Predictors of NIV-related adverse events in children using long-term noninvasive ventilation.

BACKGROUND AND OBJECTIVES: An increasing number of children with diverse medical conditions are using long-term noninvasive ventilation (NIV). This study examined the impact of demographic, clinical, and technology-related factors on long-term NIV adverse events in a large cohort of children using long-term NIV. METHODS: This was a multicenter retrospective review of all children who initiated long-term NIV in the province of Alberta, Canada, from January 2005 to September 2014, and followed until December 2015. Inclusion criteria were children who had used NIV for 3 months or more and had at least one follow-up visit with the NIV programs. RESULTS: We identified 507 children who initiated NIV at a median age of 7.5 (interquartile range: 8.6) years and 93% of them reported at least one NIV-related adverse event during the initial follow-up visit. Skin injury (20%) and unintentional air leaks (19%) were reported more frequently at the initial visit. Gastrointestinal symptoms, midface hypoplasia, increased drooling, aspiration and pneumothorax were rarely reported (<5%). Younger age and underlying conditions such as Down syndrome, achondroplasia, and Duchenne muscular dystrophy were early predictors of unintentional air leak. Younger age also predicted child sleep disruption in the short term and ongoing parental sleep disruption. Obesity was a risk factor for persistent nasal symptoms. Mask type was not a significant predictor for NIV-related short- or long-term complications. CONCLUSIONS: This study demonstrates that NIV-related complications are frequent. Appropriate mask-fitting and headgear adaptation, and a proactive approach to early detection may help to reduce adverse events.

Acceptance of and adherence with long-term positive airway pressure treatment in adults with chronic obstructive pulmonary disease: A systematic review protocol.

BACKGROUND: Long-term noninvasive positive airway pressure (PAP) treatment is effective treatment for sleep-related breathing disorders and chronic hypercarbic respiratory failure secondary to chronic obstructive pulmonary disease (COPD). PAP treatment may be delivered as continuous positive airway pressure or noninvasive ventilation. Success in initiating PAP treatment and barriers to its use in adult patients with COPD are largely unknown. This systematic review aims to identify the acceptance of and adherence to PAP treatment prescribed for long-term use in adult patients with COPD and to summarize variables associated with these measures. METHODS: Seven online electronic databases will be searched by an experienced medical librarian to identify records containing the concepts "obstructive airways disease" and "noninvasive positive airway pressure" and "acceptance" or "adherence". Randomized and non-randomized studies of interventions will be included. Citation lists from relevant articles will be reviewed, and experts will be contacted regarding unpublished studies. Abstracts from key conferences between 2018-2023 and Google Scholar search results will be reviewed for inclusion. Titles, abstracts and full texts will be reviewed independently for inclusion by two reviewers. Data extraction will be completed by one author using a pre-established form and primary outcomes confirmed by a second author. Methodological quality will be evaluated. If sufficient data are available for meta-analysis, a pooled summary statistic for the primary outcome will be calculated using a random-effects generic inverse-variance meta-analysis, weighted proportion or weighted medians-based approach. Subgroup analysis will explore clinically meaningful sources of heterogeneity. Variables that are associated with acceptance and adherence will be described. DISCUSSION: Long-term PAP treatment is a complex intervention prescribed to patients with COPD for several indications. Synthesis of the evidence on success with PAP treatment and variables associated with acceptance or adherence will inform program and policy development for supporting patients with COPD who are prescribed this therapy. TRIAL REGISTRATION: Systematic review registration: This protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO) on July 13, 2021 (registration number CRD42021259262), with revisions submitted on April 17, 2023.

Characterization of craniofacial-based clinical phenotypes in children with suspected obstructive sleep apnea.

