (Lambdoid)

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3
Q

in dolicocephalic patients, which suture is likely to have closed early?

A

Sagittal

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4
Q

in brachycephalic patients, which suture is likely to have closed early?

A

coronal suture

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5
Q

what is Scaphocephaly?

A

fusion of the saggital suture

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6
Q

the upper lip is formed by fusion of __________________ process and ________________________

A

medial nasal

maxillary protuberance

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7
Q

the fusion of medial nasal process and maxillary protuberance happens by week ___

A

5

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8
Q

the frontonasal process gives rise to two ____________ processes and two ____________ processes

A

median nasal

lateral nasal

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9
Q

what gives rise to the bridge of the nose, septum of nose, premaxilla, and philtrum

A

median nasal prominences

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10
Q

what gives rise to the ala of the nose

A

lateral nasal processes

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11
Q

The primary palate is formed by the ________ week from the 2 medial nasal processes and separates the developing oral and nasal tissues.

As the oronasal cavity expands, the secondary palate is formed between the ___th & ___th week by the two ___________ processes.

A

6th

6th and 8th

palatal

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12
Q

the palatal processes are part of the ____________ process.

A

maxillary

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13
Q

The palate closes by week ___ but generally happens by week 7.5-8, marking the end of the __________ period.

A

12

embryonic period

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14
Q

After the palatal processes have fused they continue to grow further __________, while merging in the midline to form the ________________ and ___________. This process is completed by 12 weeks.

A

posteriorly

soft palate and uvula

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15
Q

CLP pts have reduced __________ facial height

A

upper

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16
Q

Alveolar grafting should be performed when 2/3 of the __________ root is formed.

A

canine

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17
Q

Infants with repaired CLP have deficiency of soft palate and likely _________________ insufficiency

A

velopharyngeal

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18
Q

Pt with CLP are best retained with retainer and a ____________

A

pontic

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19
Q

medical term for soft palate

A

velum

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20
Q

_______________ occurs when speech is affected by an inability to build up intraoral pressure due to communication between the oral and nasal cavity

A

hypernasality

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21
Q

the incidence of CLP in whites is 1/___

A

1/800

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22
Q

CLP is most common in which population? Rank these:

white, black, indian, asian

A

Indian > Asian > White > Black

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23
Q

The probability of CLP when 1 sibling or kin is affected is ___%

A

4%

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24
Q

The probability of CLP when no sibling but 1 kin is affected is ___%

A

4%

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25
Q

The probability of CLP when no sibling but 1 parent is affected is ___%

A

4%

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26
Q

The probability of CLP when 2 siblings are affected is ___%

A

9%

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27
Q

The probability of CLP when 1 sibling and 1 parent are affected is ___%

A

17%

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28
Q

the rule for repair of cleft lip is that it happens:

A

10 weeks, 10 lbs, 10 Hg

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29
Q

cleft lip happens due to failure of fusion of the ________________ and ______________ prominence

A

median nasal & maxillary prominence

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30
Q

Which is NOT a finding associated with cleft palate:

  1. Rotated incisors
  2. Missing laterals
  3. Ectopic eruption
  4. Supernumeraries
  5. Impacted centrals
  6. Posteriorx-bite
A
  1. Impacted centrals
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31
Q

the palate is formed by week ___ in utero

A

6

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32
Q

repair of cleft palate happens at ___ - ___ months

A

10 - 18 mo

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33
Q

cleft palate happens due to failure of fusion of the ___________ processes (outgrowth of the maxillary processes)

A

palatine

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34
Q

in cleft palate repair, tissue scarring leads to ____________ growth

A

decreased

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35
Q

cleft lip and palate occurs at week ___ - ___

A

6 - 8

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36
Q

what is more common? cleft lip or cleft palate?

A

cleft lip

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37
Q

Cervical clefts and diverticula are formed by failure of fusion of brachial ____________

A

arches

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38
Q

the tongue forms in utero in week ___. The anterior forms from the ___ pharyngeal arch and the posterior portion forms fro the ___ and ___ pharyngeal arch.

