OMSITE OMSITE - STUDY

OMSITE (OMSITE - STUDY

DATA) 551 QUESTIONS WITH ACTUAL

SOLUTIONS!!

• 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 1 / 4

What is the spectrum for Branchial Arch syndromes? Answer - OAV- orbital- auriculo-vertebral spectrum

• 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 2 / 4

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 3 / 4

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

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