Volume assured pressure support mode use for non-invasive ventilation in pediatrics.

There has been increasing interest in utilizing volume assured pressure support (VAPS) modes of ventilation for children, which historically had only been favored in adult populations. In addition to patients with obesity hypoventilation syndrome, newer pediatric populations for which it has recently been prescribed include congenital central hypoventilation syndrome and children with neuromuscular disease such as Duchenne muscular dystrophy and spinal muscular atrophy. Given its expanding use in pediatrics, greater familiarity with VAPS is essential for pediatric pulmonologists and sleep physicians. This review article will highlight methods of initiation for this mode, specific ventilator settings, discussion of suitable pediatric patient populations, ventilator titrations via formal polysomnograms and detailed ventilator data downloads specific interpretation. Finally, common challenges to be aware of and how to troubleshoot relevant machine alarms will be reviewed.

Sleep disordered breathing in infants identified through newborn screening with spinal muscular atrophy.

BACKGROUND: Spinal muscular atrophy (SMA) is a genetic disorder that may result in neuromuscular weakness and respiratory insufficiency. Gene replacement therapy has changed the trajectory of this condition, but long-term outcomes related to sleep disordered breathing are not known. METHODS: This was a retrospective review of infants with SMA identified via newborn screening who subsequently received onasemnogene abeparvovec at the Hospital for Sick Children (Ontario, Canada). Polysomnograms were conducted at the time of confirmed diagnosis as well as regularly thereafter. RESULTS: Eleven children (4 female) were identified via newborn screen (7 with 2 copies of the SMN2 gene and 4 with 3 copies of the SMN2 gene) and received onasemnogene abeparvovec at a median age of 3.6 weeks. All eleven infants met criteria for sleep disordered breathing based on their first completed polysomnograms but improved over time. Three infants required respiratory technology, including a premature infant who was prescribed nocturnal supplemental oxygen therapy for central sleep apnea and two symptomatic infants with neuromuscular weakness who required nocturnal noninvasive ventilation. We did not find a correlation between motor scores and polysomnogram parameters. CONCLUSION: Children treated with onasemnogene abeparvovec have reduced sleep disordered breathing over time. Polysomnograms revealed abnormal parameters in all children, but the clinical significance of these findings was unclear for children who were asymptomatic for sleep disordered breathing or neuromuscular weakness. These results highlight the need to evaluate both motor scores and respiratory symptoms to ensure a holistic evaluation of clinical status.

Adenotonsillectomy for obstructive sleep apnea in children with cerebral palsy: Risks and benefits.

OBJECTIVES: To determine outcomes following adenotonsillectomy for obstructive sleep apnea (OSA) and the impact of motor and swallowing impairment on respiratory complications in children with Cerebral Palsy (CP). METHODS: A retrospective review of children with CP and sleep disordered breathing (SDB) who underwent adenotonsillectomy (2003-2021) was performed. Children with CP were age-matched to children without CP. Motor and swallowing function was assessed using the Gross Motor Functional Classification System (GMFCS) and the Eating and Drinking Ability Classification System (EDACS). The primary outcome was postoperative obstructive apnea-hypopnea index (OAHI). Secondary outcomes were cure rate, complications, and need for additional interventions. RESULTS: Ninety-seven children with CP were assessed for SDB, and 74 underwent polysomnography. Moderate or severe OSA was found in 49% (36/74). Adenotonsillectomy was performed in 30% (29/97). All children who underwent adenotonsillectomy experienced an initial reduction in OAHI (31.7/h to 2.9/h, p < 0.0001). Children with CP were less likely to achieve an OAHI<1 compared with children without CP (62.5% vs 81.8%, p = 0.23). Children with CP had more postoperative complications (43.5% vs. 8.7%) and greater odds of respiratory complications compared with children without CP (OR 8.9 95% CI 2.1-37.9). Children with CP and a GMFCS score of 5 and EDACS score between 3 and 5 had more respiratory complications post-adenotonsillectomy compared to those with GMFCS<5 (p = 0.002) and EDACS<3 (p = 0.031). CONCLUSION: Children with CP had an improved OAHI initially following adenotonsillectomy but had higher rates of post-adenotonsillectomy complications. Respiratory complications after adenotonsillectomy were more common in children with motor and swallowing impairment. Findings may provide better preoperative planning for caregivers.

