UMEM Educational Pearls

Category: Pediatrics

Title: Pierre Robin Syndrome

Keywords: Pediatric Airway, Pierre Robin Syndrome, Micrognathia, Emergent Tracheostomy, LMA (PubMed Search)

Posted: 11/30/2007 by Sean Fox, MD (Updated: 10/14/2019)
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Pierre Robin Syndrome

  • The prime features of this condition are a small jaw (micrognathia), cleft palate, and posteriorly positioned tongue.
  • In the newborn period, 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.
    • In severe cases, a tracheostomy may be required to provide a stable airway for the patient. (We just had an emergent tracheostomy done in our NICU this month).
  • Severity of airway obstruction varies from mild to life-threatening.
    • With only mild distress, attempt to relieve the obstruction by placing the child on his or her stomach; gravity will help to keep the tongue out of the airway.
    • Resuscitation of babies with more severe obstruction may be difficult because the micrognathia and the posteriorly protruded tongue can contribute to inadequate face-mask ventilation and make endotracheal intubation difficult (or impossible).
    • Consider LMA as a bridge to tracheostomy.
    • As soon as you recognize the presence of mirognathia, have someone call pediatric anesthesia and pediatric surgery.

Baraka, A. Laryngeal Mask Airway for Resuscitation of a Newborn with Pierre-Robin Syndrome. Anesthesiology. 83(3):646-647, September 1995.