Syndromic Craniosynostosis & Apert Syndrome

Chapter 15 of Volume 2, Head, Neck & Craniofacial. Revision notes and examiner-style viva questions with model answers for the FRCS (Plast).

27 viva questions in this chapter

1. Please describe this child.
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2. What is FGFR and how does a mutation cause this?
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3. How do you distinguish these syndromes?
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4. How do you assess and manage the airway?
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5. Does midface advancement fix the airway?
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6. The globe has subluxed. What do you do?
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7. Is the pressure raised, and why?
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8. How would you detect it?
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9. The pressure is raised and the cause is disproportion. What operation?
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10. Take me through a Le Fort III.
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11. External RED frame or internal distractors?
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12. When would you do a monobloc instead?
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13. Set out the plan from birth.
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14. What is Apert syndrome?
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15. Describe this hand and tell me how you would treat it.
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16. Classify this child.
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17. Where would this child be managed, and by whom?
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18. How is this child tested genetically in the UK?
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19. How would you consent for a decade of staged surgery?
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20. How would you manage the skull?
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21. When and how do you advance the midface?
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22. How would you manage the syndactyly?
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23. Describe what you see, and take me through how you’d approach this child.
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24. She desaturates during feeds and has stridor when upset. Weight has dropped from the 50th to the 9th centile. Still in hospital at four months. Her mother says: “Everyone keeps talking about her head and her hands. Nobody’s telling me why she can’t feed.”
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25. Take me through her surgical plan from now to skeletal maturity. What operations, in what order, and what drives the timing?
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26. Go.
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27. Bolt monitoring confirms sustained raised ICP. MRI shows no hydrocephalus and no Chiari. The vault looks adequately expanded posteriorly. Why is his pressure up, and what will you do?
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