Non-invasive Ventilation In Children Research Articles

Mask interfaces and devices for home noninvasive ventilation in children.

Home noninvasive ventilation (NIV), including continuous (CPAP) and bilevel (BPAP) positive airway pressure, is increasingly used in children worldwide. In this narrative review, we present a comprehensive summary of the equipment available for home NIV in pediatrics, excluding neonates. NIV may be challenging in young children, as the majority of the equipment has been developed for adults. Regarding the interfaces, only a few masks have been specifically developed for young children in recent years, while older children may benefit from a large variety of interfaces. Even though much progress has been made, skin injuries are still present, and need to be managed rapidly. Several studies addressed the management of the side effects, but recent studies are lacking regarding orofacial anomalies. No recent study reported the available interfaces for young children and the strategies for an optimal mask fit. Regarding the devices, an adapted NIV device to pediatrics that allows an adequate patient's breathing detection should guarantee optimal ventilatory efficiency and monitoring of NIV. A close follow-up and regular monitoring should be mandatory to rule out the potential issues, optimize NIV therapy and ascertain the efficacy of NIV. However, studies are lacking to guide the choice of devices in young children and the optimal management of home NIV in pediatrics. We summarized the characteristics of the different interfaces available for young children and the limitations of NIV devices. We finally addressed potential areas for future research on long-term home NIV in children.

Development of personalized non-invasive ventilation masks for critically ill children: a bench study

BackgroundObtaining a properly fitting non-invasive ventilation (NIV) mask to treat acute respiratory failure is a major challenge, especially in young children and patients with craniofacial abnormalities. Personalization of NIV masks holds promise to improve pediatric NIV efficiency. As current customization methods are relatively time consuming, this study aimed to test the air leak and surface pressure performance of personalized oronasal face masks using 3D printed soft materials. Personalized masks of three different biocompatible materials (silicone and photopolymer resin) were developed and tested on three head models of young children with abnormal facial features during preclinical bench simulation of pediatric NIV. Air leak percentages and facial surface pressures were measured and compared for each mask.ResultsPersonalized NIV masks could be successfully produced in under 12 h in a semi-automated 3D production process. During NIV simulation, overall air leak performance and applied surface pressures were acceptable, with leak percentages under 30% and average surface pressure values mostly remaining under normal capillary pressure. There was a small advantage of the masks produced with soft photopolymer resin material.ConclusionThis first, proof-of-concept bench study simulating NIV in children with abnormal facial features, showed that it is possible to obtain biocompatible, personalized oronasal masks with acceptable air leak and facial surface pressure performance using a relatively short, and semi-automated production process. Further research into the clinical value and possibilities for application of personalized NIV masks in critically ill children is needed.

Effect of long term noninvasive ventilation in children on parent's quality of life.

Improving or maintaining the quality of life of the family of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) is a major concern; but studies are scarce. The aim of the study was to evaluate the impact of long term CPAP or NIV in children on anxiety, depression, quality of sleep, and quality of life of their parents. Validated questionnaires evaluating anxiety and depression (hospital anxiety and depression scale), sleep quality (Pittsburgh sleep quality index), daytime sleepiness (Epworth sleepiness scale), and parents' quality of life (PedsQL family impact module) were completed by parents of children who were started on CPAP/NIV before (M0) and after 6-9 months (M6) of treatment. The questionnaires of 36 parents (30 mothers, 6 fathers) of 31 children were analyzed. For the entire group, no significant change was observed in anxiety, depression, sleep quality, daytime sleepiness, and quality of life between M0 and M6. When analyzing questionnaire class changes between M0 and M6: anxiety was relieved in 23% of parents and worsened in 29%, depression was relieved in 14% and worsened in 20%, sleep quality improved in 43% and worsened in 27%, sleepiness improved in 26% and worsened in 17%, with no change in the other parents. Long term CPAP/NIV in children had no significant effect on parents' anxiety, depression, sleep quality, and quality of life.

