What is the Pierre Robin sequence?
Pierre Robin sequence is also known as Pierre Robin syndrome or Pierre Robin malformation. It is a rare congenital birth defect characterized by an underdeveloped jaw, backward displacement of the tongue and upper airway obstruction. Cleft palate is also commonly present in children with Pierre Robin sequence.
How common is Pierre Robin sequence?
Isolated Pierre Robin sequence affects an estimated 1 in 8,500 to 14,000 people.
How do you treat a Pierre Robin sequence?
An infant with Pierre Robin sequence usually needs to be bottle fed, with breast milk or formula, using special nipples. The child may need supplemental calories to fuel the extra effort it takes to breathe and swallow. Surgery is necessary to repair the cleft palate.
How do I know if I have Micrognathia?
Diagnosis of micrognathia
- The relationship between your child’s tongue and lower jaw.
- If your child has a cleft palate.
- If your child has any facial asymmetries.
- The relationship of your child’s upper jaw to their lower jaw.
- Presence of a tongue tie (lingual frenulum)
- The overall health of your child.
How do I know if my baby has Micrognathia?
To diagnose microcephaly after birth, your baby’s provider measures your baby’s head circumference during a physical exam. Head circumference is the distance around your baby’s head. Your baby’s provider then compares your baby’s measurement to that of other babies of the same sex and age.
What does Pierre Robin sequence look like?
General Discussion. Summary. Pierre Robin sequence (PRS) is characterized by a small lower jaw (micrognathia) and displacement of the tongue toward the back of the oral cavity (glossoptosis). Some infants also have an abnormal opening in the roof of the mouth (cleft palate).
What is the treatment for Pierre Robin sequence?
Changes ( mutations ) in the DNA near the SOX9 gene are the most common genetic cause of isolated cases of Pierre Robin sequence. [2] Treatment is focused on the specific needs of each patient, but may include surgery to assist with breathing and feeding modifications to prevent choking. [1]
Is Pierre Robin syndrome genetic?
While some studies suggest there may be a genetic link, others point to crowding in the uterus or certain neurological conditions. Babies born with Pierre Robin sequence commonly experience trouble breathing and feeding early on, resulting from the tongue’s position, smaller jaw size and the cleft palate formation.
Can infants with Pierre Robin sequence learn to feed themselves?
Infants with minor degrees of Pierre Robin sequence can learn to feed using specially adapted nipples and bottles. However, for babies with more severe Pierre Robin sequence, the risk of inhaling fluid into the lungs is high. A feeding tube may be recommended as a temporary solution to allow for proper weight gain.
