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Plastic Surgery( Grabb)Ali jasim > Craniofacial Microsomia > Flashcards

Craniofacial Microsomia Flashcards

1
Q

Craniofacial microsomia This is the second most common congenital craniofacial anomaly after cleft lip or palate

A

T

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2
Q

Abnormality of structures arises from the second and third branchial arches

A

F Abnormality of structures arises from the first and second branchial arches

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3
Q

What differents between Obwegeser’s and McCarthy’s methods?

A

the early distraction of the mandible and orthodontic control of the growing maxilla (as per McCarthy’s) versus waiting for skeletal maturity to perform orthognathic surgery for correction of both jaws

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4
Q

In Craniofacial distraction, there will be cartilaginous interphase

A

The only process to create new bone and avoid grafts/flaps;
bone formation takes place without cartilaginous
intermediate

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5
Q

The OAV spectrum includes a variable phenotype and affects a greater range of structures, wich structures?

A

The OAV spectrum includes a variable phenotype and affects a greater range of structures, including the skeletal and soft-tissue components of the face, orbits, and cranium, as well as the axial skeleton and visceral
structures

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6
Q

CFM is defined by which anomaly anomalies?

A

(most commonly epibulbar dermoid or lipodermoids), auricular anomalies ranging from microtia to accessory tragal remnants), and vertebral anomalies.

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7
Q

Pathogenesis of CFM

A

insult due to hemorrhage/ ischemia caused by stapedial artery malformation, or disruption of neural crest cell migration

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8
Q

deficiency of the stapedial artery in irrigating the affected tissues
in the period prior to the formation of the external carotid artery

A

T

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9
Q

more accurate to consider CFM as a subset of OAV

A

T

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10
Q

more accurate to consider CFM as a subset of OAV and CFM

A

T

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11
Q

Thalidomide affects the neural crest cell migration

A

F retinoic acid and diabetes
(i.e., hyperglycemia) with neural crest cell apoptosis in embryogenesis

Thalidomide effect the stapedial artery

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12
Q

stapedial artery, which is a temporary hyoid artery collateral

A

T

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13
Q

How many percent of CM run in families?

A

45% of patients with
OAV dysplasia/Goldenhar syndrome/CFM had some family history
of similar findings but only 6% to 8% of first-degree relatives were
affected

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14
Q

number of chromosomal anomalies have
been loosely tied to CFM

A

T

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15
Q

OAV dysplasia, including which chromosomal defect?

A

OAV dysplasia, including trisomies of chromosomes 7, 9, 18, and 22 exhibit an autosomal dominant pattern of inheritance with variable penetrance

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16
Q

most cases are sporadic

A

T

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17
Q

Some study exhibit an autosomal dominant pattern of inheritance with variable penetrance.

A

T

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18
Q

CFM more commonly tends to affect the left side

A

F CFM more commonly tends to affect the right side
(~60%) and is more frequent in males (up to 65% males)

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19
Q

a rate of bilateral presentation

A

ranging from as low as 5% to
over 30% of cases.

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20
Q

The most obvious finding, CFM

A

Mandibular hypoplasia is usually the most obvious finding, as well as
the most common, present in over 90% of patients

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21
Q

condyle is affected to some degree in every patient with CFM

A

T

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22
Q

degree of condyle and ramus malformation corresponds to changes
in the angel

A

F degree of the condyle and ramus malformation corresponds to changes in the glenoid fossa

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23
Q

The glenoid fossa loses concavity and flattens
and can be malformed frankly in approximately 27% of patients

A

T

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24
Q

pivot point for the hypoplastic ramus or condyle is translated anteriorly when compared with the contralateral TMJ

A

T

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25
Q

In unilateral cases, the chin will deviate to the contralateral side

A

In unilateral cases, the chin will deviate to the affected side

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26
Q

Up to 27% of patients also
present with hypodontia

A

T which correlates in severity to the degree of
mandibular hypoplasia.

