Pierre Robin sequence (PRS)

Overview

Pierre Robin sequence (PRS) is a congenital defect in which a baby is born with a small lower jaw, which causes the tongue to fall back into the mouth and block the airway, often leading to difficulty in breathing and eating. This condition may also cause the baby to have a U-shaped cleft palate (open roof of mouth).

Pierre Robin causes

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.

Pierre Robin risk factors

Because Pierre Robin syndrome is a genetic anomaly in multiple chromosomes, there are no concrete risk factors to be aware of. It may also be connected with a different syndrome, in rare cases.

Pierre Robin symptoms

Symptoms of Pierre Robin sequence are a small lower jaw with tongue pushed to back of throat blocking the airway.

Pierre Robin diagnosis

If your health care team suspects Pierre Robin syndrome, an airway assessment will be administered. A tracheostomy may also be necessary, if there is a chance of a laryngotracheal anomaly.

Pierre Robin treatment

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 position changes, 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.