Children with respiratory distress can make even the most confident physician wish that the shift had ended a little sooner. Children with airway anomalies will make that already tight sphincter tone rise to epic levels. One of the most difficult airway cases I ever personally encountered was with a child who had Pierre Robin Sequence and immediately after birth was floppy and blue. No one (not even Peds Anesthesia) could intubate this little one. Fortunately, I was in the NICU and I had a Peds Surgeon immediately available to perform an emergent tracheostomy, but, what would I have done if this child was born in my ED, or I had no immediate access to a surgeon?

Pierre Robin Sequence

• Is poorly defined, but generally thought of consisting of:
  • Small jaw (micrognathia)
  • Posteriorly displaced tongue (glossoptosis)
  • Varying degrees of airway obstruction 
  • May also include cleft palate.
• Respiratory compromise from obstruction is of greatest concern.
  • Because the tongue is positioned in the back of the mouth, it tends to block the airway and cause respiratory distress.
• Many will also have feeding difficulties.

Pierre Robin Sequence: What to do

• Mild distress:
  • Even the mildest cases are often associated with hypoxia during sleep.
  • Placing the child in PRONE position will help (just like with the child with tracheomalacia); gravity will help to keep the tongue out of the airway.
• More significant distress:
  • Micrognathia and the posterior tongue can contribute to inadequate face-mask ventilation and make endotracheal intubation difficult (or impossible).
  • Don’t forget the Basics!!
    • Jaw Thrust (as much as you can)
    • Long Nasopharyngeal Airways can help provide ventilation!
    • Consider LMA as a bridge to tracheostomy.
  • Sometimes the Basics leave you a bit short…
    • Transtracheal Ventilation may be the only option you have, so be prepared to do it. (see Transtracheal Ventilation Morsel and Video)
    • And don’t be too proud… As soon as you recognize the presence of respiratory distress with the presence of mirognathia, have someone call pediatric anesthesia and pediatric surgery.