Pierre Robin Sequence
Pierre Robin Sequence is a condition that affects a baby’s mouth. It usually involves a cleft palate and a small lower jaw. Breathing difficulties occur because esophagus and airway blocking by the tongue.
What is Pierre Robin Sequence?
Pierre Robin Sequence is a condition that affects a baby’s mouth. It usually involves a cleft palate and a small lower jaw. In this condition, the tongue is further back in the mouth than normal. When this happens, it can block the airway, causing problems with feeding and breathing.
What Causes Pierre Robin Sequence?
For most babies, there is no known cause of Pierre Robin Sequence. It can start with a small problem early in pregnancy, like slow growth of the jaw. This can cause problems with the palate and tongue. Being pregnant with more than one baby can increase the risk for the condition because there is less room in the womb. This puts pressure on developing babies.
Sometimes, a genetic syndrome or diagnosis can be associated with Pierre Robin Sequence.
What are the Signs and Symptoms of Pierre Robin Sequence?
Prenatally, the main sign of Pierre Robin Sequence is a small jaw which may be seen on a prenatal ultrasound. A prenatal MRI can also be done to get a better look at your baby’s face to look for signs of a cleft palate and better define the severity of the condition, as well as detect other anomalies if present. The MRI often helps in planning a safe delivery for your baby.
How is Pierre Robin Sequence Diagnosed?
After signs of Pierre Robin Sequence are seen on a prenatal ultrasound and MRI, a physical exam and further testing will be done after birth to formally diagnose the condition.
In rare cases, especially if there are concerns of other associated fetal abnormalities, special arrangements may need to be made for delivery of the baby to secure the airway at the time of delivery.
How is Pierre Robin Sequence Treated?
Your doctor will watch your baby’s development with ultrasounds during your pregnancy. After birth, your baby will have an exam and may be referred to a craniofacial specialist. Many children get better on their own as they grow and the jaw gets bigger.
In mild cases, Pierre Robin Sequence can be managed by placing your baby on their stomach or side to sleep. If your baby has trouble eating, a feeding therapist can help you with feeding positions and specialized nipples. A tube can also be inserted through the nose for feeding.
In some cases, surgery may be done to enlarge the lower jaw or bring the tongue forward. This can make it easier to eat and breathe.
If a cleft palate is present, surgery may be done to close the hole.
Possible Problems
Babies with Pierre Robin Sequence have an increased risk of heart conditions. Your baby’s doctor will recommend an echocardiogram (echo). This is a test that looks at the anatomy and blood flow in the heart. Babies with Pierre Robin Sequence also have an increased risk of eye problems. Your baby’s doctor will recommend an eye exam. This is a test that looks at the anatomy of the eyes.
Lifelong Considerations
Babies with breathing and feeding issues that are managed well usually have a good outlook for their future health. Routine health monitoring with a craniofacial team is recommended throughout childhood.
However, if feeding problems are not managed, babies can have poor weight gain and failure to thrive. Breathing problems must be managed or the baby’s brain may not get enough oxygen. This can cause brain damage. Babies with other issues, such as heart or eye conditions, will need additional specialty follow-up.