STUDY OBJECTIVES: We conducted this study to identify phenotypes of obstructive sleep apnea (OSA) in children based on lifestyle, sleep habits, age, obesity, sex, soft tissue facial features, and specific craniofacial abnormalities. METHODS: Seventy-three children with symptoms of pediatric OSA who underwent overnight observed polysomnography participated in this study. Soft tissue facial features were assessed using a 3-dimensional stereophotogrammetric system. Craniofacial abnormalities were evaluated based on the most common facial features associated with orthodontic treatment needs. Data regarding lifestyle, sleep habits, age, obesity, and sex were also collected. To identify phenotypes of OSA, a sequential analysis was then performed on categories of variables using fuzzy clustering with medoids. RESULTS: Craniofacial abnormalities and soft tissue facial features defined clusters. Three clusters were identified. Cluster 1 comprised a group of younger children (5.9 ± 3.8 years) without obesity, without craniofacial abnormalities, and with smaller soft tissue facial features dimensions. Cluster 2 comprised a group of older children (9.6 ± 3.9 years) without obesity and with larger mandibular dimensions and mildly arched palates (71.4%). Cluster 3 comprised a group of older children (9.2 ± 3.9 years) with obesity and a history of health issues (68.4%), excessive lower facial height (63.2%), and midface deficiency (73.7%). No differences were observed across clusters regarding sleep features. A moderate severity of obstructive and mixed respiratory events was observed in all 3 clusters. CONCLUSIONS: The study results did not identify distinct phenotypes of pediatric OSA based on soft tissue facial features or craniofacial abnormalities alone. Age and body mass index likely modify the effect of soft tissue facial features and craniofacial abnormalities as risk factors for OSA in children. CITATION: Fernandes Fagundes NC, Loliencar P, MacLean JE, Flores-Mir C, Heo G. Characterization of craniofacial-based clinical phenotypes in children with suspected obstructive sleep apnea. J Clin Sleep Med. 2023;19(11):1857-1865.

Long-term non-invasive ventilation in children: Transition from hospital to home.

Long-term non-invasive ventilation (NIV) is an accepted therapy for sleep-related respiratory disorders and respiratory insufficiency or failure. Increase in the use of long-term NIV may, in part, be driven by an increase in the number of children surviving critical illness with comorbidities. As a result, some children start on long-term NIV as part of transitioning from hospital to home. NIV may be used in acute illness to avoid intubation, facilitate extubation or support tracheostomy decannulation, and to avoid the need for a tracheostomy for long-term invasive ventilation. The decision about whether long-term NIV is appropriate for an individual child and their family needs to be made with care. Preparing for transition from the hospital to home involves understanding how NIV equipment is obtained and set-up, education and training for parents/caregivers, and arranging a plan for clinical follow-up. While planning for these transitions is challenging, the goals of a shorter time in hospital and a child living well at home with their family are important.

Behavioral and emotional characteristics of Canadian children with obesity and moderate-severe sleep-disordered breathing treated with positive airway pressure: longitudinal changes and associations with adherence.

STUDY OBJECTIVES: Behavioral characteristics and outcomes of positive airway pressure (PAP) therapy in children with obesity and moderate-severe sleep-disordered breathing (SDB) have not been reported. Our aims were to 1) determine baseline behavioral/emotional symptoms of this population and characterize changes over time with PAP, and 2) examine associations between baseline behavioral/emotional symptoms and PAP adherence. METHODS: This multicenter prospective cohort study of children with obesity prescribed PAP for moderate-severe SDB assessed PAP adherence (≥ 4 h/night, >50% of nights, usage diaries, downloads) and compared behavioral/emotional characteristics with parent- and child-reported Conners Rating Scale (Conners) and the Child Behavior Checklist (CBCL) at baseline and 1 year after PAP prescription between adherent and nonadherent participants; scores at baseline were compared retrospectively between adherence groups. RESULTS: Twenty-four children were included (median 14.1 years [IQR:12.4,16.0]; 87.5% males). Baseline Conners and CBCL scores were elevated (parent- and child-reported Conners inattention and hyperactivity subscales and CBCL subscales [total, internalizing, externalizing]). Baseline parent-reported Conners scores were significantly more elevated in the nonadherent than adherent group (inattention: 73.3 ± 8.5 vs 60.5 ± 14.6, P = .01; hyperactivity: 70.9 ± 11.1 vs 59.1 ± 16.0, P = .05). This difference was present 1 year later for inattention (P = .01) but not for hyperactivity (P = .09). Parent-reported CBCL scores improved over 1 year in adherent but not nonadherent participants. CONCLUSIONS: We found that children with obesity and moderate-severe SDB have elevated symptoms of behavioral/emotional concerns on standardized testing. Parent-reported emotional characteristics improved in the adherent but not in the nonadherent group. Children with greater inattention/hyperactivity at baseline were less adherent to PAP, suggesting this may contribute to PAP nonadherence. CITATION: Constantin E, MacLean JE, Barrowman N, et al. Behavioral and emotional characteristics of Canadian children with obesity and moderate-severe sleep-disordered breathing treated with positive airway pressure: longitudinal changes and associations with adherence. J Clin Sleep Med. 2023;19(3):555-562.

