In this section
How you say it: Pee-air Row-barn Sequence
Pierre Robin Sequence (PRS) is a condition where babies are
born with a small lower jaw and a cleft palate (an opening in the roof
of the mouth). For some babies, this can affect their breathing
either from birth or shortly after birth. Most babies also have
feeding difficulties and problems gaining weight.
In Victoria, an average of 10 babies per year are diagnosed with
PRS, or one in 5000 live births (Victorian Perinatal Data Collection
Breathing difficulties in conjunction with a small jaw and cleft
palate may show in one or more of the following ways:
IMPORTANT: If you see any of these signs,
seek immediate medical treatment at the nearest hospital emergency department.
Sometimes the breathing difficulties may not be obvious. Feeding
problems and/or poor weight gain in the first weeks or months
after birth may be thought to be due to the cleft palate. In
most cases it is due to airway obstruction and occurs because your baby finds it hard to feed and breathe at the same time. Also, extra energy is
needed when breathing is obstructed.
You might notice two or more of the following signs in your baby:
If a baby does not receive appropriate medical treatment, they may have ongoing problems with nutrition and weight gain, or they may stop breathing.
NB: Because PRS is quite rare, it is sometimes not
diagnosed at birth especially if there do not appear to be immediate
breathing problems. Therefore, any baby with a small jaw and cleft
palate with feeding issues or difficulty gaining weight should be
suspected to have PRS.
The cause of PRS is still not fully
understood. It is thought to start as a small jaw, which causes the
tongue to stop the roof of the mouth (palate) closing during normal
development. Why the jaw is small in the first place is not known,
but it may be genetic. Some babies do have a family history of PRS or
similar problems, although most don't. Babies with PRS are screened
for other possible problems such as eye, kidney, bone and
occasionally heart conditions. However, for most babies, no other
problems are found.
Difficulty breathing can occur after birth because the small and
recessed jaw makes the tongue sit further towards the back of the
throat, narrowing or blocking the airway. The problems with
breathing may be better or worse at different times, and may be worse when your baby is in certain positions
(especially lying flat on their back), or when feeding or crying.
The teatment will depend on how bad the breathing and feeding
problems are. Your baby may be required to stay in hospital for
observation and treatment, including:
Sometimes, assistance to keep the airway clear is required. This would involve the insertion of a tube known as a nasopharyngeal (pron.
nay-so-far-an-jeel) tube (see Figure 1).
The majority of babies will outgrow the breathing problems
within three to six months as their airway grows.
Occasionally this does not occur and breathing problems persist,
or the airway obstruction becomes severe and life threatening. If
this occurs surgical treatment will be considered.
Surgical treatment involves
jaw distraction (lengthening) surgery, or in rare
Appointments with the neonatologists and respiratory team
following discharge will ensure respiratory and feeding problems
are resolving. These appointments will be quite frequent af first, until your baby is over three months of age and
no breathing issues have arisen.
Consultation with a facial surgeon and consideration for
jaw distraction (lengthening) may be required at a later
date if breathing problems do not resolve over time.
Consultation with a plastic surgeon will be required for a cleft
palate. Repair of the cleft palate will often be delayed until
after 12 months of age in babies with PRS.
Hearing appointments will be made for regular assessments, as
hearing difficulties often occur due to fluid in the middle ear
that does not drain out in the usual way. It is common for an ear,
nose and throat surgeon to insert ventilation tubes in the ears
(called 'grommets') at the time of palate closure. These tubes help
fluid drain from the middle ear.
All babies with PRS should have at least one appointment with a
children's eye doctor for a review.
If problems other than a small jaw and cleft palate are found,
or if there is any family history of such problems, consultation
with a genetics team will be arranged. Genetic testing may be helpful
in these circumstances.
Developed by The Royal
Children's Hospital Neonatal Medicine, in consultation with
Neonatology and Respiratory Medicine. Illustrations: Educational
Resource Centre. First published: January