DATA) 551 QUESTIONS WITH ACTUAL
SOLUTIONS!!
2 variations of nasolabial flaps Answer - Island Flap with a subq tissue pedicle
Peninsular flap with a cutaneous pedicle
Hallmarks of Treacher Collins abnormalities Answer - Maxillary/mandibular
hypoplasia
Cleft lip/palate
Choanal atresia
Microtia and associated condyctive hearing loss
Hypoplasia of lower eyelids/lateral canthus
orbital hypoplasia and antimongoloid palpebral fissures
Biggest concern for a baby born with Treacher collins syndrome? Answer -
Airway compromise
- choanal atresia and severe retrognathia
secure airway with positioning all the way to tracheostomy
At what ages do you do recons for Treacher Collins patients
- what 3 major things are reconstructed? Answer - >7 years for
orbitozygomatic complex (maturation at 8)
>7 years for Ear
Skeletal maturity for orthognathics
,What is the spectrum for Branchial Arch syndromes? Answer - OAV- orbital-
auriculo-vertebral spectrum
2 major classifications for Branchial Arch Syndromes
- what do the 2 classifications represent? Answer - OMENS- comprehensive
Kaban/Pruzanksy- mandibular only
What is the OMENS classification for? what does it stand for? Answer -
OMENS: Comprehensive system for Branchial arch syndromes
- Orbital distortion
- Mandibular hypoplasia
- Ear anomalies
- Nerve involvement
- Soft tissue deficiency
What arches fail to develop resulting in Cleft lip/palate? Answer - Branchial
arch 1/2 leading to failure of neuroectodermal fusion
What is the most common craniofacial defect? whats the second? Answer -
Cleft lip/palate
Hemifacial microsomia
What is another term for hemifacial microsomia? Answer - Unilateral
otomandibular dysostosis or lateral facial dysplasia
How is Hemifacial Microsomia (HFM) and Goldenhar syndrome related?
Answer - HFM and Goldenhar syndrome are considered varients under the
,same clinical continuum termed OAV's (orbit-auricular-vertebral). Goldenhar
has HFM as well as vertebral and epibulbar dermoid anomalies
1st and 2nd branchial arches are formed by what cells? Answer - Neural Crest
cells (NCC)
First branchial arch syndrome affect what anatomy? Answer - maxilla,
mandible, zygoma, muscles of mastication, trigeminal nerve, anterior auricle
(tragus,helix) , malleolus, incus
Second branchial arch syndromes affect what anatomY? Answer - hyoid bone,
facial expression muscles, stapes, antihelix/antitragus/ lobule
Etiology of Hemifacial Microsomia? (HFM) Answer - Vascular injury to the
stapedial artery and anomalous migration of neural crest cells
What artery supersedes the external carotid artery? Answer - Stapedial artery
Impaired VEGF can damage blood supply to Meckels cartialge leading to what?
Answer - mandibular hypoplasia and deformation of malleus/incus
OTX2 gene deletion can cause what? Answer - Mandibular dysostosis
Minimal diagnostic criteria for HFM?
What's the gold standard image to assess facial asymmetry Answer - Ipsilateral
mandible/auricular defects
, asymmetric mandible OR auricule defects with involvement of 2+ indirectly
associated anomalies
Family history of hemifacial microsomia
PA CEPH
Kaban Classification Answer - Hemifacial Microsomia
- Grade 1: hypoplasia of mandible
- Grade 2a: short ramus/normal glenoid fossa
- Grade 2b: malpositioned glenoid fossa requiring recon
- Grade 3: gross distortion or agenesis of ramus
Name one syndrome that causes craniofacial microsomia Answer - Goldenhar
syndrome
Symptoms of Goldenhar syndrome include Answer - Facial asymmetry
MICROSTOMIA/MICROGNATHIA, short neck
Tetrology of fallot/ VSD's
spinal abnormalities
coloboma
benign growths of eyes (dermoid cysts)
microtia or anotia and associated hearing loss
pre-auricular tags
Cleft lip/palate
Causes of Goldenhar syndrome Answer - Most are sporadic