Background:. Neuromuscular scoliosis (NMS) can displace the diaphragm into the thorax, reducing lung volume and impairing respiration. As an alternative to posterior spinal fusion, growth-friendly surgery (GFS) allows thoracic growth while correcting deformity. We evaluated associations of GFS with thoracic dimensions and diaphragm positioning, hypothesizing that GFS would alleviate respiratory constraints in pediatric NMS. Methods:. We reviewed data from 45 patients who underwent GFS for NMS at a mean age of 8 years, from 2003 to 2022. Radiographic measures were lung volume, space available for lung (SAL) ratio, diaphragm intrusion index (DII), diaphragm vertebral level (DVL), T1-T12 height, and T1-S1 height, assessed preoperatively, postoperatively, and at 2-year follow-up. We also analyzed Early-Onset Scoliosis 24-item Questionnaire (EOSQ-24) outcomes related to lung function. A “latest follow-up” time point was included for patients with extended data (mean: 7.2 years; range: 2.0-18.5). Paired t tests compared preoperative and follow-up values. Alpha = 0.05. Results:. Mean lung volume increased from 1,992 cm3 preoperatively to 2,917 cm3 postoperatively and 3,210 cm3 at 2 years (p < 0.001), remaining stable (3,590 cm3) at latest follow-up. SAL ratio improved from 0.79 preoperatively to 0.91 postoperatively and 0.89 at 2 years, with no change at final follow-up. DII improved from 0.59 (right) and 0.56 (left) preoperatively to 0.69 (right) and 0.70 (left) at 2 years, with no decline thereafter (p < 0.001). DVL showed bilateral descent by approximately 1.3 vertebral levels through 2 years and remained stable. T1-T12 and T1-S1 heights increased to 21 and 34 cm, respectively, at 2 years (p < 0.001). EOSQ-24 scores improved postoperatively, with further gains by 2 years in energy (from 55 to 83), pulmonary function (from 69 to 94), and fatigue (from 48 to 19). Conclusions:. GFS was associated with improvements in thoracic dimensions, diaphragm position, lung volume, and respiratory-related quality of life in pediatric NMS, underscoring the potential of GFS to address deformity and long-term respiratory health in this population. Level of Evidence:. Level III. See Instructions for Authors for a complete description of levels of evidence.

The chest and spine deformity in neuromuscular disease (NMDz) can impact respiratory mechanics and pulmonary function by changing the orientation of the muscles and joints of the respiratory system and placing them in a mechanically unfavorable position. This increases mechanical load on weak respiratory muscles and eventually can cause respiratory failure. Therefore, chest and spine deformity in NMDz will both lead to increased respiratory "load" and decreased respiratory muscle "pump", an exceptionally bad combination. While the current pharmacotherapies used for progressive neuromuscular disease focus on slowing progression, a similar approach has been used for decades in managing chest and spine deformity in patients with NMDz. There are, however, variable approaches to doing so and a recognition that not all "neuromuscular scoliosis" is the same and that each patient type (i.e. hypotonic vs. hypertonic) requires a different approach. Figuring out what approach to use requires both an understanding of the underlying pathophysiology of a particular neuromuscular condition and considering available options for and timing of surgical interventions. The remaining discussion will focus on hypotonic neuromuscular scoliosis.