Biphasic non-invasive lung ventilation in the neonatal intensive care unit: the choice of tactics for starting respiratory support in preterm infants

The article deals with modern clinical and pathogenetic features of the variants of the respiratory pattern of non-invasive lung ventilation with two phases of airway pressure in the neonatal intensive care unit. The epidemiology, pathogenesis and assessment of the severity of the clinical picture of the neonatal respiratory distress syndrome are presented. The anatomical and physiological features of cardiorespiratory adaptation and targeted indicators of complex monitoring of the vital functions of the body of premature babies during the initiation of the starting two-phase non-invasive ventilation of the lungs are discussed. Early predictors of failure and criteria for optimizing the clinical efficacy of initial biphasic non-invasive ventilation in children with extremely low birth weight are considered. The clinical and prognostic role and features of perinatal screening were assessed when choosing the tactics of starting respiratory support in premature infants in the neonatal intensive care unit.

Outpatient Utilization of the RAM Cannula for Nasal Noninvasive Ventilation in Children.

The RAM cannula® consists of nasal prongs that can be used to administer oxygen, continuous, and bilevel positive airway pressure therapies. Studies have reported the efficacy and utility of the RAM cannula in inpatients requiring noninvasive ventilation (NIV); however, there is limited literature on the use of the RAM cannula to provide NIV in the outpatient setting. This study aimed to describe the clinical features and outcomes of children who used NIV via RAM cannula in the outpatient setting. Retrospective review. We conducted a retrospective review of children treated with outpatient NIV via RAM cannula at our institution between January 2010 and March 2023. The analyzed data included age, diagnoses, indications for NIV, duration of RAM cannula use, complications, and outcomes at 6 months. We identified 20 patients who used outpatient NIV via RAM cannula during the study period. The median age at initiation of NIV via RAM cannula was 5.8 months (IQR 2.4-9.9 months). Indications for NIV included sleep-related hypoventilation (15%), restrictive lung disease (25%), obstructive sleep apnea (45%), and chronic respiratory failure (50%), with 6 patients having ⩾2 indications for NIV. RAM cannula was utilized for inability to tolerate conventional NIV interfaces (80%), to alleviate dyspnea (60%), and to avoid tracheostomy (55%). Patients used NIV via RAM cannula for a median duration of 7.7 months (IQR 3.7-20.6 months). Patient outcomes included ongoing usage of RAM cannula (55%), changing to conventional NIV interfaces (15%) or oxygen (10%), weaning off respiratory support (5%), and death (15%). There were no complications related to using the RAM cannula. Our study demonstrates the utility of outpatient NIV via RAM cannula in children with a variety of diagnoses until clinical improvement or tolerance of conventional interfaces, and for avoidance of tracheostomy.

Impact of COVID-19 pandemic on adherence to noninvasive ventilation in children.

The aim of this study was to measure changes in adherence to noninvasive ventilation (NIV) in children during the first year of the COVID-19 pandemic. Retrospective chart review of children (0-18 years) using home NIV through the Stollery's Pediatric NIV program in Edmonton, Canada during March 2019 to March 2021. Demographics, clinical characteristics, and adherence information from machine downloads were collected prior to and during the first year of the COVID-19 pandemic. Paired t tests and Chi-square compared adherence prepandemic and during pandemic and repeated analysis of variance tests compared adherence pre- and 0-6 and 6-12 months during pandemic. Eighty-two children met inclusion criteria (62% male; age 8.6 ± 4.6 years). Overall, there were no changes in NIV adherence during pandemic (average NIV minutes pre- and during pandemic of 390 ± 219 and 405 ± 300 minutes, respectively). When separated into increased vs decreased adherence groups, adherence differences pre- compared to during pandemic became significant, with no differences across demographic/clinical variables or prepandemic adherence. There were no changes in NIV adherence during the initial 6 months of pandemic compared to prepandemic, but NIV use significantly increased by 9-57 minutes during the following 6-12 months. Bilevel positive airway users had longer NIV use than continuous positive airway pressure users at all time points, with an overall increasing trend over time. Despite the significant life disruption created by COVID-19 and changes to virtual care, children using home NIV maintained adequate adherence. Qualitative research is needed to understand the nuances of using NIV during the pandemic and potential advantages of virtual care for support of these children and families. Halperin H, Chalifour M, Bedi PK, etal. Impact of COVID-19 pandemic on adherence to noninvasive ventilation in children. J Clin Sleep Med. 2023;19(1):179-188.

