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.
