Purpose Statement:

Neonates with Pierre Robin sequence suffer from micrognathia and glossoptosis often causing significant airway obstruction which may lead to inability to feed and failure to thrive. A number of therapeutic maneuvers have been reported to stabilize the upper airway in PRS, ranging from positioning to surgery. It has been well documented that mandibular distraction is effective in preventing the need for tracheostomy, yet the percentage of neonates that need surgical intervention versus those that may be treated more conservatively is not well documented. While at least one algorithm detailing the management cascade has been published, the frequency with which each of the maneuvers is utilized has not been reported. The purpose of this study is to document that frequency in a busy NICU associated with a large annual delivery rate.

Methods and Materials:

All charts of neonates with respiratory distress, born and cared for at a single tertiary hospital with large delivery, high risk obstetrics and NICU services between July 1, 2003 and June 30, 2006, were reviewed retrospectively to identify those neonates who had both respiratory distress and a small mandible. All managements and outcomes for the respiratory distress, as well as length of hospital stay, were recorded for each neonate.

Results:

There were 9 patients born at a single large institution over a three-year period that were classified as having Pierre Robin sequence. Four of these patients (44%) underwent mandibular distraction and the remaining five were managed conservatively without the need for a surgical airway. All four distractions were successful with regards to correction of micrognathia and glossoptosis, however, two infants who underwent mandibular distraction required a post-distraction tracheotomy for unrelated airway problems.

Conclusions:

Whereas the utility of neonatal/perinatal mandibular distraction osteogenesis for PRS has been established, the frequency with which such intervention is required is unreported. Based on this review of all infants with this condition born in a high volume labor and delivery facility over a three year period, 44% will require mandibular distraction in an attempt to avoid a tracheostomy. Whether distraction will be successful in this avoidance depends on multiple factors, such as, the length of time of intubation prior to and following distraction, comorbidities, surgical technique and other factors.