Noninvasive ventilation in children: A review for the pediatric anesthesiologist.

Preserving adequate respiratory function is essential in the perioperative period. Mechanical ventilation with endotracheal intubation is widely used for this purpose. In select patients, noninvasive ventilation (NIV) may be an alternative to invasive ventilation or may complement respiratory management. NIV is used to provide ventilatory support and increase gas exchange at the alveolar level without the use of an invasive artificial airway such as an endotracheal tube or tracheostomy. NIV includes both continuous positive airway pressure (CPAP) and noninvasive positive pressure ventilation. Indications for NIV range from acute hypoxic respiratory failure in the intensive care unit or the emergency department, to chronic respiratory failure in patients with neuromuscular disease with nocturnal hypoventilation. In the perioperative setting, NIV is commonly applied as CPAP, and bilevel positive airway pressure (BPAP). There are limited data on the role of NIV in children in the perioperative setting, and there are no clear guidelines regarding optimal timing of use and pressure settings of perioperative NIV. Contraindications to the use of NIV include reduced level of consciousness, apnea, severe respiratory distress, and inability to maintain upper airway patency or airway protective reflexes. Common problems encountered during NIV involve airway leaks and asynchrony with auto-triggering. High-flow nasal oxygen (HFNO) has emerged as an alternative to NIV when trying to decrease the work of breathing and improve oxygenation in children. HFNO delivers humidified and heated oxygen at rates between 2 and 70L/min using specific nasal cannulas, and flows are determined by the patient's weight and clinical needs. HFNO can be useful as a method for preoxygenation in infants and children by prolonging apnea time before desaturation, yet in children with decreased minute ventilation or apnea HFNO does not improve alveolar gas exchange. Clinicians experienced with these devices, such as pediatric intensivists and pulmonary medicine specialists, can be useful resources for the pediatric anesthesiologist caring for complex patients on NIV.

Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences?

Individuals with Duchenne muscular dystrophy (DMD) frequently develop sleep-disordered breathing. Noninvasive ventilation is often prescribed for sleep-disordered breathing treatment based on the American Academy of Sleep Medicine (AASM) criteria. In 2018, DMD disease-specific criteria for sleep-disordered breathing were established. Our study aimed to examine the clinical interpretation differences using these different criteria. We performed a multicenter, retrospective chart review of children with DMD followed at The Hospital for Sick Children, Toronto, Canada, and Rady Children's Hospital, San Diego, California, who underwent polysomnography from August 1, 2012, to February 29, 2020. Baseline characteristics and polysomnography data were summarized using descriptive statistics. Agreement for the diagnosis of sleep-disordered breathing evaluated by kappa statistics and sensitivity/specificity analysis was assessed. One hundred five male children with DMD (mean ± SD age: 12.1 ± 3.8 years; body mass index z score: 0.2 ± 2.3) were included. The proportions of children with DMD that met at least 1 AASM criterion and at least 1 DMD criterion were 45.7% and 67.6%, respectively. We found that 32.4% of children met neither AASM nor DMD criteria. Overall agreement between AASM and DMD criteria was moderate (k = 0.57). There was almost perfect agreement in sleep apnea diagnosis (k = 0.90); however, there was only slight agreement in hypoventilation diagnosis (k = 0.12) between AASM and DMD criteria. There were more children with DMD diagnosed with nocturnal hypoventilation and prescribed noninvasive ventilation using DMD criteria compared with AASM criteria. Future studies should address whether the prescription of noninvasive ventilation for children with DMD based on both criteria is associated with different clinical outcomes. Hurvitz MS, Sunkonkit K, Massicotte C, Li R, Bhattacharjee R, Amin R. Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences? J Clin Sleep Med. 2022;18(2):609-615.

Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that leads to chronic respiratory insufficiency and failure. Use of home noninvasive ventilation (NIV) has been linked to improved outcomes including reduced mortality. Despite the importance of NIV, factors promoting optimal NIV usage and determinants of nonadherence have not been rigorously examined. Moreover, given that respiratory issues in DMD span between childhood and adulthood, examination across a broad age group is needed. The objectives of this study were to (1) evaluate NIV usage across a broad spectrum of patients with DMD, including both children and adults, and (2) identify biological and socioeconomic determinants of NIV usage and NIV nonadherence. We performed a retrospective review of all patients with DMD from February 2016 to February 2020 who underwent evaluation at associated pediatric and adult neuromuscular disease clinics. NIV use was determined objectively from device downloads. A priori, we defined nonadherence as < 4 hours use per night, quantified as the percentage of nights below this threshold across a 30-day period within 6 months of a clinic visit. We also assessed the average hours of NIV usage over this time period. Predictors examined included demographics, social determinants, and pulmonary function. 33 patients with DMD were identified, 29 (87%) of whom were using NIV (13 age < 21 years). Mean age was 22.9 ± 6.6 years (range 13-39 years), body mass index was 23.4 ± 10.4 kg/m2, and seated forced vital capacity was 23% ± 18% predicted. Mean nightly NIV usage was 7.4 ± 3.8 hours and mean percentage of nonadherent nights was 13% ± 30%. In univariable analysis, age did not predict use. Those with lower forced vital capacity had higher NIV usage hours (P = .01) and a trend toward less nonadherence (P = .06). Higher estimated household income demonstrated a trend toward increased usage hours and less nonadherence (both P = .08). Multivariable analysis found increased usage hours were predicted best by higher income, higher inspiratory positive airway pressure, and higher bicarbonate. Nonadherence was higher in those with lower income or higher forced vital capacity. In this cohort of adult and pediatric patients with DMD, most individuals were using NIV. While usage hours were higher with lower lung function, substantial variability remains unexplained by examined factors. Nonadherence was observed in some individuals, including those with advanced disease. Further investigations should focus on evaluating patient-oriented outcomes to define optimal NIV usage across the spectrum of disease and determine strategies to counteract issues with nonadherence. Hurvitz MS, Bhattacharjee R, Lesser DJ, Skalsky AJ, Orr JE. Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy. J Clin Sleep Med. 2021;17(10):1973-1980.

Average volume-assured pressure support vs conventional bilevel pressure support in pediatric nocturnal hypoventilation: a case series.

Average volume-assured pressure support (AVAPS) is a modality of noninvasive ventilation that provides a targeted tidal volume by automatically adjusting the inspiratory pressure support within a set range. Pediatric studies evaluating the efficacy of AVAPS in treating nocturnal hypoventilation are confined to case reports. The aim of this study was to compare AVAPS to conventional bilevel positive airway pressure (BPAP) support in improving hypercarbia in a cohort of pediatric patients with nocturnal hypoventilation. Retrospective review of patient records at an established tertiary pediatric sleep laboratory over a 6-year period. Ventilatory and sleep study parameters from AVAPS and conventional BPAP titration studies were compared. AVAPS was used only if hypoventilation was not controlled using conventional BPAP. Inspiratory pressures, tidal volumes, and adherence were downloaded on final titrated ventilatory settings. Comparisons were made using paired t test. A total of 19 patients (11 boys, 8 girls; median age 10.5 years, range 1 to 20 years) were identified. Diagnoses included neuromuscular disease (n = 9), obstructive hypoventilation (n = 5), parenchymal lung disease (n = 4), and congenital central hypoventilation syndrome (n = 2). AVAPS demonstrated significant improvement in peak (P = .009) and mean (P = .001). Transcutaneous CO₂ parameters compared to conventional bilevel. Oxygenation on AVAPS showed positive trend but did not reach statistical significance. AVAPS delivered higher tidal volumes (P = .04) using similar pressures. There was no statistically significant difference in obstructive apnea-hypopnea index, respiratory arousal index, sleep efficiency, and adherence between AVAPS and conventional BPAP. AVAPS was an effective alternative to conventional BPAP in improving hypercarbia in our selective cohort of pediatric patients. Prospective, longitudinal studies are needed to evaluate the benefits of AVAPS feature in the pediatric population.

