Table of ContentsView AllTable of ContentsSymptomsFrequencyCausesTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Frequency
Causes
Treatment
Pierre Robin sequence (or syndrome) is a rare congenital condition that results in a combination of problems present at birth. These problems include an underdeveloped lower jaw, cleft palate, a tongue that is placed very far back in the throat, and airway obstruction.
These abnormalities at the time of birth frequently lead to speech problems in children with Pierre Robin sequence.
This article discusses the causes of Pierre Robin Sequence, how it is treated, and what the prognosis is.
This photo contains content that some people may find graphic or disturbing.See PhotoJean Marc Giboux / Getty Images
This photo contains content that some people may find graphic or disturbing.See Photo
This photo contains content that some people may find graphic or disturbing.
Jean Marc Giboux / Getty Images
Function of the Lingual Frenulum and Pain Causes
Symptoms of Pierre Robin Sequence
Pierre Robin sequence is a rare congenital condition that causes a constellation of defects of the head and face, including:
These problems can cause problems with speech and other issues as the child grows. In 10-85% of cases, other systemic manifestations may occur including:
How Common Is Pierre Robin Sequence?
The incidence of Pierre Robin sequence is between 1 in 8,500 and 1 in 14,000 births, affecting males and females equally.
Pierre Robin sequence can occur on its own but is also associated with several other genetic conditions includingStickler syndrome, CHARGE syndrome, Shprintzen syndrome, Mobius syndrome, trisomy 18 syndrome, trisomy 11q syndrome, deletion 4q syndrome, and others.
Causes of Pierre Robin Sequence
There are a few theories as to what causes Pierre Robin sequence:
When Pierre Robin sequence occurs without any other anomaly disorders, a DNA mutation that reduces the activity of a gene called SOX9 may be to blame. The SOX9 protein aids in skeletal development and less of it may contribute to the craniofacial abnormalities in Pierre Robin sequence.
Pierre Robin Sequence vs. SyndromePierre Robin sequence and Pierre Robin syndrome are the same disorder. Pierre Robin syndrome was the earlier name for the condition. It was renamed as a sequence because the malformation leads to a sequence of events that cause additional problems.
Pierre Robin Sequence vs. Syndrome
Pierre Robin sequence and Pierre Robin syndrome are the same disorder. Pierre Robin syndrome was the earlier name for the condition. It was renamed as a sequence because the malformation leads to a sequence of events that cause additional problems.
How Pierre Robin Sequence Is Treated
Natural growth also often cures any respiratory (airway) problems that may be present. Sometimes an artificial airway (such as a nasopharyngeal or oral airway) needs to be used for a period of time. Cleft palate must be repaired surgically as it can cause problems with feeding or breathing. Many children with Pierre Robin sequence will require speech therapy.
What Is the Prognosis for Children With Pierre Robin Sequence?With treatment, children with Pierre Robin sequence can go on to lead normal lives. Isolated Pierre Robin sequence (when the condition occurs without another associated syndrome) does not usually increase mortality risk.
What Is the Prognosis for Children With Pierre Robin Sequence?
With treatment, children with Pierre Robin sequence can go on to lead normal lives. Isolated Pierre Robin sequence (when the condition occurs without another associated syndrome) does not usually increase mortality risk.
Summary
Pierre Robin sequence is a rare congenital condition that results in a range of problems affecting the head and face. Classic symptoms inlcude an underdeveloped lower jaw, cleft palate, and airway obstruction.
Although there is no cure for Pierre Robin sequence, the condition can be managed with surgeries and other treatments. Speech therapy may also be necessary as the child grows.
1 SourceVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Soto E, Ananthasekar S, Kurapati S, et al.Mandibular distraction osteogenesis as a primary intervention in infants with Pierre Robin sequence.Ann Plast Surg. 2021;86(6S Suppl 5):S545-S549. doi:10.1097/SAP.0000000000002702Additional ReadingCleft Palate Foundation. Pierre Robin Sequence.Medline Plus. Pierre Robin sequence.Medscape. Pierre Robin Syndrome.
1 Source
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Soto E, Ananthasekar S, Kurapati S, et al.Mandibular distraction osteogenesis as a primary intervention in infants with Pierre Robin sequence.Ann Plast Surg. 2021;86(6S Suppl 5):S545-S549. doi:10.1097/SAP.0000000000002702Additional ReadingCleft Palate Foundation. Pierre Robin Sequence.Medline Plus. Pierre Robin sequence.Medscape. Pierre Robin Syndrome.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Soto E, Ananthasekar S, Kurapati S, et al.Mandibular distraction osteogenesis as a primary intervention in infants with Pierre Robin sequence.Ann Plast Surg. 2021;86(6S Suppl 5):S545-S549. doi:10.1097/SAP.0000000000002702
Cleft Palate Foundation. Pierre Robin Sequence.Medline Plus. Pierre Robin sequence.Medscape. Pierre Robin Syndrome.
Cleft Palate Foundation. Pierre Robin Sequence.
Medline Plus. Pierre Robin sequence.
Medscape. Pierre Robin Syndrome.
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