Pierre Robin syndrome

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Infant hard and soft palates

Robin sequence

Infants must be kept face down, which helps the tongue fall forward and keep the airway open. These problems get better over the first few years as the lower jaw grows to a more normal size.

In moderate cases, the patient requires placement of a tube through the nose and into the airway to avoid airway blockage. In severe cases, surgery is needed to prevent upper airway obstruction. A tracheostomy (surgery to make a hole in the windpipe) is sometimes required.

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

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

Choking and feeding problems may go away spontaneously as the jaw grows. There is a significant risk of problems if the airway is not protected against obstruction.

• Feeding through a tube may occasionally be necessary to avoid choking
• Difficulty breathing may lead to low blood oxygen and brain damage
• Pulmonary hypertension
• Congestive heart failure
• Death

This condition is often apparent at birth. Call if choking episodes or breathing problems increase in frequency. Airway blockage may be indicated by a high-pitched, crowing noise when the child inhales (stridor) or blueness of the skin (cyanosis). Also call if other breathing problems occur.

Review Date: 11/21/2005
Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.