Pierre Robin Sequence
Also known as: Pierre Robin Syndrome.
What is Pierre Robin Sequence?
In 1923, a French physician named Pierre Robin described a child with a small jaw, tongue that is displaced back towards the throat and cleft palate. Therefore, this combination of findings became referred to as Pierre Robin (pronounced Roban) Sequence. Other names have been used for the condition such as Pierre Robin Syndrome and Pierre Robin Triad.
Common clinical characteristics of Pierre Robin Sequence are:
- micrognathia (small lower jaw) or retrognathia (set back lower jaw)
- glossoptosis (displacement of the tongue to the back of the throat)
- cleft palate
- and airway obstruction leading to difficulty with breathing
What causes Pierre Robin Sequence?
Between 7-10 weeks of pregnancy, a fetus’s jaw grows rapidly which allows the tongue to move down away from the roof of the mouth. Once the tongue has moved down, the palate (roof of the mouth) can close. If the lower jaw is small, there is not enough space for the tongue to move down. The tongue remains positioned along the roof of the mouth often resulting in the palate not closing (cleft palate). Because it is a sequence of events that occurs to cause this condition, it is called Pierre Robin Sequence.
Most children with Pierre Robin Sequence (PRS) will not have other problems and in those children PRS is an isolated finding. However, some children will have an underlying genetic condition associated with Pierre Robin Sequence. Children with PRS should be evaluated by a Clinical Geneticist to determine if PRS in their child is isolated or part of a genetic condition.
In children with isolated Pierre Robin Sequence, the cause is often unknown. However, crowding of the fetus during pregnancy, decreased jaw movement due to neurological conditions, and possibly genetic factors are thought to play a role in PRS.
What is the prognosis for my child with Pierre Robin Sequence?
Children with Pierre Robin Sequence often have difficulty with feeding and breathing during early infancy. All children should be evaluated and monitored closely by a multidisciplinary Craniofacial Team during this period. Children with more significant feeding and breathing difficulties may need specialized medical and/or surgical intervention.
With appropriate management, children with Pierre Robin Sequence will attend school, have friends, and most of all enjoy life!
What is the chance of my future children having Pierre Robin sequence?
The risk that future children will have Pierre Robin sequence depends on whether it is an isolated finding or part of a genetic condition. When isolated, the risk is typically 1 to 5 percent. If it is due to a genetic condition, the risk for future children will depend on the diagnosis. When considering a future pregnancy, genetic counseling can help you understand your specific risk and options for screening and/or prenatal diagnosis.
This page was last updated on: November 13, 2020 03:14 PM
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