What is Pierre Robin Sequence?
Pierre Robin Sequence is a birth condition where the child is born with a lower jaw that is too small in size (micrognathia) or set back from the upper jaw (retrognathia). The tongue tends to become displaced towards the back of the throat as a result. This places the child at risk of falling back and causing an obstruction to the airway (glossoptosis). Often infants present with a cleft palate (an opening inside the mouth between the nose and mouth) however the lips remain intact.
What causes Pierre Robin Sequence?
Pierre robin’s sequence is caused by a failure in the normal development of the lower jaw before birth. At about 7-10 weeks into a pregnancy, the palate (roof of the mouth) forms on each side of the mouth. The tongue descends from between the two halves of the palate and the lower jaw must grow rapidly to allow enough room for the tongue to position itself, in infants with Pierre Robin Sequence the lower jaw does not grow properly, preventing the palate from closing, resulting in a cleft palate. The underdeveloped lower jaw causes the tongue to be positioned at the back of the mouth. This “sequence” of events is the reason why the condition is known as Pierre Robin Sequence.
How common is this Pierre Robin Sequence?
This condition occurs from 1 in 2,000 to 30,000 births which is rare compared to how common a cleft lip and/or palate occurs (1 in 700 live births). Pierre Robin Sequence can occur on its own as an isolated condition or as a feature of another syndrome or chromosomal condition. Parents who have had one child with isolated Pierre Robin Sequence roughly have between a 1 and 5% chance of having a second child with this condition.
Pierre Robin Sequence also occurs more frequently in children with environmentally-induced (teratogenic) syndromes including Fetal Alcohol Syndrome and Fetal Hydantoin Syndrome.
What problems can occur with Pierre Robin Sequence?
As the condition varies in severity depending on the individual, some children will present with more complications than others. The most common problems tend to be associated with breathing and feeding in early infancy. Most children with cleft palate are also prone to hearing complications such as build-up of fluid behind the eardrum. These ear infections can cause temporary hearing loss that can lead to a delay in speech and language development. This delay may be further worsened due to the abnormalities in the child’s oral structure making it difficult to accurately produce certain speech sounds.
How is Pierre Robin Sequence treated/managed?
Often surgery is required to close off the cleft palate, increase the lower jaw size and assist with breathing and hearing complications. Due to the high risk of feeding, speech and language difficulties it is important for the child to be monitored by a speech pathologist throughout early childhood. Speech pathologists conduct feeding assessments to ensure the child is safely swallowing food and drinks. Treatment can range from postural adjustments and selection of the right utensils to modification of the solids and liquids to ensure that they are not entering the airway causing lung infections. Ongoing Speech and language therapy often targets accurate speech sound production post surgery, however can range through to sentence structure and understanding language concepts (e.g. in front, behind) depending on the individual needs of the child.
For more information on this topic or any speech related fields, contact the ENT Clinic on 1300 123 368 and make an appointment with our speech pathologists Ashleigh Fattah or Jenna Butterworth.
About Ashleigh Fattah
Ashleigh Fattah is a Speech Pathologist who sees both children and adults. She has experience from a Master of Speech and Language Pathology degree and a spectrum of clinical environments including private practice, inpatient and outpatient hospitals, school and community based practice.
Ashleigh is committed to providing evidence-based interventions for children and adults alike, tailoring therapy to each client’s individual needs. Her clinical experience enables her to provide comprehensive one-on-one, group and school program interventions. Ashleigh is trained in providing an array of up-to-date therapy techniques to help both children and adults achieve their goals.
- Pierre Robin Australia Inc- Pierre Robin Sequence Syndrome
- Cohen, MM Jr. (1999). Robin Sequence and Complexes. American Journal of Medical Genetics 84: 311-315.
- Lehman, JA, Fishman, JR, Neiman, GS (1995). Treatment of Cleft Palate Associated with Robin Sequence: Appraisal of Risk Factors. Cleft Palate-Craniofacial Journal 32: 25-29. Pashayan, HM and Lewis, MB (1984). Clinical Experience with Robin Sequence. Cleft Palate Journal 21:270-276.
- Sheffield, LJ, Reiss, JA, Gilding, M (1987). A Genetic Follow-Up Study of 64 Patients with Pierre Robin Complex. Cleft Palate-Craniofacial Journal, May 1992, Volume 29:3, pp. 201-253.