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General information

Please see the information on the Pierre Robin home page.

 

Management of the jaw and breathing

Some babies with Pierre Robin sequence have very little problem with breathing. It is generally advised that, contrary to advice for most babies, babies with Pierre Robin sequence are nursed on the side or towards the front (rather than on the back).

Occasionally a small tube needs to be passed through the nose, behind the tongue, to improve breathing. This is called a nasopharyngeal airway or prong.

In more severe cases, some surgeons sew the tongue to the lip as a temporary measure just to hold it forward. This is not done very much in the UK but is carried out quite widely in the USA.

Another option in severe cases is to actually lengthen the jaw by a technique known as mandibular distraction.

Finally, a very small number of babies with major problems with their breathing require tracheostomies.

Surgery

There is some debate between cleft teams and surgeons about the best time to repair the palate in babies with Pierre Robin sequence. The problem here is that repairing the palate may temporarily make the breathing worse. Some surgeons operate at the usual age for cleft palate repair (as early as six months) but accept that there may be some difficulties with breathing after the operation and may for example use a nasopharyngeal airway (nasal prong) in the period after the operation. Other surgeons prefer to wait in the hope that as the jaw grows the breathing will get better and the chances of breathing difficulties after the operation will be reduced.

The operation should take place in a hospital with good paediatric facilities. It is likely to take between 1½ and 3 hours, depending on the extent of the cleft, and babies will usually stay in hospital for at least two days.

Feeding may take some time to be re-established after cleft palate repair but in most cases should be re-established in one to two weeks.

Because the clefts in babies with Pierre Robin sequence tend to be very wide, it is very often necessary for relaxing cuts to be made on the side of the palate but these heal very quickly. There is also an increased risk of developing a small hole in the repair. These sometimes heal spontaneously but occasionally require a further small operation.

Long term outcome

The chin does grow forwards in infants with Pierre Robin sequence. The final position of the jaws has probably more to do with genetics than the Pierre Robin sequence. The change is gradual, with improvement in the first year but continuing through childhood.