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 M. 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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