Sleep Outcomes in Neonates with Pierre robin sequence Undergoing External Mandibular Distraction: A Longitudinal Analysis.

BACKGROUND Pierre robin sequence (PRS) is a common cause of upper airway obstruction in newborns. Airway obstruction is often worse during sleep, leading to severe obstructive sleep apnea (OSA). Standardized management algorithms are lacking. Although mandibular distraction osteogenesis (MDO) is a frequently used surgical approach, literature regarding outcomes after treatment in early infancy is sparse. Herein we report sleep and respiratory parameters in a cohort of neonates undergoing external MDO. METHODS A retrospective,14-year, single-institution, study of neonates with PRS who underwent polysomnography before (PSGPRE) and after (PSGPOST) MDO.Data was analyzed to identify trends in respiratory parameters and sleep architecture pre vs. post-surgery. RESULTS Thirty-one neonates were included. Age at PSGPRE was 13(5,34) days and at PSGPOST was 80(50,98) days. All neonates had severe OSA at baseline (defined as PSGPRE obstructive apnea hypopnea index O-AHI≥10).Post-operatively, there was a significant reduction in O-AHI (PSGPRE 38.3[23.4,61.8] vs. PSGPOST 9.4[5.3,17.1][p<0.0001]),and a significant improvement in sleep efficiency (PSGPRE 62%[53,71] vs. PSGPOST73%[67,80][p=0.0022]) and nadir of oxygen saturation (PSGPRE 74%[68,82] vs. PSGPOST 83%[79,87][p=0.0001]). Although twenty-six neonates (84%) had a 50% reduction in O-AHI post-operatively, all neonates had obstructive sleep apnea and fifteen neonates (48%), had persistent severe OSA following surgery. CONCLUSIONS Our study represents the largest published cohort of sleep outcomes in neonates with PRS and severe OSA undergoing external MDO. Although these neonates have significant improvement in sleep outcomes following surgery, they are at risk of persistent OSA. We propose that neonates with PRS undergo PSG before and soon after MDO to objectively assess improvement in OSA as they may require additional evaluation for sites of multi-level airway obstruction and treatment.