1. Introduction - What is PRS?
Parents can find receiving a diagnosis for their baby with Pierre Robin Sequence (PRS) upsetting and stressful so we want to provide you with up to date information about the condition.
We aim to:
- provide you with accurate and up to date information and support
- reassure you that you are not alone
- offer some hints and tips that might be useful
This resource will enable you to understand about your baby's condition and treatment along with the support networks available to you. Click on each of the 4 boxes to find out some facts about PRS.
What is PRS?
Pierre Robin Sequence (PRS) is a condition where babies are born with a small jaw and a cleft palate (a gap in the roof of the mouth) causing the tongue to sit high up and towards the back of the mouth. This can cause issues with breathing as the tongue can block the airway. This is because babies breathe through their nose. These breathing difficulties can start from birth or shortly afterwards.
It is called a sequence as a sequence of events happens during pregnancy. The jaw does not grow as much as it should which pushes up the tongue and prevents the roof of the mouth from joining together. Some babies (about 30%) can have other conditions that have developed with it. PRS occurs in about 90 babies a year in the United Kingdom and is named after the doctor who discovered it. Because it is a rare condition it is not always diagnosed at birth.
What causes PRS?
It is not clear what causes PRS but it is thought to be caused by pressure on the jaw when the baby is developing and growing in the womb. It can also run in families and have a genetic (inherited) cause.
Not all babies with PRS will have a cleft palate but because many do it is often the cleft team that has the expertise to support them.
What does PRS mean for my baby?
PRS causes breathing issues which can be managed with the support of specialist health professionals from your regional cleft network. Breathing issues usually improve within 6- 12 months as your baby's airway grows. Most babies with PRS also have feeding difficulties and can be slow to gain weight.This is due to a combination of the tongue position, co-ordinating swallowing and using extra calories to breathe. Help and support with feeding is provided by your clinical nurse specialist or other member of the cleft network who will visit within 24 hours of your baby being referred to them.
Some babies with PRS experience changes inbreathing and/or feeding at around six weeks of age as they grow.
Will my baby need surgery?
A cleft palate will need to be repaired usually in the first year of life. A small number of babies with PRS will require surgical airways or jaw distraction.
A Consultant Plastic Surgeon discusses surgery
Consultant Jonathan Syme-Grant discusses surgery
Karine: Parents will often ask after the diagnosis of Pierre Robin Sequence, will my child need surgery? As a consultant plastic surgeon, could you explain to parents the when, how and why of palate repair in Pierre Robin Sequence.
Johnathan: Sure Karine. The most important thing for any parents who have just had a baby with a diagnosis of Pierre Robin Sequence is helping the baby to be well and that process needs to take priority so babies with Robin Sequence and a cleft palate will need surgery but I would just like to stress that that’s something that often needs to get put on the back burner in the early days and weeks while the child is thriving and able to breathe and growing nicely as any child should. Once they are doing that then we would look to repair their cleft palate and it’s different for every child but it’s usually in the region of around 9 months to a year. The reason we do that is because the cleft affects the soft part of the palate and prevents the body from being able to separate air flow from the mouth and nose and that’s essential in why is that important but what we are doing now in talking requires that anybody can do that to make the right sounds so really important for talking and also for swallowing because you don’t want to have food and drink coming down your nose for the rest of your life. So the timing is when the baby is well, the rational is because it’s really important to have a full and happy life and the why will obviously be discussed in much greater detail with your cleft team, but what we do here in Nottingham is that when the children are ready they come in as inpatients and they are asleep for the procedure and everything they need to have a functional palate is there, it just needs to be rearranged effectively and afterwards babies are incredible at healing they recover really quickly and get back to doing what they like to do which is learning, doing things and then they will also remain under the care of the wider cleft team. Some parents ask do we use any plastic because we are plastic surgeons or anything artificial but the answer is no we don’t put anything else there, as I said babies have everything they need for a functional palate it just needs to be rearranged a little bit.
Macmillan Long Term Follow Up Clinical Nurse Specialist
The Macmillan Long Term Follow Up Clinical Nurse Specialist will be available at the end of treatment days and will discuss healthy lifestyle and long term support and surveillance plans for children.