0791 Polysomnographic Outcomes of Obstructive Sleep Apnea in Infants with Pierre-Robin Sequence

Abstract Introduction Children with Pierre-Robin sequence (PRS) are known to have a high association with sleep-disordered breathing (SDB). Mandibular distraction osteogenesis (MDO), a surgical process involving lengthening of the jaw, has been used to improve SDB in these patients, preventing the need for tracheostomy or positive airway pressure therapy. Currently, limited data regarding polysomnographic outcome of SDB after mandibular distraction is available. Therefore, we aimed to assess the prevalence of SDB in pediatric patients with PRS and the polysomnographic outcome of MDO. Methods This was a single-center retrospective study that included children with confirmed PRS who underwent polysomnography (PSG) for evaluation of SDB. Obstructive sleep apnea (OSA) severity was categorized by the obstructive apnea-hypopnea index (OAHI) as normal ( 1 events/hr), mild (1–4.9 events/h), moderate (5–9.9 events/h), and severe (≥ 10 events/h). Pre- and post-MDO PSG variables were compared using a paired t-test. Results Baseline PSG data were available for 16 children (8 males, 8 females) with mean age 374.2 days. Average BMI was 14.4 (±2.4 kg/m3) and 87.5% of patients were Caucasian. OSA prevalence was 87.5%, apnea hypopnea index (AHI) was severe in 68.8% and the mean AHI was 46.3 (± 43.8). Eleven patients had MDO with a mean age of 27.1 days (± 20.5) at time of MDO and distraction duration of 33.2 days (± 7.6). Six patients had pre and post MD PSGs. Average total sleep time (TST) increased significantly from 207.3 min (± 85.7 min) to 351.3 min (± 126.7 min); p=0.023. Average apnea-hypopnea index (AHI) and average REM AHI decreased significantly from 79.0 events/h (± 41.6 events/h) to 18.2 events/h (± 25.0 events/h), p=0.017; and 106.3 events/h (± 43.4 events/h) to 37.0 events/h (± 51.0 events/h), p=0.040, respectively. Average oxygen saturation nadir during sleep significantly improved from 72.0% (± 5.1%) to 85.2% (± 12.4%), p=0.030; and percentage of carbon-dioxide time spent above 50 mmHg improved significantly from 68.0% (± 17.7%) to 49.8% (± 6.0%) p=0.027. Conclusion Children born with PRS have a high prevalence of severe OSA. Additionally, MDO significantly improves TST, AHI, oxygen saturation nadir, and CO2 levels in children with SDB and PRS. Support (if any)