Pierre Robin sequence
Babies with Pierre Robin sequence have the following problems
-small lower jaw
-a tongue that is placed further back than it should, and tends to fall backwards
-airway obstruction
-most babies also have a cleft palate
What causes Pierre Robin sequence?
As the name of this condition suggests, Pierre Robin is a sequence of events which start in the womb. Something happens to affect how the the lower jaw bone develops, so that it doesn't develop as well as it should. This then affects the position of the tongue: the tongue is higher, and this in turns affects the development of the palate, leading to cleft palate. The tongue also appears larger in the mouth due to the smaller size of the jaw.
Although numerous factors are thought to contribute to this sequence of events, no single cause is definitively responsible. Pierre Robin can be part of certain genetic conditions, or caused by low amniotic fluid levels, or connective tissue and muscular disorders. Two thirds of babies with Pierre Robin sequence don't have any other problems, but the remaining third have other problems also.
What problems does Pierre Robin sequence cause?
The tongue in babies with Pierre Robin has a tendency to fall back into the breathing passages, which may lead to airway obstruction. Feeding may also be difficult due to the positioning of the tongue and difficulties with breathing.
How is Pierre Robin sequence treated?
Breathing is usually better if the baby is nursed on the front or on the side. A period of observation in the Neonatal Unit may be required after birth, to monitor breathing and feeding and to ensure that the baby is growing and developing appropriately. Feeding through a naso-gastric tube may be required if the baby gets too out of breath to feed.
If positioning isn't enough, breathing can be helped by the placement of a plastic breathing tube into the nose, or by using a mask to deliver extra air.
The tube is called a nasopharyngeal airway. The tube goes from the nose to the back of the tongue, and pushes the tongue out of the way. This allows the baby to breathe better. If the nasopharyngeal airway is required, parents are taught how to insert it and manage it at home.
The breathing mask is called CPAP. This pushes extra air / oxygen into the lungs.
Either nasopharyngeal airway or CPAP are valid options. The medical team will discuss these with family, and offer what is best for the child, and what the hospital has most experience of.
Rarely, if breathing can't be managed using above methods, babies may require surgery to help breathing. The options would be surgery to bring the jaw forwards (mandibular distraction), or a tracheostomy. The surgical team would discuss the two options, pros and cons, and suitability, with the baby's family.
Babies grow rapidly between 3 and 12 months of age, and there is a good chance that airway problems will just get better during this time.
Management of babies with Pierre Robin sequence is complex, with numerous possible scenarios, and a variety of treatment options. Babies are usually cared for by numerous expert health professionals in specialist centres.
Section contributor:
Mitra Mummadi MRCS
ENT registrar