Scoliosis is a common complication of neuromuscular disorders. These patients are frequently recalcitrant to nonoperative treatment. When treated surgically, they have the highest risk of complications of all forms of scoliosis. While recent studies have shown an improvement in the rate of complications, they still remain high ranging from 6.3 to 75% depending upon the underlying etiology and the treatment center (Mohamad et al. in J Pediatr Orthop 27:392-397, 2007; McElroy et al. in Spine, 2012; Toll et al. in J Neurosurg Pediatr 22:207-213, 2018; Cognetti et al. in Neurosurg Focus 43:E10, 2017). For those patients who are able to recover from the perioperative period without major complications, several recent studies have shown decreased long-term mortality and improved health-related quality of life in neuromuscular patients who have undergone spine fusion (Bohtz et al. in J Pediatr Orthop 31:668-673, 2011; Ahonen et al. in Neurology 101:e1787-e1792, 2023; Jain et al. in JBJS 98:1821-1828, 2016). It is critically important to optimize patients preoperatively to minimize the risk of post-operative complications and maximize long-term outcomes. In order to do so, one must familiarize themselves with the common complications and their treatment. The most common complications are pulmonary in nature. With reported rates as high as 23-29%, pre-operative optimization should be employed for these patients to minimize the risk of post-operative complications (Sharma et al. in Eur Spine J 22:1230-1249, 2013; Rumalla et al. in J Neurosurg Spine 25:500-508, 2016). The next most common cause of complications are implant related, with 13-23% of patients experiencing an implant-related complication that may require a second procedure (Toll et al. in J Neurosurg Pediatr 22:207-213, 2018; Sharma et al. in Eur Spine J 22:1230-1249, 2013) Therefore optimization of bone quality prior to surgical intervention is important to help minimize the risk of instrumentation failure. Optimization of muscle tone and spasticity may help to decrease the risk of instrumentation complications, but may also contribute to the progression of scoliosis. While only 3% of patients have neurologic complication, significant equipoise remains regarding whether or not patients should undergo prophylactic detethering procedures to minimize those risks (Sharma et al. in Eur Spine J 22:1230-1249, 2013). Although only 1.8% of complications are classified as cardiac related, they can be among the most devastating (Rumalla et al. in J Neurosurg Spine 25:500-508, 2016). Simply understanding the underlying etiology and the potential risks associated with each condition (i.e., conduction abnormalities in a patient with Rett syndrome or cardiomyopathies patients with muscular dystrophy) can be lifesaving. The following article is a summation of the half day course on neuromuscular scoliosis from the 58th annual SRS annual meeting, summarizing the recommendations from some of the world's experts on medical considerations in surgical treatment of neuromuscular scoliosis.

Indigenous peoples around the world bear a disproportionate burden of chronic respiratory diseases, which are associated with increased risks of morbidity and mortality. Despite the imperative to address global inequity, research focused on strengthening respiratory health in Indigenous peoples is lacking, particularly in low-income and middle-income countries. Drivers of the increased rates and severity of chronic respiratory diseases in Indigenous peoples include a high prevalence of risk factors (eg, prematurity, low birthweight, poor nutrition, air pollution, high burden of infections, and poverty) and poor access to appropriate diagnosis and care, which might be linked to colonisation and historical and current systemic racism. Efforts to tackle this disproportionate burden of chronic respiratory diseases must include both global approaches to address contributing factors, including decolonisation of health care and research, and local approaches, co-designed with Indigenous people, to ensure the provision of culturally strengthened care with more equitable prioritisation of resources. Here, we review evidence on the burden of chronic respiratory diseases in Indigenous peoples globally, summarise factors that underlie health disparities between Indigenous and non-Indigenous people, propose a framework of approaches to improve the respiratory health of Indigenous peoples, and outline future directions for clinical care and research.

There are some syndromes that present with unique manifestations pertaining to the spinal column. A good working understanding of these common syndromes is useful for the spinal deformity surgeons and related healthcare providers. This review attempts to encompass these unique features and discuss them in three broad groups: hypermobility syndromes, muscle pathology-related syndromes, and syndromes related to poor bone quality. This review explores the features of these syndromes underpinning the aspects of surgical and medical management. This review represents the proceedings of the Paediatric Half-Day Course at the 57th Annual Meeting of the Scoliosis Research Society.