Prader-Willi Syndrome-Improvement of Cognitive Function through Noninvasive Ventilation in Children

Prader-Willi Syndrome is a complex congenital disorder characterized by neonatal hypotonia, short stature, childhood...

Non-invasive Ventilation in Children With Neuromuscular Disease.

The respiratory muscles are rarely spared in children with neuromuscular diseases (NMD) which puts them at risk of alveolar hypoventilation. The role of non-invasive ventilation (NIV) is then to assist or “replace” the weakened respiratory muscles in order to correct alveolar hypoventilation by maintaining a sufficient tidal volume and minute ventilation. As breathing is physiologically less efficient during sleep, NIV will be initially used at night but, with the progression of respiratory muscle weakness, NIV can be extended during daytime, preferentially by means of a mouthpiece in order to allow speech and eating. Although children with NMD represent the largest group of children requiring long term NIV, there is a lack of validated criteria to start NIV. There is an agreement to start long term NIV in case of isolated nocturnal hypoventilation, before the appearance of daytime hypercapnia, and/or in case of acute respiratory failure requiring any type of ventilatory support. NIV is associated with a correction in night- and daytime gas exchange, an increase in sleep efficiency and an increase in survival. NIV and/or intermittent positive pressure breathing (IPPB) have been shown to prevent thoracic deformities and consequent thoracic and lung hypoplasia in young children with NMD. NIV should be performed with a life support ventilator appropriate for the child's weight, with adequate alarms, and an integrated (±additional) battery. Humidification is recommended to improve respiratory comfort and prevent drying of bronchial secretions. A nasal interface (or nasal canula) is the preferred interface, a nasobuccal interface can be used with caution in case of mouth breathing. The efficacy of NIV should be assessed on the correction of alveolar ventilation. Patient ventilator synchrony and the absence of leaks can be assessed on a sleep study with NIV or on the analysis of the ventilator's in-built software. The ventilator settings and the interface should be adapted to the child's growth and progression of respiratory muscle weakness. NIV should be associated with an efficient clearance of bronchial secretions by a specific program on the ventilator, IPPB, or mechanical insufflation-exsufflation. Finally, these children should be managed by an expert pediatric multi-disciplinary team.

Non-invasive Ventilation for Children With Chronic Lung Disease.

Advances in medical care and supportive care options have contributed to the survival of children with complex disorders, including children with chronic lung disease. By delivering a positive pressure or a volume during the patient's inspiration, NIV is able to reverse nocturnal alveolar hypoventilation in patients who experience hypoventilation during sleep, such as patients with chronic lung disease. Bronchopulmonary dysplasia (BPD) is a common complication of prematurity, and despite significant advances in neonatal care over recent decades its incidence has not diminished. Most affected infants have mild disease and require a short period of oxygen supplementation or respiratory support. However, severely affected infants can become dependent on positive pressure support for a prolonged period. In case of established severe BPD, respiratory support with non-invasive or invasive positive pressure ventilation is required. Patients with cystic fibrosis (CF) and advanced lung disease develop hypoxaemia and hypercapnia during sleep and hypoventilation during sleep usually predates daytime hypercapnia. Hypoxaemia and hypercapnia indicates poor prognosis and prompts referral for lung transplantation. The prevention of respiratory failure during sleep in CF may prolong survival. Long-term oxygen therapy has not been shown to improve survival in people with CF. A Cochrane review on the use NIV in CF concluded that NIV in combination with oxygen therapy improves gas exchange during sleep to a greater extent than oxygen therapy alone in people with moderate to severe CF lung disease. Uncontrolled, non-randomized studies suggest survival benefit with NIV in addition to being an effective bridge to transplantation. Complications of NIV relate mainly to prolonged use of a face or nasal mask which can lead to skin trauma, and neurodevelopmental delay by acting as a physical barrier to social interaction. Another associated risk is pulmonary aspiration caused by vomiting whilst wearing a face mask. Adherence to NIV is one of the major barriers to treatment in children. This article will review the current evidence for indications, adverse effects and long term follow up including adherence to NIV in children with chronic lung disease.