Understanding caregiver experiences with disease-modifying therapies for spinal muscular atrophy: a qualitative study.

OBJECTIVE: Spinal muscular atrophy (SMA) is a neuromuscular disorder that manifests with motor deterioration and respiratory complications. The paradigm of care is shifting as disease-modifying therapies including nusinersen, onasemnogene abeparvovec and risdiplam alter the disease trajectory of SMA. The objective of this study was to explore caregivers' experiences with disease-modifying therapies for SMA. DESIGN: Qualitative study including semistructured interviews with caregivers of children with SMA who received disease-modifying therapies. Interviews were audio recorded, transcribed verbatim, coded and analysed using content analysis. SETTING: The Hospital for Sick Children (Toronto, Canada). RESULTS: Fifteen family caregivers of children with SMA type 1 (n=5), type 2 (n=5) and type 3 (n=5) participated. There were two emerging themes and several subthemes (in parentheses): (1) inequities in access to disease-modifying therapies (variable regulatory approvals, prohibitively expensive therapies and insufficient infrastructure) and (2) patient and family experience with disease-modifying therapies (decision making, hope, fear and uncertainty). CONCLUSION: The caregiver experience with SMA has been transformed by the advent of disease-modifying therapies. Consistent and predictable access to disease-modifying therapies is a major concern for caregivers of children with SMA but is influenced by regulatory approvals, funding and eligibility criteria that are heterogenous across jurisdictions. Many caregivers described going to great lengths to access therapies, highlighting issues related to justice, such as equity and access. This diverse population reflects contemporary patients and families with SMA; their broad experiences may inform the healthcare delivery of other emerging orphan drugs.

Unexplained Significant Central Sleep Apnea in Infants: Clinical Presentation and Outcomes.

OBJECTIVE: Unexplained significant central sleep apnea in term infants presents as central apneas with associated oxygen desaturations requiring respiratory support and monitoring for prolonged periods. However, there is a paucity of literature describing idiopathic central sleep apnea (ICSA) in term or near-term infants. Our aim was to describe the clinical manifestations, polysomnography data, interventions, and trajectory of ICSA in infants. DESIGN: This is a retrospective study of infants (gestational age ≥ 35 weeks) who presented with significant central apneas and were subsequently diagnosed with ICSA following polysomnography and clinical investigations between January 2011 and April 2021 at a tertiary care hospital in Canada. Polysomnography data, clinical investigations, and treatments were documented. RESULTS: Eighteen infants (male, 78%; median gestational age 38 weeks) with ICSA were included. Initial polysomnograms were completed at a median (interquartile range [IQR]) age of 1.2 (0.6-1.6) months (n = 18) and follow-up polysomnograms at 12.4 (10.6-14.0) months (n = 13). Compared to baseline diagnostic polysomnograms, at follow-up there was a significant reduction in the median (IQR) central apnea-hypopnea index (26.1 [18.2-52.9] versus 4.2 [2.6-7.2] events/hour; p = 0.001), desaturation index (30.9 [12.2-57.4] versus 3.9 [3.0-7.9] events/hour; p = 0.002), average transcutaneous carbon dioxide (41.9 [40.1-47.3 versus 39.4 [37.5-42.7] mmHg; p = 0.025), and improved nadir oxygen saturation (79.8 [69.1-83.0] versus 85.5 [83.2-87.8]%; p = 0.033), respectively. Prescribed treatments included supplemental oxygen (14/18, 78%), caffeine (5/18, 28%), and noninvasive ventilation (1/18, 6%). CONCLUSIONS: Infants with significant unexplained ICSA have a favorable clinical trajectory over time. Further research is needed to understand the etiology of this rare disorder.

Respiratory characteristics in children with spinal muscular atrophy type 1 receiving nusinersen.