BACKGROUND:Cerebral palsy (CP) can cause scoliosis with large thoracolumbar or lumbar curves. Such curves may impair pulmonary function by causing the abdomen and diaphragm to encroach on the thorax. Our purpose was to investigate changes in diaphragm position and other thoracic radiographic measurements at 2 years after posterior spinal fusion (PSF). METHODS:Retrospective review of data from 56 pediatric patients (Gross Motor Function Classification System >3) who underwent PSF for CP-related (neuromuscular) scoliosis at one US academic hospital from 2010 to 2018. In this study, we used radiographs taken preoperatively and 2 years after PSF to measure lung volume, diaphragm intrusion index (DII), diaphragm vertebral level (DVL), space available for the lung (SAL), and T1-S1 height. RESULTS:Lung volume had increased by a mean 902 cm 3 (range, -735 to 2697 cm 3 ) at 2-year follow-up. DII improved from a mean (and SD) of 61%±12% to 71%±11% on the left side and 58%±14% to 68%±11% on the right ( P <0.001). DVL increased caudally by a mean 1.2 vertebral levels bilaterally, with a mean postoperative position between T8 and T9. Lung space became more symmetrical as the SAL increased from 0.76 to 0.91 ( P <0.001). T1-S1 height increased by a mean 7.5±4.3 cm. CONCLUSIONS:These findings suggest a new way to understand changes in thoracic volume and redistribution of thoracic and lumbar balance when correcting the collapsing spinal deformity in CP. A more caudal postoperative diaphragm position with less diaphragm intrusion into the thorax may reflect an improved length-tension configuration, which could in turn produce greater diaphragmatic strength and endurance. LEVEL OF EVIDENCE:Level III.

Respiratory symptoms are ubiquitous in children and, even though they may be the harbinger of poor long-term outcomes, are often trivialised. Adverse exposures pre-conception, antenatally and in early childhood have lifetime impacts on respiratory health. For the most part, lung function tracks from the pre-school years at least into late middle age, and airflow obstruction is associated not merely with poor respiratory outcomes but also early all-cause morbidity and mortality. Much would be preventable if social determinants of adverse outcomes were to be addressed. This review presents the perspectives of paediatricians from many different contexts, both high and low income, including Europe, the Americas, Australasia, India, Africa and China. It should be noted that there are islands of poverty within even the highest income settings and, conversely, opulent areas in even the most deprived countries. The heaviest burden of any adverse effects falls on those of the lowest socioeconomic status. Themes include passive exposure to tobacco smoke and indoor and outdoor pollution, across the entire developmental course, and lack of access even to simple affordable medications, let alone the new biologicals. Commonly, disease outcomes are worse in resource-poor areas. Both within and between countries there are avoidable gross disparities in outcomes. Climate change is also bearing down hardest on the poorest children. This review highlights the need for vigorous advocacy for children to improve lifelong health. It also highlights that there are ongoing culturally sensitive interventions to address social determinants of disease which are already benefiting children.

Patients with early onset scoliosis are at high risk of sleep disordered breathing, sleep disruption, and adverse consequences of poor sleep. In this study, we aim to assess the prevalence of periodic limb movements of sleep in a cohort of children with early onset scoliosis and identify factors that correlate with the presence of periodic limb movements. This is a retrospective chart review of 40 patients with EOS (ages 1–17 years) who underwent a PSG from 2003 through 2019. Data collected included age, sex, and polysomnography parameters. Descriptive statistics were used: independent T test and Pearson correlation. The average age was 9.6 years (SD 5.2); 22 were female. Eleven patients (27.5

CASE:We describe treatment of severe multilevel congenital thoracic fusion in a 3-year-old girl with Apert Syndrome by posterior element excision, posterior column osteotomies, and gradual distraction with magnetically controlled growing rods (MCGR) with 3-year follow-up. We also describe short-term follow-up with similar management in an 8-year-old patient with a congenitally fused thoracic spine from Jarcho-Levin syndrome. CONCLUSION:Posterior element resection and targeted posterior column osteotomies combined with gradual distraction with MCGR offers a promising treatment course for children with severe thoracic insufficiency syndrome derived from congenital fusions.