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27
Q

Maxilla and Zygoma arise from maxillary process of the second branchial arch

A

F. arise from maxillary process of the first branchial arch

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28
Q

The ramus and
condyle are usually affected much more than the body or ipsilateral
parasymphysis,

A

T

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29
Q

severe
cases showing an absence of the zygomatic arch.

A

T

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30
Q

Consequences of smaller maxilla?

A

the smaller maxilla is associated with a superior displacement of the maxillary sinus and nasal
base,

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31
Q

the abnormal temporal bone only considered one of the cranial manifestation of HFM

A

F the temporal and sometimes frontal bones

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32
Q

plagiocephaly may be seen in up to 12% of
patients.

A

T

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33
Q

noting asymmetry of cranial base landmarks in CFM patients

A

T

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34
Q

the most common site of extracranial bony involvement

A

the most common site of extracranial bony involvement is the axial skeleton, with vertebral or rib anomalies in 8% to
79% of patients. The manifestations of vertebral anomalies are varied
and include hemivertebrae, block vertebrae, scoliosis/kyphoscoliosis,
and spina bifida

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35
Q

the percentage of patients with cranial nerve involvement

A

the nervous system may be involved in CFM in 2% to 69%

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36
Q

Facial nerve paresis
and palsies have been reported, as well as involvement of the vestibulocochlear nerve (CN VIII), but any cranial nerve may be involved.

A

T

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37
Q

Epibulbar dermoids are more frequently associated with Goldenhar
syndrome only

A

F Epibulbar dermoids are more frequently associated with Goldenhar
syndrome but may also be seen in CFM

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38
Q

Developmental disorders percentage

A

in 8% to 73% of patients

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39
Q

The presence of
Brain anomalies is well correlated with ocular findings, and epi-bulbar
dermoid specifically.

A

T

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40
Q

The most common nerve involove in HFM?

A

The cranial nerve most commonly involved is the facial nerve (CN
VII)

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41
Q

soft-tissue structures that
are significantly involved

A

auricular appendage, macrostomia, muscles of mastication, and salivary glands

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42
Q

Percentage of macrostomia

A

Macrostomia can be seen in up to 35% of patients

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43
Q

Clefting of the lip and palate has also been reported and
has been raised as an argument against the hematoma pathogenesis

A

T

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44
Q

Pruzansky
grade I and II mandibular hypoplasia found this relationship to hold
in regard to the temporalis muscle,

A

F Pruzansky
grade I and II mandibular hypoplasia found this relationship to hold
in regard to the masseter, but not the temporalis muscle,

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45
Q

Salivary gland involvement only
hypoplasia or agenesis of the parotid duct or the gland itself

A

T

46
Q

What is the systemic manifestation of CFM

A
  • cardiovascular findings in up to 33% of patients
  • Genitourinary defects are seen in up to 18% of patients
  • More rarely, pulmonary 15% and gastrointestinal 12%.
47
Q

Malformation or hypoplasia of the auricular structures is a sine qua
non for CFM, with all patients presenting with some abnormality

A

T

48
Q

More
severe presentations include all degrees of microtia, anotia, and aural
atresia

A

T

49
Q

Isolated microtia or aural atresia likely does not warrant imaging of the spine or kidneys

A

T. Isolated by microtia or aural atresia
likely does not warrant imaging of the spine or kidneys as the prevalence of abnormalities does not appear increased in this population

50
Q

The middle ear is also often frequently affected, with moderate hypoplasia or atresia seen in up to 90% of patients

A

T

51
Q

hearing loss is sensornueral in the vast majority of cases (73%-86%)
and maybe present on the contralateral side in unilateral CFM cases

A

F hearing loss is conductive in the vast majority of cases (73%-86%)
and may be present on the contralateral side in unilateral CFM cases

52
Q

OMENS classification depends on what ?

A

orbit, mandible, ear, nerve,
and soft tissue

53
Q

Modification Pruzansky classification for mandible hypoplasia depend on what ?