A

4th week

anterior 1st

posterior 3rd and 4th

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39
Q

Hypotelorism occurs due to __________ clefts;

hypertelorism occurs due to _________ clefts.

A

oro-ocular clefts

midline clefts

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40
Q

Crouzon’s syndrome is associated with what three distinct features?

A

CrOU

  1. Craniosynostosis: coronal or lambdoid
  2. Ocular proptosis: shallow orbits. Crossed (strabismus) and/or wide set eyes
  3. Underdeveloped midface: receded cheekbones or exopthalmus (bulging eyes)
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41
Q

Patients with Crouzon’s syndrome are subject to visual disturbances; doing a __________ can prevent blindness.

A

LeFort II (online sources say LeFort III)

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42
Q

In addition to the 3 major features, patients with Crouzon’s syndrome could be subject to cleft ____________.

A

palate

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43
Q

In patients with Apert’s syndrome, they present with peaked heads and unusual facial appearance. This is due to the associated __________________ where ALL sutures fuse prematurely.

A

craniosynostosis

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44
Q

Is Crouzon’s syndrome autosomal Domminant?

A

yes

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45
Q

Apert’s syndrome is autosomal _________

A

dominant

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46
Q

Which syndrome is due to a mutation of fibroblast growth factor receptor 2.

A

Apert’s syndrome

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47
Q

The most recognizable clinical appearance of Apert’s syndrome is:

A

syndactyly - usually the 2nd, 3rd or 4th fingers

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48
Q

In a child with Apert’s syndrome, as growth occurs, the bones in the __________ and ________ become progressively fused, reducing flexibility and function

A

hands and feet

49
Q

Apert’s syndrome is associated with buying eyes due to a hypoplastic:

A

orbital rim

50
Q

How does Apert’s syndrome affect intellectual development?

A

The clild may be mentally challenged to varying degrees

51
Q

a patient with Apert’s syndrome will have cleft __________, ________ stature, and hearing loss/frequent ear infections

A

palate, short stature

52
Q

Treatment for Apert’s syndrome include surgery to correct. the skull, especially concerning the _________ suture, mid face and palate. They must also see a _____________ specialist.

A

coronal

hearing

53
Q

Treacher collins syndrome is autosomal ___________-

A

dominant

54
Q

Symptoms of Treacher collins syndrome include:

  1. Abnormal to almost completely absent low set ________________
  2. Abnormal hearing canal with ______________________
  3. Very small _________ but very large _______________
  4. Defect in lower _______________, coloboma that extends to lower lid, decreased _______________
  5. Scalp hair extends onto _________
  6. Cleft ____________
  7. Flat cheekbones and facial clefts
    8.
A
  1. external ears
  2. conductive hearing loss
  3. small jaw, large mouth
  4. eyelid, eyelashes
  5. cheeks
  6. palate
  7. cheekbones
55
Q

How does Treacher Collins Syndrome affect intelligence?

A

it does not - normal intelligence

56
Q

Treacher Collins Syndrome diagnosis with genetic testing for mutations on the __________ gene

A
  • treacle
57
Q

Treatment for Treacher Collins Syndrome includes correcting _____________ and plastic surgery to affect receding chin and other defects

A

hearing loss

58
Q

Cleft lip and palate can cause feeding ____________, __________ development problems, ear ____________

A

difficulties

speech

infections

59
Q

The incidence of cleft palate alone is 1 in _____

A

2500

60
Q

For cleft patients, lip closure happens at ___ weeks and palate closure happens at ___ year

A

week 10

1 year

61
Q

Prenatal __________ consumption decreases the chance of cleft lip/palate by 33%

A

folate

62
Q

Which syndrome occurs during development of the oral cavity when the chin does not completely come away from the baby’s chest as it should

A

Pierre Robin Sequence

63
Q

Pierre Robin Sequence results in a combination of characteristics including a Small, pushed back _________, large tongue w/ _____________, and a wide, U shaped ____________________

A

mandible

glossoptosis

cleft of the hard and soft palate

64
Q

Sometimes, in patients with ____________________, the problem also affects the growth of the trachea and soft tissues of the pharynx, and the baby’s airway can be severely decreased in size or development. Because of the small jaw and large tongue, the airway may become obstructed. Some of these children require a trach or other surgical interventions to increase the airway size.