Temporal and Regional Trends in Obstructive Sleep Apnea Using Administrative Health Data in Alberta, Canada.

Rationale: Obstructive sleep apnea (OSA) is a common treatable condition with important health and societal consequences. Objectives: We aimed to assess the annual incidence and prevalence of clinically recognized and geographic clustering of OSA in Alberta, Canada, using administrative health data case definitions. Methods: We used two administrative health databases in Alberta to identify ICD-9 and ICD-10 (International Classification of Diseases, Ninth and 10th Revisions, respectively) diagnostic codes for adults and children at least 2 years old diagnosed with OSA between 2003 and 2020. We defined OSA using an algorithm developed and validated in Alberta: at least three claims or one hospitalization within 2 years. We mapped residential postal codes to 70 subregional health authorities (SRHAs). Crude, age group- and sex-specific incidence and prevalence, and age group- and sex-standardized rates were calculated for Alberta and SRHAs. Spatial scan statistics identified clusters of SRHAs in which OSA cases were higher (hot spots) or lower (cold spots) than expected. Results: Between 2003 and 2020, OSA prevalence increased from 0.14% to 4.59%. The annual incidence of OSA increased after 2013. Incidence and prevalence were higher in older adults and children aged 2-11 years compared with 12-17 years. Cluster analysis revealed regional variation in OSA incidence and prevalence over time with no consistent pattern except for cold spots in one large metropolitan center (Calgary). Conclusions: From 2003 to 2020, the incidence and prevalence of clinically recognized OSA increased but varied by geography. Administrative health data can be used to guide interventions aimed at improving health service delivery and the quality of OSA care.

Sleep in children and young adults with cystic fibrosis.

Large gains have been made in the management of respiratory diseases associated with cystic fibrosis (CF). Initial studies evaluating sleep issues in CF focused on respiratory problems of nocturnal hypoxia, alveolar hypoventilation and risk of airway obstruction from nasal polyps with treatment evaluations including long term oxygen therapy or noninvasive ventilation in case of nocturnal hypercapnia. More recent studies include patients whose lung function is better preserved, and have permitted more focus on sleep patterns and sleep quality. This literature identified that reduced sleep duration and poor sleep quality are common and may be explained by chronic pain and cough, frequent stools, gastro-oesophageal reflux, nasal obstruction or sinusitis, and drugs such as corticosteroids or beta-agonists. In the teenage years, poor sleep hygiene, sleep debt and poor sleep quality are associated with depression, poor academic performance, less physical activity, and a decrease in quality of life. Restless leg syndrome also seems to be common in adult patients with CF. These sleep problems seem more important in patients with a low lung function but may also be observed in patients with preserved lung function. The consequences of poor sleep may potentially exaggerate the multi-organ morbidity of CF, such as pain, inflammation, susceptibility to infection, and glucose intolerance, but these aspects are largely under-evaluated. Sleep should be evaluated on a routine basis in CF and prospective studies assessing the benefits of interventions aiming at improving sleep duration and sleep quality urgently needed.

Preserved Sleep for the Same Level of Respiratory Disturbance in Children with Prader-Willi Syndrome.

Debate remains as to how to balance the use of recombinant human growth hormone (rhGH) as an important treatment in Prader-Willi syndrome (PWS) with its potential role in obstructive sleep apnea. This single-center, retrospective study assessed differences in overnight polysomnography results between children with and without PWS and changes in respiratory parameters before and after the initiation of rhGH treatment in those with PWS. Compared with age-, sex-, and body-mass-index-matched controls (n = 87), children with PWS (n = 29) had longer total sleep time (434 ± 72 vs. 365 ± 116 min; p < 0.01), higher sleep efficiency (86 ± 7 vs. 78 ± 15%; p < 0.05), and lower arousal events (8.1 ± 4.5 vs. 13.0 ± 8.9 events/h; p < 0.05). Mean oxygen saturation was lower in PWS children (94.3 ± 6.0 vs. 96.0 ± 2.0%; p < 0.05), with no other differences in respiratory parameters between groups. Eleven children with PWS (38%) met the criteria for further analyses of the impact of rhGH; polysomnography parameters did not change with treatment. Compared with other children undergoing polysomnography, children with PWS had more favorable markers of sleep continuity and lower oxygen saturation for the same level of respiratory disturbance. rhGH administration was not associated with changes in respiratory parameters in PWS.

