Pierre Robin Sequence

Pierre Robin sequence (PRS) is a disorder in which a baby is born with a small lower jaw (mandible), causing the tongue to fall back into the mouth and block the airway, which may lead to difficulty breathing. This condition can also cause the baby to have a cleft palate (open roof of mouth). The child will present with a very small or receding chin and the tongue will appear large. The cleft in the palate will have a U-shape, which is more typical of clefts formed due to Pierre Robin sequence.

What causes Pierre Robin sequence?

Pierre Robin sequence happens during a series of events during the child’s development as an embryo (that is why it is called a sequence). The cause of these events is unknown.

What is treatment like for Pierre Robin sequence?

The most important goal in treatment of Pierre Robin sequence is making sure the child is able to breathe adequately. This can be achieved by changing the baby’s position, placement of nasal trumpets (tubes that keep the airway open), tongue-lip adhesion surgery, mandibular distraction, or tracheostomy.

It will also be important to make sure the child is able to feed without difficulty. Our speech pathologist and feeding specialist will help your child every step of the way during this process.

The cleft palate is usually surgically repaired around 12 months of age. The timing of this surgery will depend on the child’s breathing and your surgeon. Your child may also need ear tubes placed, which will be determined by your child’s ENT surgeon. Your child will be followed by all the members of our dental team over the course of your child’s life.

What else should I know about this procedure?

As a cleft and craniofacial specialist, Dr. Aboutanos is qualified and ready to care for your child as they grow and develop. She is a member of the accredited Cleft and Craniofacial Team at St. Mary’s Hospital, and she will help coordinate your child's care with other specialists in a multi-disciplinary manner.