A

Pierre Robin Sequence

65
Q

Cyanosis is common at birth in babies with __________________ syndrome

A

Pierre Robin Sequence/Syndrome

66
Q

Gardner’s Syndrome is autosomal _____________

A

dominant

67
Q

Which syndrome is caused by a mutation in the APC gene?

A

Gardner’s Syndrome

68
Q

In patients with Gardner’s Syndrome, you can find multiple _________ throughout the intestinal tract, extracolonic tumors including osteomas of the skull, thyroid cartilage, epidermoid cysts and fibromas.

A

polyps

69
Q

Patients with which syndrome have a higher risk of developing desmoid tumors, abnormalities of the retina, skin tumors, and bone tumors

A

Gardner’s Syndrome

70
Q

Is Gardner’s Syndrome associated with facial clefts?

A

no

71
Q

Gardner’s Syndrome is associated with _____________ teeth and _____________ radiographic appearance of the jaw

A

supernumerary

cotton wool

72
Q

Marfan’s Syndrome is autosomal ___________

A

dominant

73
Q

Which syndrome is due to a mutation in the FBNI (fibrillin I) gene?

A

Marfan’s Syndrome

74
Q

Name the syndrome associated with a disorder of the connective tissue – the tissue that strengthens the body’s structures. Disorders of CT affect the skeletal, CV, eyes and skin.

A

Marfan’s Syndrome

75
Q

Disorders of CT, such as ________________ affect the skeletal, CV, eyes and skin.

A

Marfan’s syndrome

76
Q

In patients with Marfan’s syndrome 30% have no family hx and are“sporadic”cases–believed to result from ____________

A

spontaneous new mutation

77
Q

Which syndrome is associated with disruption in changes in elastic tissues, particularly the aorta, eye, and skin, as well as overgrowth of the long bones of the body, resulting in tall stature and long limbs.

A

Marfan’s Syndrome

78
Q

Patients with Marfan’s syndrome present with long limbs and spiderlike fingers (arachnodactyly), chest abnormalities (pectus excavatum/carinatum), curvature of the spine, and a particular set of facial features including a _________ palate and ____________ teeth.

A

high, arched

crowded

79
Q

Patients with ____________ syndrome often have _____________ abnormalities including enlargement (dilation) of the base of the aorta (aortic root), with aortic regurgitation, and mitral valve prolapse and may develop a dissecting aortic aneurysm.

A

Marfan’s

cardiovascular

80
Q

Name some characteristics of Marfan’s Syndrome:

A

see list

81
Q

How does a patient’s diagnosis of Marfan’s syndrome affects dental care?

A

Preventive antibiotics before dental procedures to prevent endocarditis

82
Q

Cleidocranial dysostosis is autosomal _____________

A

dominant

83
Q

Cleidocranial dysostosis is characterized by absent or incompletely formed ____________ bones, abnormal shape of skull with depression of the ____________ suture, characteristic facial appearance, short stature, and dental abnormalities

A

collar

sagittal

84
Q

Which syndrome is caused by the mutation of RUNX2 gene?

A

Cleidocranial dysostosis

85
Q

Cleidocranial dysostosis is characterized by a heavy protruding brow, protruding jaw and wide nasal bridge. Dentally, it is characterized by ____________ eruption and _______________ teeth and peg or abnormal shape.

A

Delayed

supernumerary

86
Q

Which condition is characterized by abnormal development of the skin, hair, nails, teeth, and sweat glands?

A

Ectodermal dysplasia

87
Q

Ectodermal dysplasia is linked to the ___ chromosome and usually affects ( women / men )

A

X

men

88
Q

Due to the lack of _____________ development in children with Ectodermal dysplasia, children may have difficulty controlling fevers. Mild illness may produce extremely high fevers.