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

Context: Children 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. Objective: To 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 Sources: The 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 Selection: The 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 Extraction: Study characteristics, subject characteristics, technology type, and outcome measurements were extracted. Results: A 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. Limitations: Articles related to long-term non-invasive ventilation use in adolescents and young adults may have been excluded. Conclusions: Children 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 Registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=206533, identifier: CRD206533.

What are the respiratory health research priorities in Alberta, Canada? A stakeholder consultation.

OBJECTIVE: The Respiratory Health Strategic Clinical Network (RHSCN) was launched to facilitate respiratory and sleep health through implementation of innovative, patient-centred, evidence-informed coordinated services in Alberta. In collaboration with project partners, the RHSCN aimed to determine the respiratory research priorities for Alberta. DESIGN: The four phases of this research prioritisation project were (1) identifying research questions from stakeholders, (2) determining which research questions had been answered in existing literature, (3) prioritising unanswered questions and (4) finalising the priorities through an inperson workshop. SETTING: The study occurred in Alberta, Canada over a 2-year period beginning in March 2017. PARTICIPANTS: A total of 448 patients, clinicians and other stakeholders consented to participate in the survey. RESULTS: A total of 595 possible questions were submitted, with 343 unique questions identified. Of the questions, 94 were out of scope, 155 answered by existing literature and 10 were combined with others, while 83 were determined to be unanswered in the literature. Stakeholders were surveyed again to prioritise the remaining 83 questions and they were reviewed by the project's Steering Committee (clinicians and patients). At the inperson workshop, the Steering Committee identified 17 research topics as priority areas for respiratory and sleep research in Alberta. CONCLUSION: A stakeholder-led research prioritisation process identified optimal clinical management/follow-up, equitable access to services, and management of social, psychological and mental health issues related to respiratory/sleep health as priority research areas.

Feasibility of split night polysomnography in children to diagnose and treat sleep related breathing disorders.

STUDY OBJECTIVES: The gold standard test for diagnosis of sleep related breathing disorders (SRBD) in children is diagnostic polysomnography (PSG). This is often followed by a titration PSG to identify optimal non-invasive ventilation (NIV) pressures. Access to pediatric PSG is limited, resulting in delays to diagnosis and initiation of treatment. Split-night PSGs (snPSG) combine a diagnostic and titration PSG into a single night study. Although described in adults, the pediatric literature on this topic is sparse. The objective of this study was to describe a large cohort of children who utilized snPSG to diagnose SRBD and initiate NIV. METHODS: This multi-center study analyzed clinical and PSG data from children with SRBD who had initiated NIV following a snPSG. Data from diagnostic and titration portions of the snPSG were analyzed separately. RESULTS: The study included 165 children who initiated NIV following a snPSG. The majority of children (61.8%) were initiated on NIV for upper airway obstruction. The population included children with medical complexity, including those with central nervous system disorders (17.0%), musculoskeletal/neuromuscular disorders (12.1%), and cardiac disorders (1.2%). Moderate to severe SRBD was present in 87.2% of children with a median apnea-hypopnea index (AHI) of 16.6 events/hour (IQR: 8.2, 38.2). The median AHI was reduced on treatment to 7.6 events/hour (IQR: 3.3, 17.1), with fewer subjects meeting criteria for severe SRBD. CONCLUSIONS: snPSG is technically feasible in children, facilitating the diagnosis of SRBD and initiation of NIV, even in those with high medical complexity.

Blood pressure variability in children with obesity and sleep-disordered breathing following positive airway pressure treatment.

Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve sleep and cardiovascular status. While improvements in sleep parameters have been confirmed, a study by Katz et al. showed no major improvement in ambulatory blood pressure. The aim of this ancillary study was to analyze short-term blood pressure variability, following positive airway pressure treatment, as a more sensitive marker of cardiovascular health. We analyzed 24-h blood pressure variability data in 17 children, taken at baseline and after 12 months of treatment. These data were derived from an already published prospective, multicenter cohort study conducted in 27 youth (8-16 years) with obesity who were prescribed 1-year of positive airway pressure for moderate-severe sleep-disordered breathing. Significant decreases were found in 24 h systolic blood pressure (p = 0.040) and nighttime diastolic blood pressure (p = 0.041) average real variability, and diastolic blood pressure (p = 0.035) weighted standard deviation. Significant decreases were noted in nighttime diastolic blood pressure time rate variability (p = 0.007). Positive airway pressure treatment resulted in a significant decrease in blood pressure variability, suggesting a clinically significant improvement of sympathetic nerve activity in youth with obesity and sleep-disordered breathing. IMPACT: Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults.

