Craniofacial Dysostosis (Alperts, Crouzons, Pfeiffers)

 

 

Background 

 

  • Alpert syndrome:

    • Difficult bag mask ventilation (high arched palate, choanal atresia)

    • Difficult airway: cervical spine fusion, tracheal stenosis

    • ↑ ICP

    • Congenital heart disease (10%) & other malformations

    • Developmental delay

    • Syndactyly

  • Crouzons:

    • Difficult bag mask ventilation & intubation.  Patients may be significantly obstructed requiring early tracheostomy

    • Mild developmental delay

    • No visceral or extremity invovement

    • Potential for ↑ ICP

 

 

Considerations

 

  • Pediatric patient

  • Difficult airway: BMV, intubation:

    • Midface hypoplasia

    • Airway obstruction (OSA)

  • CNS:

    • May have ↑ ICP

    • Visual loss, developmental delay

  • For skull surgery:

    • Long surgery (blood loss, hypothermia, positioning injuries)

    • Venous air embolism

  • Discharge to PICU 

 

 

 

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