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What is PRS?

Note:  The correct name for PRS is Pierre Robin Sequence.  Many times it is referred to as a syndrome, but it is actually a sequence of events that led up to the condition.  If there is no other condition present, then it would be considered "Isolated Pierre Robin Sequence."   If there is another condition present, then it is referred to as "Pierre Robin Sequence."

The correct pronunciation is Pierre, just like it sounds and then Robin is pronounced like Ro-ban.  Pierre Robin was a French physician who described the association of a small lower jaw and breathing problems in infants.  It was first described in 1923.  You might see it called Pierre Robin Syndrome, Pierre Robin Sequence, Pierre Robin Complex, Robin Anomalad, Robin Sequence or Robin Complex.  

PRS is characterized by a combination of three features, possibly due to the underdevelopment of the lower jaw. The lower jaw is abnormally small (micrognathia), the tongue is displaced downwards (glossoptosis), and there is an abnormal opening in the roof of the mouth (cleft palate).  The tongue might have a tendency to ball up in the back of the mouth causing the airway to block and possible apnea. 

It is important that the baby be seen by a geneticist early in life, so they can watch for other findings that may occur.  PRS is associated with many other genetic disorders, or can be found independent of any genetic causes.  The cause is unknown and it does not tend to run in families, unless there is another genetic condition present.   One study showed the chances of parents having a second child with PRS to be 1 in 30,000.  It is best to look at the whole picture with your child's geneticist to discuss these issues.

Many children with PRS  have recurrent ear infections and end up needing tubes surgically placed.  Some children have more than one tube surgery due to the tubes' tendency to fall out as the child grows.  Your child needs to be watched closely for ear infections and hearing function.  Hearing levels may fluctuate due to infections.  An audiologist is an important part of the medical team and your child needs regular hearing checks.

Other surgeries would include palate repair and possibly surgeries to enhance their speech.  The cleft palate is typically a horseshoe shaped wide cleft.  Once the cleft has been closed, many children progress with their speech abilities.  Check with your pediatrician or team members to see what speech services and developmental services are available in your area.

Feedings are almost always an issue with PRS children.  Many end up with g-tubes because they can't gain weight properly.  Every child is different and will feed differently.  There are several bottles available for cleft palate babies and you may go through a course of trial and error to find the correct one.  The nipples may have to be adjusted by making the cuts longer to ease the feedings.  Babies will have a tendency to wear out during feedings because they use so much energy to eat.

Breathing issues are abundant with PRS children as well and until the breathing is controlled, the baby will have trouble gaining weight.  Often, when the child cries it will keep the airway open.  However, when the child relaxes or falls asleep, airway obstruction can occur.   Sometimes positioning them on their stomach will help them, because it will force the tongue to fall forward and keep the airway open.  Some teams use the tongue lip adhesion method to hold the tongue down.   Other, more severe cases, require tracheostomies to aid in breathing until the jaw can grow out and allow a place for the tongue to go.  Other babies don't need anything special to breathe.  It really depends on the child, the size of the chin and the tongue.  Some teams perform a jaw lengthening surgery to increase the size of the lower jaw allowing the tongue to "lay down" and thus avoiding the trach.  These issues need to be discussed in detail with your child's medical team.

Dental issues are very common in PRS children.   The lower jaw sometimes does not grow to the same size as the upper jaw and the teeth are crowded.  Many of these children go through orthodontics to correct teeth placement.

Most school age children with PRS will require some speech therapy, especially to reduce nasality in their speech.  The speech language pathologist will also work with them to correct bad habits.  Make sure you request an evaluation if your school does not offer it.

Fortunately, the small jaw grows out pretty fast in childhood.  Most PRS adults do not look as though they ever had a small jaw.   Sometimes the only visible factor in an adult is found through x-rays.  With other adults their small jaw is just part of life for them.

 

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