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What is Pierre Robin Sequence?
Pierre Robin Sequence (PRS) is the name of a birth condition that results when the lower jaw fails to develop normally during the first trimester. The lower jaw may be micrognathic (small in size) or retrognathic (set back from the upper jaw). The abnormal size or position of the mandible causes the tongue to be displaced posteriorly into the throat (glossoptosis). The displaced tongue is unable to drop out of the way when the palatal shelves are closing and so PRS often includes the presence of a U-shaped cleft of the soft palate. A cleft lip is not part of the Pierre Robin Sequence. Due to glossoptosis, airway issues are the primary concern post delivery. Infants may outgrow their airway issues by 4-8 months of age because of jaw growth and better tongue control by the older infant.
Common characteristics of Pierre Robin Sequence
- Mandibular hypoplasia, retrognathia, micrognathia (relating to the jaw or jawbone)
- Glossoptosis (tongue displacement or retraction)
- U-Shaped posterior cleft/bifid uvula
- Small or obstructed airway leading to an increased respiratory rate, snoring, stridor, cyanosis, sweating, irritability, restlessness, or sleep apnea
Possible side effects of Pierre Robin Sequence
- Failure to Thrive due to airway issues and feeding challenges
- Congenital heart murmur
- Eye defects (Stickler Syndrome)
- Ear disease, infections and potential hearing loss
Diagnosis of Pierre Robin Sequence
Pierre Robin is diagnosed by clinical exam. It is relatively uncommon, occurring in approximately 1 in 2,000 to 1 in 30,000 live births depending on how it is defined. A genetic consultation is recommended to rule out the possibility of an associated syndrome, such as Stickler Syndrome.
Treatment for Pierre Robin Sequence
Because of the complex issues that can be associated with Pierre Robin Sequence, a child will benefit from being treated at a medical center where he/she will have access to pediatric specialists from many clinical areas. The Inova Children's Hospital craniofacial team covers 11 different specialties including: plastic and reconstructive surgery (craniofacial surgery), pediatric neurosurgery, genetics, otolaryngology, speech-language pathology, audiology, pediatric psychology, pediatric dentistry, oral maxillofacial surgery, orthodontics and nursing. The team collaborates with additional specialists as needed in order to provide comprehensive coordinated care that ensures each child’s optimal function, health and well-being during every phase of treatment.
List of typical treatment options
- Airway maintenance by conservative means such as prone positioning
- If positioning is not adequate, an obturator or tongue-lip adhesion may be necessary to pull the tongue forward
- In severe cases, neonatal bone distraction is performed to improve the airway and avoid nasopharyngeal intubation, endotracheal intubation, or tracheostomy
- Special feeding techniques to support feeding, for example chin support, a preemie nipple, a crosscut nipple and/or a Volufeed bottle
- Use of special cleft palate bottles to assist in feeding
- NG tube/G-tube as needed
Ongoing evaluation and monitoring
- Swallow study to evaluate the infant's ability to suck, swallow and breathe
- Polysomnogram to evaluate breathing, oxygen saturation, heart rate and carbon dioxide level. The study is performed during sleep, while awake, and during eating
- Monitor jaw for catch-up growth during the first year of life and determine if jaw growth was sufficient enough to bring the tongue forward and relieve airway obstruction
- Monitor child for airway obstruction due to enlarged tonsils, adenoids, or excessive weight
- Monitor child for failure to thrive and dehydration
- Delay palatal closure until at least 12-18 months of age to take advantage of jaw growth and improved airway
- Mandibular Distraction may be considered if jaw growth is poor and airway is compromised
- Monitor speech and language development
- Rule Out Stickler Syndrome and other conditions
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