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Pierre Robin sequence

Pierre Robin syndrome; Pierre Robin complex; Pierre Robin anomaly

Pierre Robin sequence (or syndrome) is a condition in which an infant has a smaller than normal lower jaw, a tongue that falls back in the throat, and difficulty breathing. It is present at birth.

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Causes

The exact causes of Pierre Robin sequence are unknown. It may be part of many genetic syndromes.

The lower jaw develops slowly before birth, but may grow faster during the first few years of life.

Symptoms

Symptoms of this condition include:

• Cleft palate
• High-arched palate
• Jaw that is very small with a small chin
• Jaw that is far back in the throat
• Repeated ear infections
• Small opening in the roof of the mouth, which may cause choking or liquids coming back out through the nose
• Teeth that appear when the baby is born
• Tongue that is large compared to the jaw

Exams and Tests

A health care provider can often diagnose this condition during a physical exam. Consulting with a genetic specialist can rule out other problems linked to this syndrome.

Treatment

Talk to your child's doctor about safe sleeping positions. Some infants with Pierre-Robin sequence need to sleep on their stomachs instead of their back to prevent their tongue from falling back into their airway.

In moderate cases, the child will need to have a tube placed through the nose and into the airway to avoid airway blockage. In severe cases, surgery is needed to prevent a blockage in the upper airway. Some children need surgery to make a hole in their airway or to move their jaw forward.

Feeding must be done very carefully to avoid choking and breathing liquids into the airways. The child may need to be fed through a tube to prevent choking.

Support Groups

More information and support for people with Pierre Robin sequence and their families can be found at:

• Birth Defect Research for Children -- www.birthdefects.org/pierre-robin-syndrome
• American Cleft Palate Craniofacial Association -- acpacares.org/
• National Organization for Rare Disorders --rarediseases.org/rare-diseases/pierre-robin-sequence

Outlook (Prognosis)

Choking and feeding problems may go away on their own over the first few years as the lower jaw grows to a more normal size. There is a high risk for problems if the child's airways are not kept from getting blocked.

Possible Complications

These complications can occur:

• Breathing difficulties, especially when the child sleeps
• Choking episodes
• Congestive heart failure
• Death
• Feeding difficulties
• Low blood oxygen and brain damage (due to difficulty breathing)
• Type of high blood pressure called pulmonary hypertension

When to Contact a Medical Professional

Babies born with this condition are often diagnosed at birth.

Call your provider if your child has choking episodes or breathing problems. A blockage of the airways may cause a high-pitched noise when the child breathes in. It can also lead to blueness of the skin (cyanosis).

Also call if your child has other breathing problems.

Prevention

There is no known prevention. Treatment may reduce breathing problems and choking.

Related Information

Pulmonary hypertension
Heart failure

References

Dhar V. Syndromes with oral manifestations. In: Kliegman RM, Stanton BF, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 337.

Purnell CA, Gosain AK. Pierre Robin sequence. In: Rodriguez ED, Losee JE, Neligan PC, eds. Plastic Surgery: Volume Three: Craniofacial, Head and Neck Surgery and Pediatric Plastic Surgery. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 36.

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Review Date: 11/1/2021  

Reviewed By: Anna C. Edens Hurst, MD, MS, Associate Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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