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Mandibular Distraction Osteogenesis for Pierre Robin Sequence: What Percentage of Neonates Need It?

Neonates with Pierre Robin sequence have respiratory distress caused by glossoptosis due to microretrognathia. Numerous therapeutic maneuvers have been used to stabilize the upper airway in these patients. The purpose of this study is to document the frequency with which each of these maneuvers is used, including the newest technique of mandibular distraction osteogenesis, in a single hospital with a large obstetrical service (22,646 deliveries between July 1, 2003, and June 30, 2006). The neonatal intensive care unit records for 3 consecutive years were reviewed retrospectively to identify all neonates with a diagnosis of respiratory distress (40%). Fifteen of these were classified as having Pierre Robin sequence, of whom, 9 did not have other craniofacial anomalies. The neonates with confounding additional congenital anomalies were excluded. The included neonates underwent airway and craniofacial evaluations by experienced pediatric otolaryngologists, craniofacial plastic surgeons and, occasionally, pediatric pulmonologists. Nonsurgical upper airway stabilization was attempted initially for all neonates. Four neonates with isolated Pierre Robin sequence could not be stabilized nonsurgically and underwent mandibular distraction in lieu of tracheotomy. Two of these subsequently required tracheotomy for reasons other than microretrognathia and glossoptosis. The remaining 5 Pierre Robin sequence neonates were stabilized without surgery and discharged home on room air. The treatment of neonatal upper airway obstruction due to Pierre Robin sequence includes both nonsurgical and surgical interventions. Use of a therapeutic algorithm can optimize nonsurgical management and minimize the need for tracheotomy. Mandibular distraction osteogenesis is an effective treatment to avoid tracheotomy in carefully selected Pierre Robin sequence neonates.