Outcomes of Long-Term Noninvasive Ventilation Use in Children with Neuromuscular Disease: Systematic Review and Meta-Analysis.

Objectives: To determine whether children with neuromuscular disorders using long-term noninvasive ventilation (NIV), continuous or bilevel positive airway pressure, have improved health outcomes compared with alternative treatment strategies. Data Sources: This systematic review is an extension of a scoping review. The search strategy used Medical Subject Headings and free-text terms for "child" and "noninvasive ventilation." Studies of humans from 1990 onward were searched in MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed. The results were reviewed for articles reporting on neuromuscular disorders and health outcomes including mortality, hospitalization, quality of life, lung function, sleep study parameters, and healthcare costs. Data Extraction: Extracted data included study design, study duration, sample size, age, type of NIV, follow-up period, primary disease, and primary and secondary outcome measures. Studies were grouped by primary disease into three groups: spinal muscular atrophy, Duchenne muscular dystrophy, and other/multiple neuromuscular diseases. Data Synthesis: A total of 50 articles including 1,412 children across 36 different neuromuscular disorders are included in the review. Mortality is lower for children using long-term NIV compared with supportive care across all neuromuscular disease types. Overall, mortality does not differ when comparing the use of NIV with invasive mechanical ventilation, though heterogeneity suggests that mortality with NIV is higher for spinal muscular atrophy type 1 and lower for other/multiple neuromuscular diseases. The impact of long-term NIV on hospitalization rate differed by neuromuscular disease type with lower rates compared with supportive care but higher rates compared with supportive care use for spinal muscular atrophy type 1, and lower rates compared with before NIV for other/multiple neuromuscular diseases. Overall, lung function was unaltered and sleep study parameters were improved from baseline by long-term NIV use. There are few data to assess the impact of long-term NIV use on quality of life and healthcare costs. Conclusions: Long-term NIV for children provides benefit for mortality, hospitalizations, and sleep study parameters for some sub-groups of children with neuromuscular disorders. High risk of bias and low study quality preclude strong conclusions.

Expiratory activity during sleep in children.

Sleep irregularities and respiratory events (apnea, O2 desaturation or a combination thereof) are often present in the infant population. While inspiration is the main active process in the act of breathing, expiration is generally thought to occur passively. Although commonly considered as quiet during sleep, expiratory abdominal muscles have been proposed to be recruited to promote ventilation, facilitate gas exchange, and reduce the work of breathing during conditions of increased respiratory drive, exercise, or airway obstruction. In this study, we investigated the occurrence of expiratory abdominal muscle activity in polysomnographic studies of subjects (aged 0-2 years) suspected of sleep disordered breathing. Our results indicate that abdominal muscle activation occurs during sleep, most frequently during non-rapid eye movement and rapid-eye movement states compared to slow-wave sleep. Furthermore, abdominal muscle activity was present during regular breathing or associated with respiratory events (apneas or O2 desaturation). In the latter case, abdominal muscle recruitment more frequently followed the onset of respiratory events and terminated with recovery from blood O2 desaturation events. We conclude that expiratory abdominal muscle activity contributes to the pattern of respiratory muscle recruitment during sleep in infants and given its temporal relationship with respiratory events, we propose that its recruitment could facilitate proper ventilation by counteracting airway resistance and O2 desaturation in infancy across different stages of sleep.

Comparison of airway pressures and expired gas washout for nasal high flow versus CPAP in child airway replicas.

BACKGROUND: For children and adults, the standard treatment for obstructive sleep apnea is the delivery of continuous positive airway pressure (CPAP). Though effective, CPAP masks can be uncomfortable to patients, contributing to adherence concerns. Recently, nasal high flow (NHF) therapy has been investigated as an alternative, especially in CPAP-intolerant children. The present study aimed to compare and contrast the positive airway pressures and expired gas washout generated by NHF versus CPAP in child nasal airway replicas. METHODS: NHF therapy was investigated at a flow rate of 20 L/min and compared to CPAP at 5 cmH2O and 10 cmH2O for 10 nasal airway replicas, built from computed tomography scans of children aged 4-8 years. NHF was delivered with three different high flow nasal cannula models provided by the same manufacturer, and CPAP was delivered with a sealed nasal mask. Tidal breathing through each replica was imposed using a lung simulator, and airway pressure at the trachea was recorded over time. For expired gas washout measurements, carbon dioxide was injected at the lung simulator, and end-tidal carbon dioxide (EtCO2) was measured at the trachea. Changes in EtCO2 compared to baseline values (no intervention) were assessed. RESULTS: NHF therapy generated an average positive end-expiratory pressure (PEEP) of 5.17 ± 2.09 cmH2O (mean ± SD, n = 10), similar to PEEP of 4.95 ± 0.03 cmH2O generated by nominally 5 cmH2O CPAP. Variation in tracheal pressure was higher between airway replicas for NHF compared to CPAP. EtCO2 decreased from baseline during administration of NHF, whereas it increased during CPAP. No statistical difference in tracheal pressure nor EtCO2 was found between the three high flow nasal cannulas. CONCLUSION: In child airway replicas, NHF at 20 L/min generated average PEEP similar to CPAP at 5 cm H2O. Variation in tracheal pressure was higher between airway replicas for NHF than for CPAP. The delivery of NHF yielded expired gas washout, whereas CPAP impeded expired gas washout due to the increased dead space of the sealed mask.

