Pierre Robin sequence, also known as Pierre Robin complex or syndrome, is a birth defect characterized by a wide, U-shaped cleft palate, a small lower jaw and a tongue that tends to fall back toward the throat, potentially obstructing the airway.
Because this combination of features can make it difficult or impossible for a baby with Pierre Robin to feed or breathe normally, children with this condition may need special procedures at birth to ensure adequate breathing and feeding.
Pierre Robin sequence is characterized by:
Pierre Robin sequence is diagnosed at birth by physical examination. The presence of the small lower jaw distinguishes Pierre Robin sequence from a cleft palate only.
An infant with Pierre Robin sequence should be seen shortly after birth by a nurse from the Center for Craniofacial Anomalies, who will make sure he or she is able to adequately feed and breathe. The infant should also be seen for a full team evaluation.
If the tongue obstructs the airway or if the infant is having breathing difficulties, he or she will be referred to the Pediatric Pulmonary Clinic. The child may need a lip-tongue plication, a procedure that temporarily attaches the tongue to the lower lip, opening the airway and making it easier to breathe.
An infant with Pierre Robin sequence usually needs to be bottle fed, with breast milk or formula, using special nipples. The child may need supplemental calories to fuel the extra effort it takes to breathe and swallow.
Reviewed by health care specialists at UCSF Benioff Children's Hospital.
Last updated
September 27, 2011
Craniofacial Anomalies Center
513 Parnassus Avenue, S-747
San Francisco, CA 94143-0570
Phone: (415) 476-2271
Fax: (415) 476-9513
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