Pierre Robin Sequence

Text supplied by Maeve Morrison, Speech Language Therapist and Tristan de Chalain, Plastic Surgeon, Middlemore Hospital.

Pierre Robin Sequence (PRS) involves the lower jaw being either small in size (micrognathia) and/or set back from the upper jaw (retrognathia).  As a result, the tongue tends to be displaced back towards the throat, where it can fall back and obstruct the airway (glossoptosis).
 
The basic cause appears to be the failure of the lower jaw to develop normally before birth.  At about 7-10 weeks into a pregnancy, the lower jaw grows rapidly, allowing the tongue to descend from between the two halves of the palate.  If, for some reason, the lower jaw does not grow properly, the tongue cannot descend and by mechanical obstruction, will prevent the palate from closing, resulting in a cleft palate.  The small or displaced lower jaw also causes the tongue to be positioned at the back of the mouth, possibly causing breathing difficulties.

Pierre Robin Sequence presents in different way, varying in severity from child to child. Problems with breathing and feeding in early infancy are the most common issues faced.
Some children may require partial or full tube feeding for the early weeks or sometimes months until their jaw grows sufficiently; if this growth does not occur a surgical procedure called distraction osteogenesis, may be suggested to hasten jaw growth.  If nasogastric tube feeding continues for a long period of time a gastrostomy tube may be considered. 
You need to learn how to position your baby in order to minimize breathing and feeding problems (i.e., not placing your baby on his or her back, and sitting them upright and slightly forward for feeding).  For severely affected children, positioning alone may not be sufficient.  Some children may require a surgical procedure to make satisfactory breathing possible.  Your Consultant will make recommendations on what options would be best suited to your child.

Monitoring of your baby’s growth is also important, as it takes a lot more energy for a baby with PRS to feed and breathe.  Sometimes it may be suggested to supplement milk feeding to increase the calorie intake of your baby.  Even when your baby seems to manage small volumes by themselves, it can take a long time to build up quantities of milk to the levels of babies that do not have PRS.  This is a slow process that should not be rushed.  Eventually normal feeding should be established.
In many patients, the lower jaw (mandible) grows rapidly during the first year of life. Many of the initial feeding and breathing difficulties you experience should gradually resolve over this period.  In some children, the jaw may grow so quickly that by the time the child is approximately four to six years of age, the profile looks normal. Children who do not experience this “catch-up” growth may require surgery on their jaws.

The main issues you need to be aware of associated with the cleft of the palate are:

• Surgery will be required to close the cleft. Surgical repair of the palate is generally done 6-12 months later than in children without PRS.  This delay allows time for the jaw to grow out, reducing the likelihood of breathing difficulties. (refer to: Our Child -Plastic Surgery)
• Monitoring of hearing.  (refer to the Ears, Nose and Throat)
• Speech development.  (refer to Speech and Language Development)