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
