If you have a newborn in a NICU who is having trouble breathing and has a small lower jaw and possibly a cleft palate, then your child may have Pierre Robin Sequence, which is a combination of a small lower jaw (mandible) causing a restricted space for the tongue so it is pushed high and back (glossoptosis), causing airway obstruction and cleft palate. There are other diagnoses associated with a jaw deformity (Treacher Collins Syndrome, Hemifacial Microsomia, and Craniofacial Microsomia, among others) that can lead to tongue based airway obstruction. There is a continuum of “small-ness” when it comes to baby mandibles and consequently there is a continuum of upper airway obstruction, swallowing dysfunction, and presence and size of a cleft palate. Treatment ranges from nothing to prone positioning to supportive feeding to surgically advancing the jaw. See Pierre Robin Sequence page.
Common symptoms of tongue based airway obstruction include:
- Small, irregularly and asymmetrically shaped mandible (lower jaw)
- Stertor: the noise made by the tongue sucking up against the pharynx on inspiration. This is differentiated from stridor which is the noise made by a dysfunctional vocal cord (s).
- Bluish discoloration: caused by low blood oxygenation
- Chest retractions on inspiration
- Poor feeding: difficulty coordinating suck, swallow, breath cycle because of the rapid inefficient breathing
- Glossoptosis: noted by only being able to see the underside of the tongue when looking in the mouth usually with the tongue tip pointing upward toward the roof of the mouth.
- Failure to gain weight or losing weight
The Craniofacial Team of Texas strongly believes that babies with Pierre Robin Sequence should be thoroughly evaluated by a team of airway experts including a Pediatric ENT, who routinely performs pediatric airway evaluations and can perform a tracheotomy on a newborn; a full time Craniofacial Surgeon, who is capable of performing mandible distraction and complex cleft palate repairs with low fistula rates; and a Pediatric Sleep Medicine Physician (Boarded in Pediatric Sleep Medicine), who can administer and read neonatal sleep studies. Eight years ago the members of the Craniofacial Team of Texas developed and implemented the first protocol in Central Texas for evaluating a newborn with a small mandible who is struggling to breath and feed. The protocol relies on a very close and interactive consultation between the NICU team and the Airway Team and relies heavily on clinical evaluation, experience of the team, and objective data primarily derived from a complete polysomnogram (sleep study). Many NICUs are not equipped or set up to perform complete polysomnograms on babies and will try to substitute a far less accurate test called a pneumocardiogram,which measures only a few of the parameters of a complete sleep study. The full data set read by an expert in pediatric sleep is critical to objectively determining the severity of airway obstruction called sleep apnea. Just like we all look different on the outside, we are all different on the inside, with significant variations in the size of the tongue, the pharynx, the palate, the skull base plane, and nasal cavity. These factors all impact the severity of the obstruction and consequently the ultimate function of the baby’s airway. It is not accurate enough to look at the severity of the small jaw to determine the impact to a specific patient. Our goal is to treat only those patients that need treatment and avoid exposing a child to an operation or intervention that they do not need. By strictly adhering to our Team Based Airway Protocol, we are able to avoid imparting an operation on a child that would do well without one.
Once our team determines that a baby has tongue based airway obstruction, the team will want to determine the exact source of obstruction. We do this by taking the baby to the OR for a complete endoscopic airway evaluation with a pediatric anesthesiologist, pediatric ENT, and a craniofacial surgeon together as a team to make sure the baby is safely evaluated. Endoscopic evaluation of newborns with tongue based airway obstruction should not be performed by physicians that are not experienced in taking care of complex neonatal airways! In order for the evaluation to be performed safely and effectively to gain the information needed to make proper treatment decisions, the team must perform the airway evaluation in a very precise and orchestrated manner with personnel that are experienced in this examination. The goal of the airway evaluation is to determine the sources of obstruction and their individual contributions to the overall clinical picture. Some patients have multiple levels of obstruction and if only one of them is addressed the baby will continue to struggle to thrive.
Parent Questions for Their Providers
- Is the NICU where my baby is located capable of performing a complete evaluation of my baby’s complex airway?
- Do they offer complete neonatal sleep studies administered by a board certified pediatric sleep specialist (usually pediatric neurology or pulmonology trained)?
- Do they have access to a pediatric airway team (Pediatric ENT, Craniofacial Surgeon, Pediatric Sleep Specialist, and Pediatric Anesthesiologist)?
- Does the hospital have a protocol for evaluating complex airways related to tongue based airway obstruction?
- Is the hospital prepared to perform an emergency tracheotomy in a neonate in the NICU? In the Operating Rooms?
- Is the hospital prepared and experienced in performing neonatal mandibular distraction?
Call the Craniofacial Team of Texas at our office number (512-371-1142) or page Dr. Kelley via Medlink (512-323-4565) for our team to assess your baby. Members of the Craniofacial Team of Texas have privileges at all hospitals in and surrounding the Austin region.