Long-term non-invasive ventilation in children with Down syndrome: a systematic review

Introduction: Down Syndrome confers susceptibility to multiple sleep-related breathing disorders, for which long-term non-invasive ventilation (NIV) is a common treatment option. While there is a large body of evidence for the use of long-term NIV in the broader pediatric population, work specific to its use in children with Down syndrome is more limited. Understanding the benefits and challenges of long-term NIV use in this population is important for informing patient-centered care as well as fiscal health policy around equipment funding and family support. This study aims to systematically review the current state of evidence on this topic.

Methods: This systematic review is an extension of a scoping review. The search strategy used Medical Subject Headings (MeSH) and free-text terms for “child” and “non-invasive ventilation”. MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed were systematically searched for the period of 1990-2019. Included studies examined NIV use for at least three months in three or more children with Down syndrome.

Results: 21 articles met inclusion criteria and included 359 children with Down syndrome. Designs were mostly observational. Only 7 studies were undertaken exclusively in children with Down syndrome. Long-term NIV use was mainly used for the treatment of obstructive sleep apnea (OSA) and was frequently used for residual OSA following adenotonsillectomy and as a bridge to improvement in children who outgrew OSA with age. Satisfactory NIV adherence was documented in children with Down syndrome. Specific neuropsychological and cardiac benefits of long-term NIV use may be seen in this population, though data on outcomes specific to children with Down syndrome is scarce.

Conclusion: Children with Down syndrome can successfully use long-term NIV, however with no comparative studies, it is unclear whether they face more challenges with its use. With limited studies focusing on outcomes, the benefits of long-term NIV for children with Down syndrome remain difficult to define.

Keywords: non-invasive ventilation, continuous positive airway pressure, bilevel positive airway pressure, Down syndrome, obstructive sleep apnea