Long-Term Non-invasive Ventilation in Children: Current Use, Indications, and Contraindications.

This review focuses on the delivery of non-invasive ventilation—i.e., intermittent positive-pressure ventilation—in children lasting more than 3 months. Several recent reviews have brought to light a dramatic escalation in the use of long-term non-invasive ventilation in children over the last 30 years. This is due both to the growing number of children receiving care for complex and severe diseases necessitating respiratory support and to the availability of LT-NIV equipment that can be used at home. While significant gaps in availability persist for smaller children and especially infants, home LT-NIV for children with chronic respiratory insufficiency has improved their quality of life and decreased the overall cost of care. While long-term NIV is usually delivered during sleep, it can also be delivered 24 h a day in selected patients. Close collaboration between the hospital complex-care team, the home LT-NIV program, and family caregivers is of the utmost importance for successful home LT-NIV. Long-term NIV is indicated for respiratory disorders responsible for chronic alveolar hypoventilation, with the aim to increase life expectancy and maximize quality of life. LT-NIV is considered for conditions that affect respiratory-muscle performance (alterations in central respiratory drive or neuromuscular function) and/or impose an excessive respiratory load (airway obstruction, lung disease, or chest-wall anomalies). Relative contraindications for LT-NIV include the inability of the local medical infrastructure to support home LT-NIV and poor motivation or inability of the patient/caregivers to cooperate or understand recommendations. Anatomic abnormalities that interfere with interface fitting, inability to protect the lower airways due to excessive airway secretions and/or severely impaired swallowing, or failure of LT-NIV to support respiration can lead to considering invasive ventilation via tracheostomy. Of note, providing home LT-NIV during the COVID 19 pandemic has become more challenging. This is due both to the disruption of medical systems and the fear of contaminating care providers and family with aerosols generated by a patient positive for SARS-CoV-2 during NIV. Delay in initiating LT-NIV, decreased frequency of home visits by the home ventilation program, and decreased availability of polysomnography and oximetry/transcutaneous PCO2 monitoring are observed. Teleconsultations and telemonitoring are being developed to mitigate these challenges.

Risk factors for postoperative pulmonary complications in children with severely compromised pulmonary function secondary to severe scoliosis.

After corrective surgery for scoliosis, postoperative pulmonary complications lead to increases in morbidity, length of hospital stay (LOS) and mortality. This study aimed to identify associations with such respiratory complications, and to assess the utility of noninvasive ventilation (NIV) in children with severe scoliosis METHODS: This retrospective cohort study included all children aged ≤17 years who underwent spinal surgery for scoliosis between January 2009 and January 2012 at a quaternary pediatric hospital. Data were collated regarding polysomnography (PSG) and NIV use, before and after corrective surgery. Factors associated with severely compromised pulmonary function (SCPF) were established and correlations with the occurrence of postoperative pulmonary complications and LOS were identified. Altogether, 133 children had corrective surgery for scoliosis, aged 12.7 (range: 2-17) years at operation. Scoliosis causes were identified as: idiopathic (39.8%), neuromuscular disease (32.2%), syndrome (15.7%), and congenital (12%). Correlates with SCPF (forced vital capacity [FVC] <40% predicted, n = 10) included markers of sleep hypoventilation, including serum bicarbonate ≥29 mmol/L, morning pCO2 > 50mm Hg (P = .003), and overnight, episodic CO2 retention of >7 mm Hg. Using these parameters an additional eight children with SCPF were identified making a total of 18 out of 133 (13.5%) of the patients. Postoperative pulmonary complications were seen in 24 children (18%) and their occurrence correlated with higher Cobb angle (>90°), lower pulmonary function (FVC), higher serum bicarbonate and underlying neuromuscular disease. Amongst the 18 children with SCPF, regular use of NIV preoperatively was associated with reduced rate of postoperative pulmonary complications (P = .02) and reduced LOS by 6.4 days (P = .01). Nocturnal hypoventilation on PSG identifies children with SCPF. Use of NIV in children with SCPF was linked to fewer postoperative pulmonary complications and reduced duration of hospital stay.

