Craniofacial Microsomia Flashcards
the second most congenital anomaly present after C/P?
Craniofacial Microsomia
Craniofacial macrosomia arise from which bronchial arch?
arises from the first and second
branchial arches,
How many phases for craniofacial osteogenesis?
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).
Early craniofacial distraction can’t do for children? true?
The early distraction of midface (Le Fort I or III osteotomies)
can be considered if maxillary and malar asymmetry
is significant before skeletal maturity.
What is oculoauriculovertebral dysplasia?
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.
Can consider CFM as a subset of OAV
dysplasia?
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
what is Goldenhar
syndrome?
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
deficiency of the stapedial artery in irrigating the affected tissues in the period after the formation of the external carotid artery true? or false?
False , deficiency of the stapedial artery in irrigating the affected tissues in the period Before the formation of the external carotid artery
percentage of bilateral HM ?
5-30%
percetage of unilateral HM ?
60% RIGHT SIDE 65%MALE
The most common affected part of the mandible in CAM?
90% involvement of the mandible and most common site condyle followed by ramus
in case of shrinkage in the size of the maxilla, is the position of the maxillary sinus affected?or not ?
maxillary sinus displaced superiorly
Importantly, the most common site of extracranial bony involvement is ……..
the axial skeleton with vertebral or rib anomalies in 8% to
79% of patients
Epibulbar dermoids are more frequently associated with
Goldenhar syndrome but may also be seen in CFM.
how you can identify the brian involvement in CFM?
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.
The cranial nerve most commonly involved in CFM ?
The cranial nerve most commonly involved is the facial nerve ( CN VII)
Percentage of macrosomia in CFM?
Macrostomia can be seen in up to 35% of patients, with wide variations in severity.
cardiovascular findings in up to ………..of patients
33%
Genitourinary defects are seen in up to ……. of patients
18%
More rarely, pulmonary
and gastrointestinal anomalies may be encountered in up to
…………………. of patients,
15% and 12%
What is the type of hearing loss in microtia?
hearing loss is conductive in the vast majority of cases (73%-86%) and may be present on the contralateral side in unilateral CFM cases.
Does the severity of hearing loss correlate with severity of CFM?
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.
the structure reflects the degree of hypoplasia of the ramus
The size of the condyle
In addition to being short, the ramus is usually displaced toward ????
In addition to
being short, the ramus is usually displaced toward the midline
the condyle affected to some degree in every patient with CFM True or False?
True
Percentage of patients present with hypodontia in CFM?
Up to 27% of patients also present with hypodontia, which correlates in severity to the degree of
mandibular hypoplasia.
What the draw back of hypopalsia of the maxilla in CFM?
The smaller maxilla is associated
with a superior displacement of the maxillary sinus and nasal
base.
Percentage of plagiocephaly in CFM?
12%
The gleniod fossa flattened in …….. of HFM
27%
How much the percentage of the patients that hav mandibular hypoplasia in CFM?
More than 90%
The degree of condyle and ramus malformation corresponds to what structure?
. The degree of condyle and ramus malformation corresponds to changes in the glenoid fossa.
what the most common site for extracranial
bony involovment in CFM?
The axial skeleton with vertebrae or rib anamoly
The presence of brain anomaly is well correlated with ?
Ocular finding and peribulbar dermoid specifically
the most common nerve involved in CFM IS?
VII Facial
isolated microtia may serve as a useful marker for what?
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.
Isolated microtia or aural atresia
likely does not warrant imaging of the spine or kidneys, true or false?
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
OMENS+ indicate what?
include extracraniofacial abnormalities
when should use the modified Pruzansky classification?
modified Pruzansky grade should be
assigned for any child with mandibular hypoplasia
there is no indication for routine genetic testing of
patients with CFM true or false?
true
M2B of OMENS Grading system means what?
M2B Abnormally shaped and short ram us (Glenoid fossa is
Inferiorly, medially and anteriorly displaced with the hypoplastic condyle
One of the more common complications in SSO(sagittal split osteotomy) (surgical
site occurrence) is ,,,,,,,,,,
injury to the inferior alveolar nerve
