Robin Sequence – Pediatric Playbook | Boston Children’s Hospital

I’m Dr. Cory Resnick, and I’m a surgeon in the craniofacial center at Boston Children’s Hospital. Today, I’d like to talk to you about a condition known as Robin Sequence, often called Pierre Robin Sequence. Now, I know it can be very scary to hear that your child has a congenital condition that can affect breathing and feeding. While it can be a very serious medical condition, you should rest assured that, here at Boston Children’s Hospital, we can typically treat this very successfully. Robin Sequence is a combination of three facial differences that are present at birth. At 7 to 10 weeks into a typical pregnancy, the lower jaw, or the mandible, grows very rapidly. In a baby with Robin Sequence, however, the lower jaw does not come as far forward as it typically would. Instead, the lower jaw is set backwards, and this is called micrognathia. Micrognathia is the first, or initiating feature, of Robin Sequence. As a result of this small jaw, there’s not enough space for the tongue to sit normally in the floor of the mouth. Instead, the tongue is positioned at the roof of the mouth, and backwards into the back of the mouth. This is called glossoptosis, and is the second major feature of Robin Sequence. As a result of this backwards position of the tongue, air cannot always freely pass from the nose into the lungs. This airway obstruction, at the level of the back of the tongue, is the third feature of Robin Sequence. Now many babies with Robin Sequence will also have a cleft in the palate, though this is not a defining feature of Robin Sequence. An infant with Robin Sequence may have difficulty maintaining proper oxygen saturation levels while lying on his or her back. It may also be very difficult to coordinate the tasks of breathing and feeding. The findings of an infant with Robin Sequence, however, are quite variable. Many babies with Robin Sequence can be managed without an operation. Initially, when a baby can tolerate this type of management, we will attempt to turn the baby on his or her side, or belly, to help improve the airway. Sometimes, the placement of a rubber tube into the nose can help push the tongue base forward and open the airway. Other babies will tolerate the use of a specialized mask, which delivers oxygen and air under pressure, and helps to open the airway and help your baby breathe. In some babies, an operation may be necessary. At Boston Children’s Hospital, we offer three operations for the airway management component of Robin Sequence. The first of these is called a tongue-lip adhesion. A tongue-lip adhesion is an operation that brings the tongue forward and attaches it to the lower lip with sutures. Sometimes, this also involves placing sutures around the lower jaw. This brings the back of the tongue forward and away from the airway allowing air to pass more easily from the nose into the lungs. A tongue-lip adhesion must be undone at some point to allow for normal speech development. A typical time to reverse the tongue-lip adhesion is at the time of palate repair in babies who have a cleft palate. An alternative operation for babies with airway trouble and Robin Sequence is called mandibular distraction osteogenesis. This is the operation that I perform most commonly in babies with Robin Sequence. This operation utilizes the body’s own mechanism of healing to grow new bone and soft tissue to bring the lower jaw forward and thereby, bring the tongue away from the airway. In this operation, a small incision is made in the skin crease in the neck that allows access to the lower jaw. A bone cut is made with a special instrument, and a corkscrew-like device is applied to the jaw, with a bone plate in front and behind the bone cut. This device has a corkscrew that attaches the two plates, and an arm that extends out from the skin behind the ear. This is an example of the mandibular distraction device that I would use. As you can see, it’s very small and uses screws that are 1mm in diameter. When the device is applied to the jawbone, it looks like this. The only portion of the device that emerges from the skin is this activation arm. The rest is completely buried. After the initial operation, a special screwdriver can be used to advance the device, and thereby, grow the jaw. As the screwdriver is turned, the two plates of the device move away from one another, and create a gap in the jawbone. If we do this at a slow pace, bone will grow in that gap, and the jaw will get bigger, bringing the tongue away from the airway and improving breathing. When the active phase of turning has been completed, this activation arm can be removed very simply at the bedside. The remainder of the device is buried, and if the baby is doing very well at this point, often, they can be discharged to home. After about 6 weeks, the bone has typically filled in the gap, and the jawbone is now hardened in its new position. At that point, I do another small operation to remove the devices from both sides of the jaw. In those babies, in which the breathing problem is not entirely caused by obstruction of the airway at the level of the tongue, or in babies who have had a previous operation, such as a tongue-lip adhesion, or mandibular distraction osteogenesis, but have not achieved adequate breathing, an alternative operation is a tracheostomy. At Boston Children’s Hospital, we have the ability to care for your baby at every step in this process. This can begin with the management of the airway in infancy, can include placement of ear tubes, repair of the cleft palate, help with feeding and growing, speech surgery, if necessary, and even future dental and orthodontic needs. We have multispecialty clinics which bring specialists into one room to reduce your time at the hospital, and make your visits more efficient. We encourage you to call our office or go to our website to get more information about our program or schedule an appointment with one of our craniofacial surgeons.

3 thoughts on “Robin Sequence – Pediatric Playbook | Boston Children’s Hospital

  1. My son was born with Pierre Robin , he only had an upper jaw
    the rest was one big giant gap , the biggest cleft palate that was ever on record he had 2 operations one when he was 1 and a half years old and one when he was almost 4 , to close the cleft palate , and yes he had a problem with breathing as a baby because of his tongue , and the specialits created a plastic plate for him , so he could drink his bottles of milk , so he slept in the trendelburg position , he didn't had the lower jaw operation as he was born 23 years ago , i can write a long story but important things are with this children when growing up are , they can have nose bleeds when they are to active , my son suffers from headaches , because the skull is to small ( with some that have Pierre Robin the skull it stops growing at age of 4 ) the nose bleeds and headaches are a result of over heating the small skull , or when you have high temperatures as in summer time , my son still suffers when it is to hot out side like the small skull can't get rid of the heat , and yes eating is still a problem he does have his natural palate that the doctors brought together with the surgeries he had , but it is very small , so he still have trouble with eating letuce , endive , oranges but so far he is 23 years old now , we live in the Netherlands , and in the Netherlands there are 17 children /adults with Pierre Robin , in my eyes there are just a few with Pierre Robin world wide as i don't see many vids about Pierre Robin here so i want to thank you so much for this information that you uploaded for us people , i will put a link of my sons his specialists , when it come to schisis operations and for children with cleft lips , they are one of the best in the world , , perhaps you can exchange information

  2. I'm 30 and was born with PRS.
    I'm suffering a lot with lockjaw I'm just wondering if thats linked to PRS?
    I was on chemotherapy a few years ago and had lockjaw then.

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