BACKGROUND: Spinal muscular atrophy type 1 (SMA1) is a neuromuscular disorder with a natural history of chronic respiratory failure and death during infancy without ventilation. Recently, disease-modifying therapies such as nusinersen have improved disease trajectory. However, objective data on the trajectory of polysomnography outcomes, the relationship between motor scores and respiratory parameters, respiratory technology dependence and healthcare utilization in children with SMA1 remain to be elucidated. METHODS: This was a retrospective observational study of children with SMA1 receiving nusinersen between October 2016 and February 2021 at two tertiary care hospitals in Canada. Baseline polysomnography data, motor scores, respiratory technology, and unanticipated healthcare utilization were examined. RESULTS: Eleven children (five females, two SMN2 copies each) were included. Median (interquartile range [IQR]) age at diagnosis was 3.6 (2.8-5.0) months and age at diagnostic polysomnogram following nusinersen initiation was 9.4 (5.3-14.0) months. Nusinersen was initiated at a median (IQR) age of 5.4 (3.4-7.6) months and 8/11 children had respiratory symptoms at that time. Diagnostic polysomnography data showed a median (IQR) central apnea-hypopnea index (AHI) of 4.1 (1.8-10.0) and obstructive AHI of 2.2 (0-8.0) events/h. We observed an inverse relationship between motor scores and central apnea-hypopnea indices. All children required ventilatory support at the end of the study period. CONCLUSION: This study showed abnormal polysomnography parameters and need for ventilation despite nusinersen suggesting ongoing need for regular monitoring with polysomnography. Understanding the respiratory disease trajectory of children undergoing treatment with nusinersen will inform decision-making regarding optimal timing of ventilatory support initiation.

Chronic tracheostomy care of ventilator-dependent and -independent children: Clinical practice patterns of pediatric respirologists in a publicly funded (Canadian) healthcare system.

OBJECTIVES: To describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation. METHODS: A pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59-item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long-term follow-up. RESULTS: The response rate was 100% (17/17) with all Canadian tertiary care pediatric centers represented and heterogeneity of practice was observed in all domains assessed. For example, though most centers employ Bivona™ (17/17) and Shiley™ (15/17) tracheostomy tubes, variability was observed around tube change, re-use, and cleaning practices. Most centers require two trained caregivers (14/17) and recommend 24/7 eyes on care and oxygen saturation monitoring. Discharge with an emergency tracheostomy kit was universal (17/17). Considerable heterogeneity was observed in the timing and use of speaking valves and speech-language assessment. Inhaled anti-pseudomonal antibiotics are employed by most centers (16/17) though the indication, agent, and protocol varied by center. Though decannulation practices varied considerably, the requirement of upper airway patency was universally required to proceed with decannulation (17/17) independent of ongoing ventilatory support requirements. CONCLUSION: Considerable variability in pediatric tracheostomy tube care practice exists across Canada. These results will serve as a starting point to standardize and evaluate tracheostomy tube care nationally.

Scoliosis in Spinal Muscular Atrophy Type 1 in the Nusinersen Era.

Background and Objectives: The introduction of spinal muscular dystrophy (SMA)-modifying therapies, such as antisense oligonucleotide therapy, has changed the natural history of SMA. Most reports on treatment outcomes have focused on motor scores and respiratory function. The objective of this study is to document the development and progression of scoliosis in patients with SMA1 treated with nusinersen. Methods: A descriptive single-center study was conducted in patients with SMA1 who were treated with nusinersen before 6 months of age. Data were collected on patients who met criteria, including age at the first nusinersen dose, number of nusinersen doses, degree of scoliosis, respiratory parameters, feeding route, and motor scores at baseline and follow-up. The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) was subanalyzed using axial (AxS) and appendicular motor (ApS) scores to evaluate a possible correlation between scoliosis and axial muscle strength. Results: From our cohort, 31 percent (11/35) of patients had a diagnosis of SMA1. Sixty-three percent (7/11) met the inclusion criteria. All patients (7/7) showed initial improvement in their CHOP-INTEND scores in correlation with improvement on the ApS. Despite this, most patients did not show improvement in the AxS. Subsequently, all patients developed scoliosis in the first year of life with Cobb angles that ranged between 18° and 60°. Furthermore, total CHOP-INTEND scores had dropped in 2 patients alongside the development of a Cobb angle of >40°. Discussion: Despite the significant improvement in functional motor assessment in patients with SMA1, there is a progression of significant scoliosis despite treatment. Subsequently, lack or minimal improvement on the axial CHOP-INTEND scores may predict worsening on the total motor scores.