A

sweat glands

89
Q

Adults with ________________ are unable to tolerate warm environments and need special measures to maintain body temperature

A

ectodermal dysplasia

90
Q

In patients with ectodermal dysplasia, the skin is thin with light coloring, the hair is _________, and the teeth develop abnormally and many are missing.

A

absent or very thin

91
Q

What is theTx for patients with ectodermal dysplasia?

A

No specific Tx

92
Q

Down’s syndrome is a chromosomal abnormality, usually an extra copy of the ___ chromosome. Usually but not always results in mental retardation and other conditions.

A

21st

93
Q

The prominent facial features associated with Down Syndrome include

  1. ___________ nose
  2. ______________ tongue
  3. ___________ slanting eyes.
  4. Inner corner of eye may have _________ fold of skin (epicanthal fold) rather than coming to a point.
A
  1. flattened
  2. protruding
  3. upward
  4. rounded
94
Q

Patients with _____________________ and Hands short and broad hands with short fingers. They often have a single palm crease (simian crease). Retardation of normal growth and development is typical and most never reach average adult height.

A

Down’s syndrome

95
Q

For patients with Down’s syndrome, early mortality is frequently caused by:

A

congenital heart defects

96
Q

The incidence of Down’s syndrome in the US is 1 in ___

A

800

97
Q

Patients with Down’s syndrome commonly have esophageal atresia and duodenal atresia(obstruction). They also have a higher incidence of ALL:

A

acute lymphatic leukemia

98
Q

Dentally, Down’s syndrome patients present with:

A

delayed eruption

99
Q

Osteogenesis imperfecta is autosomal __________

A

dominant

100
Q

Which condition is associated with congenital abnormal fragility of bones? “Brittle bone disease?”

A

Osteogenesis imperfecta

101
Q

All 4 types of osteogenesis imperfect are caused by defects in the amount or structure of ___________

A

Type I collagen

102
Q

Fragile bones, early hearing loss and blue sclera are the 3 classic signs of:

A

osteogenesis imperfecta

103
Q

peutz jeghers syndrome is autosomal _____________

A

dominant

104
Q

Which condition is characterized by intestinal polyps and pigmented spots around lips, gums, mucous membranes and skin?

A

Peutz-Jeghers Syndrome

105
Q

Which condition is characterized by an increased production of cortisol caused by excessive production of ACTH by the pituitary gland?

A

Cushing’s Syndrome

106
Q

Adrenal and pituitary tumors, long term tx with corticosteroids, and female gender can put you at risk for which condition?

A

Cushing’s syndrome

107
Q

Symptoms of Cushing’s syndrome include;

  1. __________ face: round, red, full
  2. __________ hump: fat btwn shoulders
  3. Central __________ with protruding abdomen and thin extremities
  4. Unintentional weight gain
A
  1. Moon
  2. buffalo
  3. obesity
108
Q

Hemifacial microsomia occurs ( unilaterally / bilaterally / both )?

A

both

109
Q

Hemifacial microsomia affects which anatomical structures?

A

ear, mouth, mandible

110
Q

What facial birth defect is second most common after clefts?

A

hemifacial microsomia

111
Q

hemifacial microsomia incidence is 1 in ______ - ______

A

3500 - 4500

112
Q

hemifacial microsomia is caused by a ____________ problem

A

vascular

→ leads to clotting and poor blood supply to face

113
Q

Patients with hemifacial microsomia may have _________ and cervical spine problems

A

kidney

114
Q

geographic tongue is more common in ( females / males )

A

females

115
Q

what is the most common salivary tumor?

A

pleiomorphic adenoma

116
Q

If Autosomal Dominant and non-carrier have a child: ___% the children will be affected

A

50%

117
Q

Which gene is responsible for craniosynostoses syndromes: Crouzons, Apert, Pfeiffer, Jackson- Weiss?

A

FGFR

118
Q

Wormian bones (abnormal ossicles that develop from extra ossification centers within the cranium) are present in patients with _____________, osteogenesis imperfect and down syndrome

A

cleidocranial dysplasia