Abstract

The use of long-term non-invasive ventilation (NIV) to treat sleep and breathing disorders in children has increased substantially in the last decade; however, less data exist about its use in infants. Given that infants have distinct sleep and breathing patterns when compared to older children, the outcomes of infants on long-term NIV may differ as well. The aim of this study is to systematically review the use and outcomes of long-term NIV in infants. Ovid Medline, Ovid Embase, CINAHL (via EbscoHOST), PubMed, and Wiley Cochrane Library were systematically searched from January 1990 to July 2017. Studies on infants using long-term NIV outside of an acute care setting were included. Data were extracted on study design, population characteristics, and NIV outcomes. A total of 327 studies were full-text reviewed, with final inclusion of 60. Studies were distributed across airway (40%), neuromuscular (28%), central nervous system (10%), cardio-respiratory (2%), and multiple (20%) disease categories. Of the 18 airway studies reporting on NIV outcomes, 13 (72%) reported improvements in respiratory parameters. Of the 12 neuromuscular studies exclusively on spinal muscular atrophy type 1 (SMA1), six (50%) reported decreased hospitalizations and nine (75%) reported on mortality outcomes. Risk of bias was moderate to serious, and quality of the evidence was low to very low for all studies. Most studies had an observational design with no control group, limiting the potential for a meta-analysis. The outcomes reported in studies differed by the disease category being studied. Studies on airway conditions showed improvements in respiratory parameters for infants using NIV. Studies on neuromuscular disorder, which were almost exclusively on SMA1, reported decreased hospitalizations and prolonged survival. Overall, it appears that NIV is an effective long-term therapy for infants. However, the high risk of bias and low quality of the available evidence limited strong conclusions.

Highlights

  • RationaleLong-term non-invasive ventilation (NIV), defined as respiratory support delivered through an interface outside the airway, has become the treatment of choice for a number of chronic conditions resulting in respiratory insufficiency or sleep and breathing disorders in infants and children [1,2,3]

  • We identified a number of research gaps present in the studies included within this review

  • Improvements in respiratory parameters and discontinuation from NIV due to improvement in underlying conditions have been shown for a broad range of upper airway disorders, such as obstructive sleep apnea (OSA), Pierre Robin sequence (PRS), and LTM, in infants

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Summary

Introduction

Long-term non-invasive ventilation (NIV), defined as respiratory support delivered through an interface outside the airway, has become the treatment of choice for a number of chronic conditions resulting in respiratory insufficiency or sleep and breathing disorders in infants and children [1,2,3]. These conditions include airway disorders, neuromuscular disorders (NMDs), and disorders of the central nervous system (CNS) [3,4,5,6]. Data were extracted on study design, population characteristics, and NIV outcomes

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