Pierre Robin syndrome (also called Pierre Robin complex or sequence) is a condition present at birth that is characterized by a very small lower jaw (micrognathia). The tongue tends to fall back and downward (glossoptosis) and there is cleft soft palate.

• very small jaw with marked receding chin
• tongue appears large (is actually normal size but big relative to jaw) and is placed unusually far back in the oropharynx
• high arched palate
• cleft soft palate
• choking on tongue (a small opening in the roof of the mouth)
• natal teeth

Infants must be kept prone (face down), which allows gravity to pull the tongue forward and keep the airway open. These problems abate over the first few years as the lower jaw grows and assumes a more normal size.

In moderate cases, the patient requires placement of a nasopharyngeal airway (a tube placed through the nose and into the airway) to avoid airway blockage. In severe cases, surgery is indicated for recurrent upper airway obstruction. A tracheostomy is sometimes required.

Feeding must be done very carefully to avoid choking and aspiration of liquids into the airways.

For support and information, see www.pierrerobin.org and www.cleftline.org.

• gavage feeding (feeding through a tube) may occasionally be necessary to avoid choking
• difficulty breathing leading to low blood oxygen and brain damage
• pulmonary hypertension
• congestive heart failure
• death