Risks and Benefits of Adenotonsillectomy in Children With Cerebral Palsy With Obstructive Sleep Apnea: A Systematic Review.

OBJECTIVES/HYPOTHESIS: Assess the risks and benefits of adenotonsillectomy (AT) for obstructive sleep apnea (OSA) in children with cerebral palsy (CP). STUDY DESIGN: Systematic review. METHODS: We conducted a systematic review of Medline, Embase, and Cochrane Central Registry from 1946 to 2021. Broad search concepts included cerebral palsy, pediatric, tonsillectomy/adenoidectomy, and sleep. Additional articles were identified by searching reference lists. Studies on the safety and efficacy of AT for OSA management in children with CP were included. RESULTS: Fifteen articles met inclusion criteria. Articles were classified into one or more of four themes: intraoperative risk (n = 1), postoperative risk (n = 3), postoperative care requirements (n = 6), and surgical outcomes (n = 7). No intraoperative anesthetic complications were reported. Postoperatively, respiratory complications including pneumonia were common and necessitated additional airway management. Following AT, children with CP required close postoperative observation, experienced increased lengths of stay, and had increased odds of unplanned intensive care unit (ICU) admission. Benefits following AT were improvement in OSA as measured by a reduction in obstructive apnea-hypopnea index (OAHI) as well as improved quality of life in some; however, many patients went on to require tracheostomy due to persistent OSA. CONCLUSIONS: Children with CP who undergo AT have a significant risk of developing a postoperative respiratory complication. Realistic counseling of families around increased perioperative risks in this population is imperative and close postoperative monitoring is critical. Many children will obtain a reduction in OAHI, but additional surgical management is often required, including tracheostomy. Further research is needed to determine the best management strategy for OSA in children with CP. Laryngoscope, 132:687-694, 2022.

Assessing air travel safety in neuromuscular disease: standard versus prolonged hypoxic challenge tests.

PURPOSE: The hypoxic challenge test (HCT) is used to evaluate safety for air travel in individuals with respiratory disease by breathing in 15% oxygen for 20 min. Our aim was to determine if a prolonged HCT, lasting 120 min, identified more individuals with neuromuscular disease at potential risk than the standard HCT lasting 20 min. METHODS: This was a cross-sectional study. All of the clinical testing took place at SickKids, Toronto, Canada. Patients were included in the study if they had a diagnosis of NMD, greater than 6 years of age, resting oxygen saturation ≥ 94%, and partial pressure of carbon dioxide (pCO2) ≤ 45 mmHg. Notable exclusion criteria were left ventricular ejection fraction < 30%, presence of a tracheostomy, and use of non-invasive ventilation for more than 12 h daily. Participants underwent a standard HCT as well as the prolonged HCT on the same day. RESULTS: Twenty-three patients consented to the study. One patient was withdrawn because he was unable to follow the study procedures. The 22 study participants had a mean age of 14.9 years (standard deviation (SD) of 5 years). Seventeen (77%) participants were male. Two participants were withdrawn on the day of testing due to hypercapnia. Twenty participants completed the standard and prolonged HCTs. None of the participants had a positive standard or prolonged HCT. CONCLUSION: Our results suggest that performing a standard or prolonged HCT may, in fact, not be of clinical utility in individuals with less severe NMD.

Virtual mask fitting in pediatric patients during COVID-19: A case series.

