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CRANIOFACIAL SYNDROMES QUESTIONS WITH CORRECT ANSWERS

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CRANIOFACIAL SYNDROMES QUESTIONS WITH CORRECT ANSWERS

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Which craniosynostotic patterns are most commonly associated with Apert
Syndrome?


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Bilateral coronal sutures synostosis resulting in a turribrachycephalic
deformity or a high, flat forehead with a short anterior cranial fossa

,What disease process has associated with the coup de sabre deformity?


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Parry Romberg syndrome (or progressive hemifacial atrophy)




What are the commonly encountered diagnoses associated with hypertelorism?


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1. Encephalocele
2. Fronto-nasal dysplasia
3. Atypical facial clefting
4. Crouzon syndrome
5. Bilateral cleft lip/palate




When does Romberg disease usually present?


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First 2 decades




How many syndromes have been described to include cleft lip and/or palate?


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, More than 300




In common usage, hemifacial microsomia refers to a spectrum of disorders; what
other terms are used?


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1. Craniofacial microsomia
2. Oculo-auriculo-vertebral spectrum
3. First and second branchial arch syndrome
4. Goldenhar syndrome




What is the rationale for avoiding distraction of the hypoplastic mandible in Robin
sequence?


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"Catch up" growth eventually occurs




What is the pattern of inheritance for van der Woude syndrome?


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Autosomal dominant but with variable penetrance

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