A

subdividing the
type II hypoplastic mandible into type Ila and Ilb based on the status
of the TMJ

54
Q

there is no indication for routine genetic testing of
patients with CFM

A

T Genetic testing should be considered for any patients whose presentation might suggest the presence of another syndrome (e.g., CHARGE or Treacher Collins syndrome)

55
Q

Auditory testing should be routinely performed for all patients
with auricle malformations to assess for hearing loss

A

T

56
Q

Video fluoroscopic swallow studies should
be strongly considered for patients with Pruzansky grade III and II mandible hypoplasia

A

F Video fluoroscopic swallow studies should
be strongly considered for patients with Pruzansky grade III mandible hypoplasia

57
Q

CT imaging has been replaced Cephalograms wich routinely obtained to evaluate the shape and
size of the mandible and maxilla

A

T

58
Q

Routine plain film X-ray evaluation of
the cervical spine is recommended for all patients with CFM

A

T

59
Q

What is the most common complication of surgical site osteotomy?

A

more common complication in SSO (surgical site occurrence) is injury to the inferior alveolar nerve

60
Q

Type Ilb
mandibles are too hypoplastic to carry out distraction

A

T

61
Q

The delay intervention of CFM includes which type of surgery?

A

Le Fort I osteotomy of the maxilla,
BSSO (bilateral sagittal split osteotomy), and asymmetric osseous
genioplasty

62
Q

, self-awareness
of facial differences began at a mean age of 3 years and teasing at a
mean age of 6,

A

t

63
Q

correction of the mandible at
early age and the creation of an open bite on the affected side can
lead to sparing patients from the need for
Le Fort maxillary osteotomies

A

T

64
Q

differences
between the stability of distraction and orthognathic advancement of
the mandible may become apparent in advancements =10 mm

A

F differences
between the stability of distraction and orthognathic advancement of
the mandible may become apparent in advancements >10 mm

65
Q

Severe soft-tissue deficit is best addressed by choosing
distraction for the mandible

A

T

66
Q

Which technique is better to address the soft tissue defect?

A

Adipofascial free flaps have been associated with ptosis and
less predictable volume over time with patient weight loss or
gain. There is currently an increasing trend for serial fat injection to treat facial soft-tissue deficit because this is less likely
to undergo ptosis and patients can be regrafted with much less
morbidity

67
Q

Latency period

A

Approximately 1 week (5-7 days) is allowed for healing and
initial callus formation in the distraction zone prior to initiation of distraction

68
Q

How much is the distraction rate?

A

The distraction hardware is used to separate the bony segments at some predetermined rate (usually 0.5 mm twice per
day)

69
Q

theconsolidation period

A

This period is usually 8 weeks in length
in infants, theconsolidation period is often
shortened to 6 weeks because of rapid bone consolidation.

70
Q

skipping the latency period
and starting distraction immediately appears , does not effect
bone volume and quality at the clinical endpoints

A

T

71
Q

latency periods are often shortened to 1 day or completely
eliminated in infants

A

T

72
Q

Complications of surgery?

A

include hardware problems, soft-tissue ulceration
and breakdown, incorrect distraction vectors, and postdistraction
sequelae.

s decreased growth ofthe distracted bones or loss ofdentition at

73
Q

The central nervous system defects commonly include neural tube
defects, corpus callosum agenesis or hypoplasia, intracranial lipoma,
Arnold-Chiari malformations, hydrocephaly, ventriculomegaly, and
cerebral hypoplasia.

A

T

74
Q

Hypoplasia of the muscle tissues thereby encompassing the muscles of mastication as well as the muscles of facial expression

A

T

75
Q

the presence of a single extracraniofacial anomaly is associated with an increased chance
of others being present,

A

T

76
Q

There is some thought that an isolated accessory tragal remnant may be the only clinical manifestation in a number of patients
with milder forms of OAV dysplasia or CFM.

A

T

77
Q

the severity of craniofacial features does not appear to correlate with the degree of hearing loss,

A

T

78
Q

OMENS calculations

A

Each component is rated Oto 3, with 0
indicating normal phenotype and 3 indicating maximal abnormality,
for a total score ranging from Oto 15.