INTRODUCTION: The COVID-19 pandemic has been an unprecedented threat to our health care system. Clinicians had to pivot and develop creative and timely "virtual" solutions to provide clinical care. Our aim was to develop a standardized approach to virtual "mask fitting" for children who are either being initiated or are already on existing long-term ventilation (LTV) at a pediatric hospital. CASE AND OUTCOMES: We present three cases involving the care of children who required mask fitting for noninvasive ventilation (NIV). LTV team consultations were delivered via videoconference or phone. With the guidance of the respiratory therapist (RT), the family caregiver (FC) took measurements on their child using a standardized clinical approach (developed by the LTV RTs). Based on the measurements, an appropriate mask was selected. Successful mask fit was based on patient/FC reports, as well as objective leak data obtained from the NIV download data. DISCUSSION: Virtual clinics used for managing patients in our LTV program were feasible and efficient resulting in improved workflow for the RTs and convenience for patients and FCs. Patients and FCs had significantly less pressure to attend in-person clinics and expressed high satisfaction in terms of their experience and importantly, meeting respiratory care needs. Within the context of COVID-19, remote patient education and intervention can be delivered effectively, while reducing the risk of exposure from in-person visits to hospital. CONCLUSION: A virtual/telemedicine program to manage pediatric patients requiring mask fitting for LTV was a feasible option during COVID-19.

Decannulation following tracheostomy in children: A systematic review of decannulation protocols.

OBJECTIVE: To provide a systematic review of the existing pediatric decannulation protocols, including the role of polysomnography, and their clinical outcomes. METHODS: Five online databases were searched from database inception to May 29, 2020. Study inclusion was limited to publications that evaluated tracheostomy decannulation in children 18 years of age and younger. Data extracted included patient demographics and primary indication for tracheostomy. Methods used to assess readiness for decannulation were noted including the use of bronchoscopy, tracheostomy tube modifications, and gas exchange measurements. After decannulation, details regarding mode of ventilation, location, and length of observation period, and clinical outcomes were also collected. Descriptive statistical analyses were performed. RESULTS: A total of 24 studies including 1395 children were reviewed. Tracheostomy indications included upper airway obstruction at a well-defined anatomic site (35%), upper airway obstruction not at a well-defined site (12%) and need for long-term ventilation and pulmonary care (53%). Bronchoscopy was routinely used in 23 of 24 (96%) protocols. Tracheostomy tube modifications in the protocols included capping (n = 20, 83%), downsizing (n = 14, 58%), and fenestrations (n = 2, 8%). Measurements of gas exchange included polysomnography (n = 13/18, 72%), oximetry (n = 10/18, 56%), blood gases (n = 3,17%), and capnography (n = 3, 17%). After decannulation, children in 92% of protocols were transitioned to room air. Observation period of 48 h or less was used in 76% of children. CONCLUSIONS: There exists large variability in pediatric decannulation protocols. Polysomnography plays an integral role in assessing most children for tracheostomy removal. Evidence-based guidelines to standardize pediatric tracheostomy care remain an urgent priority.

Tough transitions: Family caregiver experiences with a pediatric long-term ventilation discharge pathway.

OBJECTIVES: Discharging a child home on long-term ventilation (LTV) via tracheostomy is complex and involves multiple healthcare providers across healthcare sectors. To date, there has been a paucity of data with respect to the experiences of families transitioning a child home on LTV. Our objective was to explore the perceptions of family caregivers (FCs) who have completed a newly developed LTV discharge pathway as they transitioned home. METHODS: We conducted 11 semi-structured interviews with FCs. Interviews focused on FC's experience with the training process, perception of competency from a knowledge and skill perspective, and opportunities for improvement. Interviews were audiotaped, transcribed verbatim, coded, and analyzed using an inductive thematic analysis approach. RESULTS: Eight mothers and three fathers of ten children participated. Six primary themes were identified: (1) making an informed decision, (2) transitioning to rehabilitation, (3) building capacity for self-care, (4) coordinating case management, (5) readying for discharge home, and (6) experiencing home care. CONCLUSION: Overall, FCs felt that the preparation and transition support obtained through the application of a standardized LTV discharge pathway allowed successful attainment of new knowledge and skills necessary to care for their child with LTV at home.

