What Is Pierre Robin Sequence?
Pierre Robin is considered a sequence rather than a syndrome because the underdeveloped jaw begins a sequence of events. In a normal sequence of events, the tongue moves down away from the roof of the mouth at around seven to ten weeks gestation, allowing the roof, or palate, of the mouth to form normally. In children with Pierre Robin Sequence, the tongue never drops down because the lower jaw does not fully develop, which interferes with the normal development of the palate.
Pierre Robin Sequence can happen alone or as part of a syndrome. Some of these syndromes include Stickler syndrome and velocardiofacial syndrome. If your child was born with Pierre Robin Sequence as part of another syndrome, you might or might not have had genetic testing to determine your child’s chances of having a birth defect. But any time a child is born with a birth defect, it is natural to feel overwhelmed with the process and challenges that your child might have to face.
Working with our team of surgeons at the Yale Craniofacial Program and a team of interdisciplinary specialists means that your child will receive among the highest-quality, comprehensive care and the latest treatment options available. Each of our team members is dedicated to helping children with birth defects be as happy and healthy as possible, so that they, too, can experience the joys of childhood.
Causes of Pierre Robin Sequence
Pierre Robin Sequence can be a part of another syndrome or it can be isolated. The two most common syndromes that can include Pierre Robin Sequence are Stickler and velocardiofacial. With Stickler syndrome, Pierre Robin Sequence occurs in 10 to 30 percent of cases. With velocardiofacial syndrome, Pierre Robin Sequence occurs about 10 percent of the time.
It is natural to wonder if you will have another child with the condition. If your child has Pierre Robin Sequence because of external factors during pregnancy, then you have a 1 in 8,500 to 14,000 chance of having another child with the condition.
It is also natural to wonder if your child’s children will have Pierre Robin Sequence. If your child has the condition because of external factors during pregnancy, he or she has a minimal chance of passing Pierre Robin Sequence on. If the condition is part of a genetic syndrome, then the chance of having a child with Pierre Robin Sequence depends on the syndrome.
Features of Pierre Robin Sequence
If Pierre Robin Sequence is suspected in your child, the doctor will conduct a careful examination.
Some of the symptoms your doctor will look for are:
- Cleft Palate with a rounded or U-shape
- Small jaw
- Obstructed airway
- Large or set-back tongue
This list should be used as a guideline. Not every symptom is included. If your child has one or more of these symptoms, it does not mean that he or she has Pierre Robin Sequence. If you are concerned that your child might have Pierre Robin Sequence, please make an appointment with your child's doctor.
Treatments for Pierre Robin Sequence
Treatment for Pierre Robin Sequence includes:
- Breathing and Feeding: Before anything else, your baby must be able to breathe. If keeping your baby on his or her belly isn’t enough to keep the airways open, your child might need a small tube placed though the nose into the upper airway.
Cleft Palate and Hearing Problems:
- Around 12 to 18 months old, the cleft palate will need to be repaired. Because children with cleft palate tend to get a fluid buildup behind the eardrum, small plastic tubes are often inserted into the eardrum at the time of the surgery for clefting.
- Simulate the outcome of proposed surgical plans and adjust any discrepancies before surgery
- Follow and monitor your child’s condition during the post-surgical healing process
- Monitor your child’s long-term development