0793 Sleep Disordered Breathing in Children with Craniofacial Syndromes

Abstract Introduction Children with Craniofacial Syndrome (CFS) are known to have a higher risk for Obstructive Sleep Apnea (OSA) due to various craniofacial abnormalities. We aim to assess OSA prevalence in a single cohort of children suffering from CFS and to stratify them by degree of OSA (mild, moderate, severe) and the overall management of OSA in CFS. Methods This was a single-center retrospective study that included children with confirmed CFS who underwent overnight polysomnography (PSG). OSA severity was categorized by obstructive apnea-hypopnea index (OAHI) as normal (< 1 event/hr), mild (1–4.9 events/h), moderate (5–9.9 events/h), and severe (≥ 10 events/h). Results PSG data were available for 46 children; 58.7% were male, with a mean age of 4.8 years. The average BMI was 18.2 (SD + 5.4 kg/m2) with an average z-score of 1.1 (SD + 4.0), and 76.1% of patients were Caucasian. Common referring symptoms were snoring (38.1%), witnessed apneas (29.1%), gasping/choking (17.4%), and oxygen desaturation (15.1%). The most common CFS was Pierre Robin Sequence (PRS) in 34.8%. OSA prevalence was 68.2% (23.3% mild, 20% moderate, 56.7% severe). The mean polysomnographic variables were total sleep time 346.2 min (± 120.0), AHI 22.2 (± 32.4), SaO2 nadir 83.8% (± 12.1), maximum CO2 level 54 mmHg (± 9.9), and arousal index 22.2 (± 23.4). The overall treatments consisted of mandibular distraction in 26.5%, continuous positive airway pressure therapy in 12.2%, tracheostomy in 6.1%, oxygen supplement in 2%, and lip-tongue adhesion in 2%. Conclusion In our cohort of children with CFS had a high prevalence of OSA with severe degree as the prevalent severity. PRS was the most common CFS and mandibular distraction was the most commonly performed procedure in infants with PRS. Support (if any)