The prevalence of SRBD in DMD is significant. There is a bimodal presentation of SRBD, with OSA found in the first decade and hypoventilation more commonly seen at the beginning of the second decade. Polysomnography is recommended in children with symptoms of OSA, or at the stage of becoming wheelchair-bound. In patients with the early stages of respiratory failure, assessment with polysomnography-identified sleep hypoventilation and assisted in initiating NIV.

Surgical intervention improves sleep behaviour in children though not temperament or development.

This study suggests a relationship between OSA, though not its severity, and pre/perinatal adversity and child development. Polysomnographic and detailed developmental assessment of community-based samples of children with OSA and control children are necessary to confirm these findings.

Study Objectives: Continuous positive airway pressure (CPAP) has been increasingly used in children with obstructive sleep apnea (OSA), though it is unclear whether it can ever be ceased. We describe the clinical, demographic, and polysomnographic (PSG) characteristics of a cohort of children with OSA who were successfully weaned off CPAP. Methods: From a pediatric cohort on CPAP for OSA at the Queensland Children's Hospital between January 2016 and December 2017, a subgroup of children who were taken off CPAP were retrospectively studied. Results: CPAP therapy was stopped for 53 children over a 2-year period; 29 of these were excluded from analysis due to change to bilevel support (n = 2), transition to adult care (n = 12), or cessation due to poor adherence (n = 15). A total of 24 children [median (interquartile range, IQR) age 4.1 years (1.0-10.5); 18 males] were successfully weaned off CPAP therapy based on improvement in clinical and PSG parameters; and were included in the analysis. These children had a median (IQR) apnea-hypopnea index (AHI) of 9.8 (5.7-46.0) at CPAP initiation, which improved to 3.3 (0.4-2.2) at CPAP cessation after a median (IQR) duration of 1.0 (0.5-2.0) year. The reasons for CPAP cessation included improved symptoms and/or PSG parameters with time (n = 11); improvement after airway surgery (n = 7), and improvement of body mass index (n = 2). In four children, CPAP therapy was ceased after initial trial due to low physician perceived clinical benefit. Conclusions: This is the first study describing the characteristics of children and likely reasons for successful CPAP cessation. Children on CPAP should be regularly screened for ongoing CPAP need.

No abstract

No abstract

No abstract

2. Pre-operative PET scans play an undoubted role in selecting patients for surgery but thus far in our service, they don't appear to be associated with significantly less RD because of the problem of microscopic metastases.