Craniofacial Dysostosis (Alperts, Crouzons, Pfeiffers)
Background
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Alpert syndrome:
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Difficult bag mask ventilation (high arched palate, choanal atresia)
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Difficult airway: cervical spine fusion, tracheal stenosis
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↑ ICP
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Congenital heart disease (10%) & other malformations
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Developmental delay
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Syndactyly
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Crouzons:
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Difficult bag mask ventilation & intubation. Patients may be significantly obstructed requiring early tracheostomy
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Mild developmental delay
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No visceral or extremity invovement
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Potential for ↑ ICP
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Considerations
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Pediatric patient
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Difficult airway: BMV, intubation:
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Midface hypoplasia
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Airway obstruction (OSA)
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CNS:
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May have ↑ ICP
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Visual loss, developmental delay
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For skull surgery:
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Long surgery (blood loss, hypothermia, positioning injuries)
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Venous air embolism
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Discharge to PICU