Craniofacial Microsomia

Question 1

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

Answer 1

T

Question 2

Abnormality of structures arises from the second and third branchial arches

Answer 2

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

Question 3

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

Answer 3

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

Question 4

In Craniofacial distraction, there will be cartilaginous interphase

Answer 4

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

Question 5

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

Answer 5

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

Question 6

CFM is defined by which anomaly anomalies?

Answer 6

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

Question 7

Pathogenesis of CFM

Answer 7

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

Question 8

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

Answer 8

T

Question 9

more accurate to consider CFM as a subset of OAV

Answer 9

T

Question 10

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

Answer 10

T

Question 11

Thalidomide affects the neural crest cell migration

Answer 11

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

Thalidomide effect the stapedial artery

Question 12

stapedial artery, which is a temporary hyoid artery collateral

Answer 12

T

Question 13

How many percent of CM run in families?

Answer 13

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

Question 14

number of chromosomal anomalies have
been loosely tied to CFM

Answer 14

T

Question 15

OAV dysplasia, including which chromosomal defect?

Answer 15

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

Question 16

most cases are sporadic

Answer 16

T

Question 17

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

Answer 17

T

Question 18

CFM more commonly tends to affect the left side

Answer 18

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

Question 19

a rate of bilateral presentation

Answer 19

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

Question 20

The most obvious finding, CFM

Answer 20

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

Question 21

condyle is affected to some degree in every patient with CFM

Answer 21

T

Question 22

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

Answer 22

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

Question 23

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

Answer 23

T

Question 24

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

Answer 24

T

Question 25

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

Answer 25

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

Question 26

Up to 27% of patients also present with hypodontia

Answer 26

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

Question 27

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

Answer 27

F. arise from maxillary process of the first branchial arch

Question 28

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

Answer 28

T

Question 29

severe cases showing an absence of the zygomatic arch.

Answer 29

T

Question 30

Consequences of smaller maxilla?

Answer 30

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

Question 31

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

Answer 31

F the temporal and sometimes frontal bones

Question 32

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

Answer 32

T

Question 33

noting asymmetry of cranial base landmarks in CFM patients

Answer 33

T

Question 34

the most common site of extracranial bony involvement

Answer 34

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

Question 35

the percentage of patients with cranial nerve involvement

Answer 35

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

Question 36

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.

Answer 36

T

Question 37

Epibulbar dermoids are more frequently associated with Goldenhar syndrome only

Answer 37

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

Question 38

Developmental disorders percentage

Answer 38

in 8% to 73% of patients

Question 39

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

Answer 39

T

Question 40

The most common nerve involove in HFM?

Answer 40

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

Question 41

soft-tissue structures that are significantly involved

Answer 41

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

Question 42

Percentage of macrostomia

Answer 42

Macrostomia can be seen in up to 35% of patients

Question 43

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

Answer 43

T

Question 44

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

Answer 44

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

Question 45

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

Answer 45

T

Question 46

What is the systemic manifestation of CFM

Answer 46

- 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%.

Question 47

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

Answer 47

T

Question 48

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

Answer 48

T

Question 49

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

Answer 49

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

Question 50

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

Answer 50

T

Question 51

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

Answer 51

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

Question 52

OMENS classification depends on what ?

Answer 52

orbit, mandible, ear, nerve, and soft tissue

Question 53

Modification Pruzansky classification for mandible hypoplasia depend on what ?

Answer 53

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

Question 54

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

Answer 54

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)

Question 55

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

Answer 55

T

Question 56

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

Answer 56

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

Question 57

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

Answer 57

T

Question 58

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

Answer 58

T

Question 59

What is the most common complication of surgical site osteotomy?

Answer 59

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

Question 60

Type Ilb mandibles are too hypoplastic to carry out distraction

Answer 60

T

Question 61

The delay intervention of CFM includes which type of surgery?

Answer 61

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

Question 62

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

Answer 62

t

Question 63

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

Answer 63

T

Question 64

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

Answer 64

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

Question 65

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

Answer 65

T

Question 66

Which technique is better to address the soft tissue defect?

Answer 66

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

Question 67

Latency period

Answer 67

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

Question 68

How much is the distraction rate?

Answer 68

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

Question 69

theconsolidation period

Answer 69

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

Question 70

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

Answer 70

T

Question 71

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

Answer 71

T

Question 72

Complications of surgery?

Answer 72

include hardware problems, soft-tissue ulceration and breakdown, incorrect distraction vectors, and postdistraction sequelae. s decreased growth ofthe distracted bones or loss ofdentition at

Question 73

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.

Answer 73

T

Question 74

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

Answer 74

T

Question 75

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

Answer 75

T

Question 76

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.

Answer 76

T

Question 77

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

Answer 77

T

Question 78

OMENS calculations

Answer 78

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

Question 79

a higher OMENS score and likelihood of other, noncraniofacial anomalies

Answer 79

T

Question 80

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

Answer 80

T

Question 81

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

Answer 81

T

Question 82

lobular remnant typically inferiorly and anteriorly displaced

Answer 82

T

Question 83

C1 Cleft terminates medial to anterior border of masseter

Answer 83

T

Question 84

C2 Cleft terminates lateral to anterior border of masseter

Answer 84

T

Question 85

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

Answer 85

T

Question 86

Up to 13.5% of patients may have swallowing difficulties

Answer 86

T

Question 87

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

Answer 87

T

Question 88

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

Answer 88

T

Question 89

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

Answer 89

T

Question 90

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

Answer 90

T

Question 91

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

Answer 91

T

Question 92

Choosing distraction versus osteotomy depend on

Answer 92

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

Question 93

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

Answer 93

T

Question 94

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

Answer 94

T

Question 95

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

Answer 95

Orthodontic treatment is applied during this period

Question 96

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

Answer 96

T

Question 97

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

Answer 97

T

Question 98

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

Answer 98

T

Question 99

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

Answer 99

T

Question 100

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

Answer 100

T

Question 101

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

Answer 101

T

Question 102

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

Answer 102

T

Question 103

How many phases for craniofacial osteogenesis?

Answer 103

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).

Question 104

isolated microtia may serve as a useful marker for what?

Answer 104

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.

Question 105

OMENS+ indicate what?

Answer 105

include extracraniofacial abnormalities

Question 106

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

Answer 106

t

Question 107

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.

Answer 107

T

Question 108

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

Answer 108

T

Question 109

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

Answer 109

T

Question 110

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

Answer 110

T