Pierre Robin Sequence

What is Pierre Robin Sequence?

Pierre Robin sequence is a condition where infants are born with an abnormally small lower jaw. This causes the tongue to sit in an abnormal position and obstruct the infant’s airway. Many, but not all, infants with Pierre Robin sequence will also have a cleft palate.

What Causes Pierre Robin Sequence?

Pierre Robin sequence has many causes. Infants may have a small jaw because of positioning during pregnancy.  Some infants develop a small lower jaw because of a genetic abnormality, or syndrome, which can put them at risk for other birth defects. Your surgeon and geneticists will assess your baby and provide guidance whether additional testing is needed to look for such issues.

What are the Symptoms of Pierre Robin Sequence?

The severity of Pierre Robin sequence can vary. The most significant cases have difficulty breathing and may require placement of breathing tube by the neonatologist or pediatrician shortly after birth.

In less severe cases patients may demonstrate:

  • Signs of increased effort breathing
  • Loud breathing or snoring
  • Difficulty feeding or early fatigue when feeding
  • Coughing or gagging when feeding
  • Inability to gain weightHow are patients with Pierre Robin sequence evaluated?

 Patients with Pierre Robin sequence undergo care with a team of specialists. These include a pediatric plastic surgeon, a pediatric ENT specialist, a feeding expert, a geneticist, and a pediatrician or neonatologist. An overnight sleep study will provide information about the severity of the airway obstruction called obstructive sleep apnea (OSA). The pediatric ENT specialist will help assess the baby’s entire airway to look for other sources of airway obstruction, and the pediatricians and geneticists will provide a thorough evaluation to look for syndromes and other conditions your baby may be at risk for.

How is Pierre Robin Sequence Treated?

Many cases of Pierre Robin sequence can be managed without surgery. In less severe cases, special positioning during feedings and sleep will improve the position of the tongue and allow an infant to breath well until they outgrow the problem. In more significant cases surgery may be required. Distraction osteogenesis (DO) is a surgical procedure that stretches the bone of the lower jaw to pull the tongue forward and improve airway obstruction. Your plastic surgeon and the rest of the team will help determine if your child is a candidate for this procedure. Patients who are not candidates for distraction osteogenesis may benefit from the placement of a tracheostomy or feeding tube. Repair of a cleft palate is important for infants to develop normal speech. This operation is usually performed around one year of age, but may be safely delayed if your child if there is concern about your child’s breathing. Your plastic surgeon will perform this operation and will determine the best time for this operation depending on your child’s condition.