Congenital central hypoventilation syndrome and ventilatory responses during cardiopulmonary exercise testing.

BACKGROUND: Previous studies have shown evidence of hypoxemia and hypercapnia during cardiopulmonary exercise test (CPET) evaluation in children with congenital central hypoventilation syndrome (CCHS). However, there are no longitudinal studies which compared CPET findings to polysomnogram (PSG) or PHOX2B mutation, to date. OBJECTIVES: To describe the longitudinal CPET findings in a cohort of children with CCHS and correlate the findings to the PSG results. METHODS: This retrospective study was conducted in children with CCHS followed in the Long-term Ventilation Program at SickKids, Toronto, Canada between September, 2013 and January, 2020. CCHS genetic mutation, age of diagnosis, ventilatory support, family history, disease associations of CCHS, CPETs, and PSG parameters were recorded and analyzed. RESULTS: A total of nine patients with CCHS (46 CPETs and 46 PSGs) were enrolled. Four (44.4%) children had polyalanine repeat mutations. The mean (SD) age at the time of diagnosis and duration of ventilatory usage were 3.2 ± 3.4 years and 11.5 ± 2.8 years, respectively. All abnormal CPETs had hypercapnia in at least 1 phase of the exercise test. Hypercapnia (12/46; 26.1%) at peak of exercise was the most common abnormality. None of the children experienced an oxygen desaturation below 90%. End-tidal CO2 (PetCO2 ) at rest and at peak exercise in the CPETs were significantly correlated with PSG TcCO2 while PetCO2 at anaerobic threshold was correlated with CO2 in pre-PSG capillary blood gas. CONCLUSION: Nocturnal hypoventilation may impact the CPET results in CCHS children. Serial CPETs should be considered standard clinical care for all CCHS children.

Volume-assured pressure support mode for noninvasive ventilation: can it improve overnight adherence in children with neuromuscular disease?

PURPOSE: Volume-assured pressure support in noninvasive ventilation (VAPS-NIV) is a newer mode providing automatic pressure support adjustment to ensure a constant alveolar ventilation. Previous studies have shown that NIV effectiveness depends on patient adherence and tolerance. The aim of this study was to determine the adherence and efficacy of VAPS-NIV compared to spontaneous-time (S/T) mode in pediatric patients with neuromuscular disease (NMD). METHODS: This was a prospective observational study. Children with NMD who utilized NIV at home for ≥ 3 months were recruited from the Long-term ventilation clinic at The Hospital for Sick Children, Toronto, Canada, from July 1, 2015, to July 1, 2019. Baseline characteristics, date of initiation of NIV, and pulmonary function tests were recorded. Polysomnogram (PSG) data and adherence were recorded and analyzed comparing VAPS and S/T modes. RESULTS: Twenty children with NMD (17 male, 85%) were enrolled. The mean (SD) age at initiation of NIV was 11.6 ± 4.6 years. The median (IQR) duration of ventilation was 1.36 (0.80-2.98) years. The mean average daily usage and the median daily usage for VAPS mode and S/T mode were 8.4 ± 1.6 versus 7.2 ± 2.5 h (p = 0.012) and 8.6 ± 1.4 versus 7.8 ± 2.1 h (p = 0.022), respectively. There was no difference in sleep architecture, gas exchange, or parent proxy report of NIV tolerance between S/T and VAPS modes. CONCLUSION: VAPS was associated with an improvement in adherence to therapy in children with NMD compared to S/T mode. Longitudinal studies are required to evaluate long-term clinical outcomes using VAPS mode in children with NMD.

Morbidity and mortality from adenotonsillectomy in children with trisomy 21.