Home Overnight Gas Exchange for Long-Term Noninvasive Ventilation in Children.

The aim of CPAP and noninvasive ventilation (NIV) is to correct sleep-disordered breathing and nocturnal gas exchange. The aim of the study was to analyze the results of a systematic home pulse oximetry ([Formula: see text]) and transcutaneous carbon dioxide ([Formula: see text]) monitoring in stable pediatric subjects on long-term CPAP/NIV or screened for CPAP/NIV weaning, and the consequent interventions in the subjects with abnormal gas exchange. The home overnight [Formula: see text] and [Formula: see text] recordings of stable pediatric subjects treated with or weaned from CPAP, NIV, or high-flow nasal cannula between January 2017 and March 2018 were analyzed. A total of 110 recordings, performed in 79 subjects, median age 6 (interquartile range [IQR] 1.5-14) y, were analyzed. Fifty-two recordings (47%) were performed during NIV, 43 (39%) during CPAP, 2 (2%) during high-flow nasal cannula, and 13 (12%) during a spontaneous ventilation weaning trial from ventilatory support. The quality of recording was excellent in 81% of recordings, 5 recordings (5%) had <4 h of recording time, 5 (5%) had artifacts on the [Formula: see text] signal, and 16 (15%) had artifacts on the [Formula: see text] signal. Gas exchange abnormalities were observed in 11 subjects with [Formula: see text] > 50 mm Hg during ≥ 2% of recording time (n = 8), mean [Formula: see text] ≥ 50 mm Hg (n = 6), mean [Formula: see text] < 35 mm Hg (n = 3), and [Formula: see text] < 90% during ≥ 2% of recording time (n = 2). Consequent interventions were (multiple interventions possible): change of device settings (n = 6), change of interface (n = 2), switched to high-flow nasal cannula (n = 1), and a control recording (n = 2). A significant number (∼12%) of systematic home [Formula: see text] and [Formula: see text] recordings in stable pediatric subjects treated with CPAP/NIV were abnormal and may be corrected by adequate therapeutic interventions.

Long Term Non-invasive Ventilation in Children With Central Hypoventilation.

Central hypoventilation (CH) is a quite rare disorder caused by some congenital or acquired conditions. It is featured by increased arterial concentration of serum carbon dioxide related to an impairment of respiratory drive. Patients affected by CH need to be treated by mechanical ventilation in order to achieve appropriate ventilation and oxygenation both in sleep and wakefulness. In fact, in severe form of Congenital Central Hypoventilation Syndrome (CCHS) hypercarbia can be present even during the day. Positive pressure ventilation via tracheostomy is the first therapeutic option in this clinical condition, especially in congenital forms. Non-Invasive ventilation is a an option that must be reserved for more stable clinical situations and that requires careful monitoring over time.

Use of non-invasive ventilation in children with congenital tracheal stenosis: a 10-year retrospective review

Use of non-invasive ventilation in children with congenital tracheal stenosis: a 10-year retrospective review

Clinical characteristics of non-invasive ventilation treatment in children with spinal muscular atrophy and sleep disordered breathing

