What to Expect

Pierre Robin sequence may first be discovered by ultrasound during pregnancy, shortly after birth, or a few weeks after birth. Once a definitive diagnosis of airway and feeding difficulties is reached, a team of specialists will meet to discuss how to navigate the best way forward for the baby. These specialists may include an ENT surgeon, a craniofacial airway orthodontist, and a plastic surgeon, as well as a neonatologist. In some cases, the best treatment to start with will be an OAP. In others it may be mandibular distraction osteogenesis (MDO), a surgery that cuts the lower jaw and implants extending screws that apply pressure to the jaw, making it elongate more quickly than it otherwise would, so that it comes to match or go beyond the upper jaw and broaden the airway. In another cases, tracheostomy may be an option, although it is often reserved as the last resort.

If the team, including the parents, decide that an OAP is the best option, the baby will be admitted to the NICU and the craniofacial orthodontist will make a mold of the baby’s mouth. The baby is awake for this procedure and anesthesia is usually unnecessary. A CT scan of the baby’s face also helps with the creation of the OAP’s tail. Once the mold is made and the images are taken, it takes a day or two for the orthodontist to fashion the OAP itself.

As soon as it is ready, the orthodontist and ENT surgeon will fit the OAP inside the baby’s mouth under nasal endoscopy (a tiny camera entering through the nose and showing the inside of the throat) so that the OAP’s tail can be adjusted into its optimal position while the baby is awake. The baby will be encouraged to feed by mouth on its own, in order to exercise its tongue and cheek muscles. The hospital stay typically lasts between two and three weeks after the OAP treatment is begun, unless the baby has other syndromic complications in addition to RS. Breathing problems resolve as soon as the OAP is put in. A sleep study conducted a few days into the OAP treatment helps to confirm that the OAP is working.

The baby will need to wear the OAP for three to six months on average. During that time, the lower jaw will typically grow fast enough to restore the proper balance in size between the upper and lower jaws (catch-up growth), and the baby’s airway will enlarge enough that even without the OAP, the baby can eat and breathe without incident.

What are the advantages of OAP?

OAP does not require surgery or anesthesia. OAP is gentle, painless, and noninvasive, so it does not leave any scars on the baby’s face. And OAP uses the baby’s own natural growth potential to enlarge and restructure the airway, to lengthen the jaw, and to strengthen the tongue and encourage nursing through the mouth.