Pierre Robin Sequence

 

Micrognathia, Pierre Robin SequenceChildren 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.

 

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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