In Pierre Robin sequence, a condition present at birth, the infant has a smaller-than-normal lower jaw, a cleft palate, a tongue that falls back in the throat and difficulty breathing. The lower jaw develops slowly before birth, but may speed up during the first year of life. The cause of Pierre Robin syndrome is unknown.
Infants with Pierre Robin syndrome may have difficulty feeding, difficulty breathing, repeated ear infections, regurgitation of liquids through the nose, teeth that appear when born (natal teeth), a tongue that is large compared to the jaw and other congenital abnormalities or syndromes. Evaluation of patients with Pierre Robin sequence may include a sleep study, evaluation of the airway by a ear, nose and throat specialist, evaluation by a geneticists and evaluation for gastroesophageal reflux.
Treatment
In mild cases, patients can have improvement in their breathing by positioning them on their side or on their stomach. In cases of severe breathing problems, surgery may be needed to open the airway. The procedure that is performed is a neonatal mandibular distraction. Small titanium plates and screws are placed in the back of the lower jaw and a bony cut is made (osteotomy) between the plates. The plates are then gradually moved apart, usually one millimeter per day, to lengthen the mandible.
New bone regenerates and fills in the gap by a process called distraction osteogenesis (see below). After the new bone has a chance to harden, the plates are then removed from the mandible. Neonatal mandibular distraction has been used to lengthen the jaw as much as 2.5 centimeters and has been highly successful in avoiding a tracheostomy in the neck. Neonatal mandibular distraction should be performed by an experienced plastic surgeon who has completed a fellowship in craniofacial surgery.
Airway management in the newborn requires teamwork by pediatric craniofacial surgeons, pulmonologists, pediatric ear, nose and throat surgeons, pediatric anesthesiologists, geneticists and the staff of the neonatal intensive care unit.
Distraction osteogenesis was first used in orthopedic surgery and later adapted by surgeons to treat children with craniofacial conditions. Distraction is a less invasive and safer technique for correcting certain types of craniofacial deformities.
We offer a full range of distraction technology and techniques, including internal and external devices.