Objective measurements for upper airway obstruction in infants with Robin sequence: what are we measuring? A systematic review.

STUDY OBJECTIVES: Identifying optimal treatment for infants with Robin sequence (RS) is challenging due to substantial variability in the presentation of upper airway obstruction (UAO) in this population. Objective assessments of UAO and treatments are not standardized. A systematic review of objective measures of UAO was conducted as a step toward evidence-based clinical decision-making for RS. METHODS: A literature search was performed in the PubMed and Embase databases (1990-2020) following PRISMA guidelines. Articles reporting on RS and UAO treatment were included if the following objective measures were studied: oximetry, polysomnography, and blood gas. Quality was appraised by the methodological index for nonrandomized studies (range: 0-24). RESULTS: A total of 91 articles met the inclusion criteria. The mean methodological index for nonrandomized studies score was 7.1 (range: 3-14). Polysomnography was most frequently used (76%) followed by oximetry (20%) and blood gas (11%). Sleep position of the infant was reported in 35% of studies, with supine position most frequently, and monitoring time in 42%, including overnight recordings, in more than half. Of 71 studies that evaluated UAO interventions, the majority used polysomnography (90%), of which 61% did not specify the polysomnography technique. Reported polysomnography metrics included oxygen saturation (61%), apnea-hypopnea index (52%), carbon dioxide levels (31%), obstructive apnea-hypopnea index (27%), and oxygen desaturation index (16%). Only 42 studies reported indications for UAO intervention, with oximetry and polysomnography thresholds used equally (both 40%). In total, 34 distinct indications for treatment were identified. CONCLUSIONS: This systematic review demonstrates a lack of standardization, interpretation, and reporting of assessment and treatment indications for UAO in RS. An international, multidisciplinary consensus protocol is needed to guide clinicians on optimal UAO assessment in RS. CITATION: Logjes RJH, MacLean JE, de Cort NW, et al. Objective measurements for upper airway obstruction in infants with Robin sequence: what are we measuring? A systematic review. J Clin Sleep Med. 2021;17(8):1717-1729.

Feasibility of three-dimensional facial imaging and printing for producing customised nasal masks for continuous positive airway pressure.

RATIONALE: Delivery of continuous positive airway pressure (CPAP) is the standard treatment for obstructive sleep apnoea in children and adults. Treatment adherence is a major challenge, as many patients find the CPAP mask uncomfortable. The study aim was to demonstrate the feasibility of delivered CPAP through customised nasal masks by assessing mask leak and comfort of customised masks compared to commercially available CPAP masks. METHODS: Six healthy adult volunteers participated in a crossover study including commercial masks in three different sizes (petite, small/medium and large) from the same supplier and a customised mask fabricated for each subject using three-dimensional facial scanning and modern additive manufacturing processes. Mask leak and comfort were assessed with varying CPAP levels and mask tightness. Leak was measured in real time using an inline low-resistance Pitot tube flow sensor, and each mask was ranked for comfort by the subjects. RESULTS: Mask leak rates varied directly with CPAP level and inversely with mask tightness. When ranked for comfort, three subjects favoured the customised mask, while three favoured a commercial mask. The petite mask yielded the highest mask leaks and was ranked least comfortable by all subjects. Relative mask leaks and comfort rankings for the other commercial and customised masks varied between individuals. Mask leak was comparable when comparing the customised masks with the highest ranked commercial masks. CONCLUSION: Customised masks successfully delivered target CPAP settings in all six subjects, demonstrating the feasibility of this approach.