Objective: To study the short-term and long-term efficacy of the non-invasive ventilation treatment in children with spinal muscular atrophy (SMA) and sleep-disordered breathing. Methods: This was a prospective research to study the effect of night-time non-invasive ventilation in children with SMA and moderate to severe sleep-disordered breathing during March 2016 to January 2018, from the Pulmonary Department of Capital Institute of Pediatrics Affiliated Children's Hospital. Patients were divided into the treated group (with night-time non-invasive ventilation) and the control group (without ventilator). Sleep breathing pressure titration was suggested to the patients who were prepared to receive non-invasive ventilation. All cases were followed up for one year. Parameters'changes in polysomnography were assessed (paired t-test) in titration patients. Frequency of respiratory tract infection during the next year in the patients with and without ventilation was collected and compared (Mann-Whitney U-test). Results: Seventeen cases were recruited. The average age was (5.1±2.9) years, 10 cases were boys and 7 cases were girls. In the titration group (8 patients), after non-invasive ventilation, the average apnea hypopnea index was (3.8±2.5) times/h (t=4.086, P=0.005), hypopnea index was (2.4±1.2) times/h (t=2.779, P=0.027), average oxygen saturation during total sleep time was 0.966±0.007 (t=-5.292, P=0.001), and the minimum oxygen saturation was 0.906±0.023 (t=-3.938, P=0.006). All the above parameters were significantly improved after treatment. Than before, which was (16.6±9.7) times/h, (7.2±4.7) times/h, 0.946±0.015, 0.786±0.092 respectively. Ventilator mode for the 9 children with long time non-invasive ventilation at home was Bi-level positive airway pressure S/T. The positive airway pressure was set at 8-14 cmH(2)O (1 cmH(2)O=0.098 kPa) in inspiratory phase and 4-6 cmH(2)O in expiratory phase. In the treated group (9 patients), the average frequency of upper respiratory tract infection was 1.0 (0, 3.0) times/year (Z=-2.245, P=0.023), the lower respiratory tract infection was 0 (0, 0) times/year (Z=-3.189, P=0.001), hospitalization was 0 (0, 0) times/year (Z=-3.420, P<0.01), and admission to intensive care unit was 0 (0, 0) times/year (Z=-3.353, P=0.029). All the above indexes were significantly decreased compared with the control group (8 patients), which was 3.0 (2.3, 7.0) times/year, 2.0 (1.3, 4.5) times/year, 1.0 (1.0, 4.3) times/year, 0.5 (0, 1.0) times/year respectively. Conclusion: Non-invasive ventilation is efficient to SMA children with sleep-disordered breathing, and also can reduce the incidence of respiratory tract infections for children with SMA.

Use of non-invasive ventilation in children with congenital tracheal stenosis

IntroductionCongenital tracheal stenosis (CTS) is a rare airway condition characterized by complete tracheal rings. Most patients undergo a slide tracheoplasty, which greatly reduces mortality but significant morbidity remains. The assessment of sleep disordered breathing (SDB) and use of non-invasive ventilation (NIV) in these children has not been described. AimTo describe the presence of SDB and use of NIV in children diagnosed with CTS over a 10-year period (2005–2015). DesignRetrospective case series at a tertiary children's hospital. ResultsThere were 16 patients identified with CTS with a median [range] age at diagnosis of 2.5 months (0–9 months). One child died in the immediate post-operative period following a slide tracheoplasty, leaving 15 survivors. There were no later deaths during follow-up while using NIV for up to 3 years after surgery. Slide tracheoplasty was undertaken in (12/15) with long-segment tracheal stenosis. 3/15 patients had a short-segment tracheal stenosis and were managed conservatively. The use of NIV occurred in 10/15 (66.67%) patients, all of whom had long-segment CTS. Pre-operative polysomnography (PSG) showed a median (±SD) obstructive apnoea/hypopnoea index (OAHI) of 14.6/hr (±6.2) which reduced to 7.2/hour (±4.2) on NIV prior to slide tracheoplasty. The median oxygen desaturation index (ODI) before NIV use was 15.3 (±19.4) episodes/hour, which reduced to 6.3 (±11) on NIV. The median period of NIV use was 5 [1–24 months] months. ConclusionPatients with CTS have obstructed sleep disordered breathing. Trials of NIV are well-tolerated and improve sleep disordered breathing.