What is the definition of Pierre Robin Sequence?

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.

What are the alternative names for Pierre Robin Sequence?

Pierre Robin syndrome; Pierre Robin complex; Pierre Robin anomaly

What are the causes for Pierre Robin Sequence?

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.

What are the symptoms for Pierre Robin Sequence?

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

What are the current treatments for Pierre Robin Sequence?

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.

What are the support groups for Pierre Robin Sequence?

The following resources can provide more information on Pierre Robin sequence:

  • Birth Defect Research for Children -- www.birthdefects.org/pierre-robin-syndrome
  • The Cleft Palate Foundation -- www.cleftline.org
  • National Organization for Rare Disorders --rarediseases.org/rare-diseases/pierre-robin-sequence

What is the outlook (prognosis) for Pierre Robin Sequence?

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.

What are the possible complications for Pierre Robin Sequence?

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 should I contact a medical professional for Pierre Robin Sequence?

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.

How do I prevent Pierre Robin Sequence?

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

Infant hard and soft palates

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.

  • Condition: Severe Pierre Robin Sequence (PRS)
  • Journal: The Journal of craniofacial surgery
  • Treatment Used: Neonatal Mandibular Distraction Osteogenesis (MDO)
  • Number of Patients: 12
  • Published —
This study evaluated the use of mandibular distraction osteogenesis (MDO) in the treatment of neonates with severe Pierre Robin sequence (undeveloped jaw; PRS).
Clinical Trial
  • Status: Recruiting
  • Phase: N/A
  • Intervention Type: Other
  • Participants: 130
  • Start Date: May 24, 2018
Brainstem Dysfunction Involvement in the Pathogenesis of Pierre Robin Sequence