Pierre Robin Sequence

What causes Pierre Robin sequence?

A baby develops the condition while in the womb. Doctors aren't sure what causes it, though possible causes include:

• A change in the baby's genes
• Having a parent with a gene for the condition
• The baby's position in the womb early in the pregnancy

Twins may be more likely to have the condition.

Are there complications of Pierre Robin sequence?

If your baby has Pierre Robin sequence, they may have problems eating and breathing. Poor eating can cause delayed growth and development. Breathing problems can be very serious because the baby could stop breathing altogether.

Pierre Robin sequence symptoms

The symptoms of the condition are caused by the shape of the baby's mouth and include:

• A small jaw
• Trouble breathing
• A cleft palate
• Feeding problems

Diagnosing Pierre Robin sequence

Often, your doctor may notice the condition while your baby is still in the womb; they'll be able to detect abnormalities in their growth through ultrasounds.

Sometimes a baby is not diagnosed until shortly after birth when they have problems breathing. If your baby's doctor suspects Pierre Robin sequence, they may request an evaluation by a specialist or transfer to the Neotatal Intensive Care Unit for further monitoring.

How do doctors at UPMC Children's Hospital treat Pierre Robin sequence?

The Cleft-Craniofacial Center at UPMC Children's Hospital of Pittsburgh specializes in treatment for Pierre Robin sequence. We treat each case individually, based on your child's specific diagnosis and symptoms. 

Your team may include:

• A pediatrician/neonatologist
• A craniofacial surgeon
• A geneticist
• An ophthalmologist
• An otorhinolaryngologist (ENT surgeon)
• A sleep medicine doctor (pulmonologist)
• A speech pathologist

Our team will fully evaluate your child for any other health issues they might have related to Pierre Robin sequence. Treating the condition can take a long time. In fact, our team may work with your child for several years. This is because some health problems related to the condition need more attention than others.

We'll provide you with the knowledge and tools to care for your child with Pierre Robin sequence, including the best way to feed them and the best sleeping positions for easier breathing. We can also recommend resources for you to support your own mental health and well-being. Caring for a baby with this condition can be stressful, so it's important that you look after your health, too.

Surgery for Pierre Robin sequence

Many children with the condition don't need surgery, as a small jaw gets larger as they grow. Oftentimes, non-surgical interventions are enough to help your child eat and breath. These can include sleeping on their belly and nasal breathing and feeding tubes. Sometimes, surgery is necessary to help children eat and breathe more easily while their jaw is still growing. G-tube placement, mandibular distraction (jaw distraction), tongue lip adhesion, and rarely tracheostomy are some surgeries that are performed to treat Pierre Robin sequence. Also, your child may need corrective surgery if they have a cleft palate, but this procedure may not be necessary right away. 

Since babies with the condition are prone to ear infections, their doctor may also recommend an ear tube surgery.

Before surgery, you should have a list of questions prepared for your team. Some questions you may want to ask include:

• Will my child need more than one surgery?
• How long will we need to be in the hospital?
• What are possible complications from the surgery?
• Why is now the best time to do the surgery?

Prognosis

Children with Pierre Robin sequence generally have a good prognosis, or outcome. The key is early treatment and close follow-up by an experienced medical team to prevent problems that may be caused by the condition.