Craniofacial Microsomia Flashcards

1
Q

the second most congenital anomaly present after C/P?

A

Craniofacial Microsomia

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2
Q

Craniofacial macrosomia arise from which bronchial arch?

A

arises from the first and second
branchial arches,

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3
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).

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4
Q

Early craniofacial distraction can’t do for children? true?

A

The early distraction of midface (Le Fort I or III osteotomies)
can be considered if maxillary and malar asymmetry
is significant before skeletal maturity.

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5
Q

What is oculoauriculovertebral dysplasia?

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

Can consider CFM as a subset of OAV
dysplasia?

A

yes with a presentation limited to disorders of tissues stemming from the first and second branchial arches, most commonly thought of as involving the ear and the mandible

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7
Q

what is Goldenhar
syndrome?

A

considered as a subset of both OAV spectrum and
CFM, is defined by ocular anomalies (most commonly epibulbar dermoids or lipodermoids), auricular anomalies (ranging from microtia to accessory tragal remnants), and vertebral anomalies

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8
Q

deficiency of the stapedial artery in irrigating the affected tissues in the period after the formation of the external carotid artery true? or false?

A

False , deficiency of the stapedial artery in irrigating the affected tissues in the period Before the formation of the external carotid artery

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9
Q

percentage of bilateral HM ?

A

5-30%

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10
Q

percetage of unilateral HM ?

A

60% RIGHT SIDE 65%MALE

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11
Q

The most common affected part of the mandible in CAM?

A

90% involvement of the mandible and most common site condyle followed by ramus

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12
Q

in case of shrinkage in the size of the maxilla, is the position of the maxillary sinus affected?or not ?

A

maxillary sinus displaced superiorly

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13
Q

Importantly, the most common site of extracranial bony involvement is ……..

A

the axial skeleton with vertebral or rib anomalies in 8% to
79% of patients

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14
Q

Epibulbar dermoids are more frequently associated with

A

Goldenhar syndrome but may also be seen in CFM.

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15
Q

how you can identify the brian involvement in CFM?

A

Developmental disorders including intellectual
disability, language or speech developmental delay, and neuropsychomotor delay have been seen in 8% to 73% of patients. The presence of brain anomalies is well correlated with ocular findings, and epibulbar dermoids specifically.

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16
Q

The cranial nerve most commonly involved in CFM ?

A

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

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17
Q

Percentage of macrosomia in CFM?

A

Macrostomia can be seen in up to 35% of patients, with wide variations in severity.

18
Q

cardiovascular findings in up to ………..of patients

A

33%

19
Q

Genitourinary defects are seen in up to ……. of patients

A

18%

20
Q

More rarely, pulmonary
and gastrointestinal anomalies may be encountered in up to
…………………. of patients,

A

15% and 12%

21
Q

What is the type of hearing loss in microtia?

A

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

22
Q

Does the severity of hearing loss correlate with severity of CFM?

A

Notably, the severity of craniofacial features does not appear to correlate with the degree of hearing loss, so all patients suspected to be at risk should undergo formal audiologic evaluation.

23
Q

the structure reflects the degree of hypoplasia of the ramus

A

The size of the condyle

24
Q

In addition to being short, the ramus is usually displaced toward ????

A

In addition to
being short, the ramus is usually displaced toward the midline

25
Q

the condyle affected to some degree in every patient with CFM True or False?

A

True

26
Q

Percentage of patients present with hypodontia in CFM?

A

Up to 27% of patients also present with hypodontia, which correlates in severity to the degree of
mandibular hypoplasia.

27
Q

What the draw back of hypopalsia of the maxilla in CFM?

A

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

28
Q

Percentage of plagiocephaly in CFM?

A

12%

29
Q

The gleniod fossa flattened in …….. of HFM

A

27%

30
Q

How much the percentage of the patients that hav mandibular hypoplasia in CFM?

A

More than 90%

31
Q

The degree of condyle and ramus malformation corresponds to what structure?

A

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

32
Q

what the most common site for extracranial
bony involovment in CFM?

A

The axial skeleton with vertebrae or rib anamoly

33
Q

The presence of brain anomaly is well correlated with ?

A

Ocular finding and peribulbar dermoid specifically

34
Q

the most common nerve involved in CFM IS?

A

VII Facial

35
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.

36
Q

Isolated microtia or aural atresia
likely does not warrant imaging of the spine or kidneys, true or false?

A

Isolated 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

37
Q

OMENS+ indicate what?

A

include extracraniofacial abnormalities

38
Q

when should use the modified Pruzansky classification?

A

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

39
Q

there is no indication for routine genetic testing of
patients with CFM true or false?

A

true

40
Q

M2B of OMENS Grading system means what?

A

M2B Abnormally shaped and short ram us (Glenoid fossa is
Inferiorly, medially and anteriorly displaced with the hypoplastic condyle

41
Q

One of the more common complications in SSO(sagittal split osteotomy) (surgical
site occurrence) is ,,,,,,,,,,

A

injury to the inferior alveolar nerve