Pierre Robin Sequence

What is it?

Pierre Robin sequence is a combination of issues which begin with a small lower jaw (also called micrognathia). The jaw makes the tongue sit in the back of the mouth, which in turn can cause breathing problems and possibly a cleft palate (PDF), Sometimes, Pierre Robin sequence is a symptom of another syndrome, such as Stickler syndrome or velocardiofacial syndrome.

Children with Pierre Robin sequence can have difficulty breathing and eating. In severe cases of Pierre Robin sequence, when a child is crying or straining, he or she may appear to breathe alright because they are straining to keep the airway open. However, when the child relaxes or falls asleep, the airway is obstructed. This can lead to exhaustion and even heart failure if it is not diagnosed and treated promptly.

What is the cause?

Some of the syndromes that cause Pierre Robin sequence are genetic; when Pierre Robin sequence occurs alone (i.e. when it's not part of a syndrome) its cause is unknown.

How is it treated?

Pierre Robin sequence may involve non-surgical care or it could require multiple surgeries, depending on your child's unique condition. The majority of children with Pierre Robin sequence grow out of the condition as their jaws grow and develop. These children must lay tummy-down because the position causes the jaw and tongue to fall forward and open the airway wider.

In serious cases of this condition, one or more surgeries may be necessary. Possible surgeries include:

  • Tracheostomy (PDF). If the airway is severely blocked, a baby may need a tracheostomy—a surgical procedure that allows a baby to breathe out of a small opening in the trachea (windpipe).
  • Cleft palate (PDF) repair. If a cleft palate occurs as a result of Pierre Robin sequence, it is usually repaired between the ages of 12 and 18 months.
  • Gastrostomy tube placement. In severe cases, this tube is surgically inserted into the stomach through the abdomen. The tube allows feedings in instances where a child is not able to get enough nutrition by eating.
  • Mandibular (jaw) distraction. This surgery, also called distraction osteogenesis, involves implanting a device into the jaw which gradually repositions the facial bones over time. This surgery is less invasive than regular jaw surgery and may reduce pain and recovery time. Children's ENT team has the world's largest group of patients that have received distraction osteogenesis for jaw conditions.
  • Glossopexy. This surgery involves attaching the tongue to the inner lip until the jaw grows enough to allow normal breathing. The tongue-lip adhesion is temporary and usually is reversed in another surgery six to nine months later.

About surgery at Children's for ENT issues related to Pierre Robin sequence

The ENT surgery team at Children's provides next-generation care to neonatal infants, newborns, children, and adolescents from throughout the Upper Midwest. This multidisciplinary team provides some of the most cutting-edge, delicate treatments available, including newborn surgery and minimally invasive surgery. Otolaryngology surgery is performed at Children's - Minneapolis, Children's - St. Paul, and Children's West.

If you are a family member looking for a Children's ENT specialist, visit Find a Doctor.

If you are a health professional looking for a consultation or referral information, please call Children's Physician Referral at 1-866-755-2121 (toll-free).

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