OBJECTIVE: Adenotonsillectomy (AT) is common in children with trisomy 21 but outcomes are variable. Therefore, practitioners must have accurate information regarding the risks of the procedure specific to trisomy 21 to help patients weigh the risks and benefits of surgery. The objective of this study was to better characterize morbidity and mortality risk factors from AT in children with trisomy 21. METHODS: A single-center retrospective chart review of children with trisomy 21 who underwent AT was conducted from 1992 to 2019. The primary outcome was 30-day post-operative complication rate. Secondary outcomes included intraoperative complications, admission duration, emergency department visits, readmissions, reoperation rate and treatment failures. RESULTS: Two-hundred and fifty one children met study criteria (median age 4.5 years). Seventy-eight patients (31.5%) had a post-operative complication requiring medical intervention, with respiratory issues (42, 53.8%), poor oral intake (29, 37.2%), and bleeding (14, 17.9%) being most common. Postoperatively, 72 patients (28.7%) had a prolonged hospital stay. Sleep disordered breathing (p = 0.003), ASA score >2 (p < 0.001), severe OSA (p = 0.003), preoperative ICU admission (p < 0.001), and aerodigestive comorbidities (p = 0.004) were associated with increased post-operative respiratory complications. No mortalities were identified. CONCLUSION: This large single institution study evaluating morbidity and mortality following AT in children with trisomy 21 identified a morbidity rate of 31.5%. These findings may improve our ability to anticipate and manage postoperative morbidity in this vulnerable population and facilitate informed discussions with patients and caregivers considering AT.

Management of Rare Causes of Pediatric Chronic Respiratory Failure.

The need for long-term noninvasive positive pressure ventilation (NiPPV) in children with chronic respiratory failure is rapidly growing. This article reviews pediatric-specific considerations of NiPPV therapy. Indications for NiPPV therapy can be categorized by the cause of the respiratory failure: (1) upper airway obstruction, (2) musculoskeletal and/or neuromuscular disease, (3) lower respiratory tract diseases, and (4) control of breathing abnormalities. The role of NiPPV therapy in select rare conditions (spinal muscular atrophy, congenital central hypoventilation syndrome, cerebral palsy, scoliosis, and Chiari malformations) is also reviewed.

Maximal Static Respiratory and Sniff Pressures in Healthy Children. A Systematic Review and Meta-Analysis.

RATIONALE: Respiratory muscle strength in children can be assessed by maximal inspiratory pressures (MIP), maximal expiratory pressures (MEP), and sniff nasal inspiratory pressures (SNIP). However, previous studies involved small cohorts of healthy children and reported wide reference ranges. OBJECTIVES: To perform a systematic review to summarize existing reference ranges for MIP, MEP, and SNIP tests in healthy children and to conduct a meta-analysis to develop comprehensive prediction equations. DATA SOURCES: Five databases were searched for relevant studies from database inception to May 29, 2017. DATA EXTRACTION: Study inclusion was limited to publications that evaluated MIP, MEP, and SNIP values in healthy children aged 18 years or younger. Studies were also excluded if testing methodology differed greatly from the 2002 American Thoracic Society Statement on Respiratory Muscle Testing. Requests for raw data were made to authors via e-mail. SYNTHESIS: A total of 18 studies including 3,509 children were systematically reviewed. Diagnostic accuracy of the included studies was assessed using the QUADAS-2 tool, which revealed a high risk of bias for flow and timing and for applicability that may influence the generalizability of our findings. All 18 studies evaluated respiratory pressures in children in seated position. MIP tests were conducted from residual volume, MEP tests from total lung capacity, and SNIP tests from functional residual capacity. The MIP and MEP values in three age groups for boys and girls were summarized using meta-analysis based on individual participant data from five studies containing 1,709 healthy children. Further analyses showed that MIP and MEP were significantly greater in boys than in girls (P < 0.0001). In both sexes, MEP values were always greater than MIP values (P < 0.05). Multivariable random effects models were then performed to establish sex-specific prediction equations. These equations found age, height, and weight to be significant predictor variables. Only two studies with SNIP values from healthy children were included in the review, but they were not part of the meta-analysis. CONCLUSIONS: We summarized the available reference ranges for MIP, MEP, and SNIP tests based on existing literature, especially for three age groups, and developed prediction equations that can be used in pulmonary function laboratories to aid clinicians. Existing literature on SNIP tests is limited, and future studies are encouraged to explore their use in children. Systematic review registered with the International Prospective Register of Systematic Reviews (PROSPERO; CRD42017072004).