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.