79
Q

a higher OMENS score and
likelihood of other, noncraniofacial anomalies

A

T

80
Q

Evaluation of the mandible should document interincisal opening and TMJ function.

A

T

81
Q

a modified Pruzansky grade should be
assigned for any child with mandibular hypoplasia

A

T

82
Q

lobular remnant typically inferiorly and anteriorly displaced

A

T

83
Q

C1 Cleft terminates medial to anterior border of masseter

A

T

84
Q

C2 Cleft
terminates lateral to anterior border of masseter

A

T

85
Q

Type Ill PRUZENSKY : The ramus is essentially absent
without any evidence of temporomandibular joint

A

T

86
Q

Up to 13.5% of patients may have swallowing
difficulties

A

T

87
Q

Cephalograms are routinely obtained to evaluate the shape and
size of the mandible and maxilla

A

T

88
Q

the mandibular hypoplasia may lead to tongue retropositioning and tongue-based airway
obstruction, particularly in bilateral cases

A

T

89
Q

Distraction is currently the favored method of advancing the mandible in children,
providing low relapse rates and expansion of the soft-tissue envelope

A

T

90
Q

theinferrior eleveo;ar
nerve is located closer to the buccal cortex in patients with CFM

A

T

91
Q

a one-stage orthognathic correction, it does not address the soft-tissue paucity

A

T

92
Q

Choosing distraction versus osteotomy depend on

A

on the patient’s age,
bone stock, soft tissue, and surgeon preference

93
Q

The mandible that has been distracted or reconstructed with a graft can exhibit growth, albeit at a slower rate than
the contralateral side

A

T

94
Q

Type Ill: The ramus, zygomatic arch, and the temporomandibular articulation is reconstructed using costochondral grafts at
approximately 4 years of age or old

A

T

95
Q

Late childhood/adolescence (6-16 years of age)

A

Orthodontic treatment is applied during this period

96
Q

infants, theconsolidation period is often
shortened to 6 weeks because of rapid bone consolidation

A

T

97
Q

One of the key decisions in mandibular distraction is the choice
of vector

A

T

98
Q

The intracranial volume gained by posterior vault distraction is markedly higher
when compared with fronto-orbital advancement

A

T

99
Q

adequate bony stock in the body with a deficient or absent ramus dictates a vertical vector (perpendicular to the occlusal plane), whereas the converse (short body but adequate ramus) dictates a horizontal
vector

A

T

100
Q

Ifboth the body and ramus are deficient, an oblique vector can
be chosen.

A

T

101
Q

Monobloc advancements incurred higher complication rates with distraction, and the opposite was seen in Le Fort III advancements

A

T

102
Q

the complication rate appears to be comparable between Distraction of the cranial vault, and the posterior vault

A

T

103
Q

How many phases for craniofacial osteogenesis?

A

There are three phases-latency (4-5 days), distraction
( 4-6 weeks, or until the desired amount of advancement is
obtained), and consolidation (6-8 weeks).

104
Q

isolated microtia may serve as a useful marker for what?

A

isolated microtia may serve as a useful marker to trigger
screening in patients for other manifestations of OAV dysplasia, as it has been shown to predict later facial growth asymmetry in up to 40% of screened patients.

105
Q

OMENS+ indicate what?

A

include extracraniofacial abnormalities

106
Q

More
severe presentations include all degrees of microtia, anotia, and aural
atresia.

A

t

107
Q

The morphology and functional status of the
TMJ are key to grading and treatment planning in a patient with
CFM. Without clear information regarding the status of the
TMJ, no definitive grade can be assigned.

A

T

108
Q

Cardiac, renal, and cervical spine evaluation is
recommended for all CFM patients

A

T

109
Q

Type I mandible hypoplasia is better addressed with distraction to produce a lower relapse rate and
may also require revision in adulthood.

A

T

110
Q

Type Ilb may be treated
with either distraction and creating of a pseudo-TMJ or a costochondral graft to create a neo

A

T

Plastic Surgery( Grabb)Ali jasim (92 decks)

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