Pierre Robin Syndrome

 

 

Considerations

 

  • Pediatric patient considerations

  • Difficult airway due to micrognathia, mandibular hypoplasia, glossoptosis:

    • Improves with age

    • Difficult bag mask ventilation & intubation 

  • Obstructive sleep apnea: 

    • Pulmonary hypertension, cor pulmonale

    • Negative pressure pulmonary edema

    • May need to be nursed prone

  • GERD, aspiration pneumonias

 

 

Airway Management

 

  • If intubation required:

    • May be impossible: maintain spontaneous ventilation 

    • Inhalational induction vs. titrated TIVA induction followed by asleep fibreoptic intubation

    • Strongly consider having an ENT surgeon equipped with a rigid bronchoscope on standby

    • Airway obstruction:

      • Oral & nasal airways, pull tongue forward, consider suturing to lip, prone position

      • LMA placement followed by fibreoptic intubation through the LMA

      • Nasal fiberoptic bronchoscopy 

      • Lightwand

  • If intubation not required (e.g. myringotomy & tubes):

    • IV/IH induction followed by LMA placement

    • Attempt to maintain SV

    • If airway obstruction occurs, paraglossal laryngoscopy may relieve obstruction & allow oxygenation to resume

    • Maintain with TIVA