Long-Term Non-invasive Ventilation in Children With Down Syndrome: A Systematic Review

Abstract

ContextChildren with Down syndrome are at risk for obstructive sleep apnea, which may not be resolved by adenotonsillecotmy, as well as other respiratory disorders that may impact breathing during sleep. Long-term non-invasive ventilation, including continuous and bilevel positive airway pressure delivery, is an alternate treatment strategy.ObjectiveTo assess the use and outcomes of long-term non-invasive ventilation in children with Down syndrome including comparison to other children using long-term non-invasive ventilation.Data SourcesThe search strategy for the scoping review 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 databases were searched (1990-2021).Study SelectionThe scoping review results were searched to identify studies including data on at least three children with Down Syndrome using long-term non-invasive ventilation.Data ExtractionStudy characteristics, subject characteristics, technology type, and outcome measurements were extracted.ResultsA total of 28 articles included 543 children with Down syndrome using long-term non-invasive ventilation. Children with Down syndrome accounted for 18% of children using long-term non-invasive ventilation. Data on efficacy, feasibility, and adherence in children with Down syndrome are comparable to other children. Children with Down syndrome may have greater difficulty initiating long-term non-invasive ventilation, longer time to establish use, and a higher rate of inability to establish use. Outcome data is limited but suggest favorable impact on cardiac function and attention.LimitationsArticles related to long-term non-invasive ventilation use in adolescents and young adults may have been excluded.ConclusionsChildren with Down syndrome make up a significant portion of the population of children using long-term non-invasive ventilation. While there is more limited data available with respect to the use and outcomes for children with Down syndrome compared to the other children, long-term non-invasive ventilation is an effective and well-tolerated therapy with no clear differences in the use or outcomes for children with Down syndrome. Additional work is needed to understand potential challenges around establishing long-term non-invasive ventilation use in children with Down syndrome.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?RecordID=206533, identifier: CRD206533.

Comparison of non-invasive ventilation use and outcomes in children with Down syndrome and other children using this technology

“…Additional studies reported no difference in adherence in children with and without impaired cognition as well as higher rates of never establishing LT-NIV use in children with DS (O'Donnell et al, 2006;Amaddeo et al, 2018). Studies exclusively on children with DS support the potential for high adherence as well as adherence rates that are typical of children using LT-NIV (Hudson et al, 2022). The current study shows adherence rates are lower in children with DS though not out of keeping with rates reported in other cohorts of children using LT-NIV.…”

“…For children with DS, up to 55% go on to use LT-NIV (including continuous positive airway pressure, CPAP, and bilevel positive airway pressure, BPAP) after incomplete resolution of OSA after surgery (Shete et al, 2010;Dudoignon et al, 2017). In a systematic review of LT-NIV use in children with DS, children with DS made up 18% of children using LT-NIV across 20 studies (Hudson et al, 2022). This is in keeping with a population study of LT-NIV use in children that reported 18% of children using LT-NIV were children with DS (Castro-Codesal et al, 2018).…”

“…Given the inherent challenges for children with DS with respect to cognitive ability, behavioral concerns, obesity, and pulmonary hypertension that may be compounded by untreated OSA or sleeprelated hypoventilation, understanding the balance of burden vs. benefit of LT-NIV use for children with DS is important. LT-NIV is an effective and well-tolerated therapy with potential benefits such that consideration of its use should not differ for children with and without DS (Hudson et al, 2022). What is not known is whether LT-NIV use and outcomes differ between children with and without DS such that a different approach to LT-NIV use is needed for children with DS.…”

P2 Intravenous ferric carboxymaltose for the treatment of refractory restless leg syndrome

“…1 Adherence to long-term continuous or bilevel pressure support in this population can be difficult comparable to other children. 2 However, there is a paucity of longitudinal data examining this within this cohort.…”

“…1 Hannah Williams*, 1 Sakina Birdseye-Dastagir, 1 Alex Thomas, 1,2 Hui-Leng Tan, Introduction Obstructive sleep disordered breathing (SDB) is highly prevalent in children with Trisomy 21 (T21). 1 Adherence to long-term continuous or bilevel pressure support in this population can be difficult comparable to other children.…”