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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.