Objective To describe our multidisciplinary bronchopulmonary dysplasia (BPD) consult team's systematic approach to BPD associated pulmonary hypertension (PH), to report our center outcomes, and to evaluate clinical associations with outcomes. Study design Retrospective cohort of 60 patients with BPD-PH who were referred to the Seattle Children's Hospital BPD team from 2018 to 2020. Patients with critical congenital heart disease were excluded. Demographics, comorbidities, treatments, closure of hemodynamically relevant intracardiac shunts, and clinical outcomes including time to BPD-PH resolution were reviewed. Results Median gestational age of the 60 patients was 25 weeks (IQR: 24–26). 20% were small for gestational age (SGA), 65% were male, and 25% received a tracheostomy. With aggressive cardiopulmonary management including respiratory support optimization, patent ductus arteriosus (PDA) and atrial septal defect (ASD) closure (40% PDA, 5% ASD, 3% both), and limited use of pulmonary vasodilators (8%), all infants demonstrated resolution of PH during the follow-up period, including three (5%) who later died from non-BPD-PH morbidities. Neither SGA status nor the timing of PH diagnosis (<36 vs. ≥36 weeks PMA) impacted the time to BPD-PH resolution in our cohort [median 72 days (IQR 30.5–166.5)]. Conclusion Our multidisciplinary, systematic approach to BPD-PH management was associated with complete resolution of PH with lower mortality despite less sildenafil use than reported in comparable cohorts. Unique features of our approach included aggressive PDA and ASD device closure and rare initiation of sildenafil only after lack of BPD-PH improvement with respiratory support optimization and diagnostic confirmation by cardiac catheterization.

Background:Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1.Methods:Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs.Results:Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs.Conclusion:We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.

The following lecture was delivered at the Scoliosis Research Society annual meeting in 2020. It addresses our current knowledge about the respiratory impact of Early Onset Scoliosis, the limitations in the data that have been published, and the opportunity for further clinical research in the field.

(1) Background: Breathing is an essential function that requires both metabolic (or au-tomatic) and voluntary (behavioral) control during wakefulness but during sleep depends on metabolic control via peripheral and central chemoreceptors. Breathing during sleep disordered breathing also depends on the maturity of the neural centers and the strength of the respiratory muscles. We do not know if the response to apnea varies with age. (2) Methods: We measured the obstructive apneas and hypopneas during REM and NREM in polysomnography studies from children referred for snoring. Exclusion criteria: younger than 1 year of age, neuromuscular or syndrome comorbidity, oxygen or positive airway pressure, central apnea, and studies with loss of airflow sensors. (3) Results: Two-hundred-and-sixty-eight sleep studies were included. Mean age was 8.7 years (4.68 SD), range 1–18 years, 160 were male, and 108 were female. The 5th centile of apnea duration during NREM is above 8 s at all ages, with a tendency to increase in the oldest groups up to 10 s. During REM sleep, it shows a gradual increase from 6 s in the youngest children to 10 s in the oldest. (4) Conclusions: Apnea/hypopnea length increases with age in children and adolescents independently from sex or severity of OSA. Using adult criteria in teens seems to be accurate.

Background: In patients with severe adolescent idiopathic scoliosis (AIS), maximal voluntary ventilation (MVV) has been shown to be more strongly correlated with Cobb angle than forced vital capacity (FVC) in an orthopaedic office setting. We sought to evaluate pulmonary function of AIS patients with changes in Cobb angle over time. Methods: 49 AIS patients with …

The aim was to investigate pulmonary function after surgical correction of adult idiopathic scoliosis. This study included 146 adult scoliosis patients aged 20–50 years (main curve in thoracic spine). Respiratory function was assessed as predicted forced vital capacity (%FVC) and the ratio of forced expiratory volume in 1 s / FVC (%FEV1) preoperatively and 2 years postoperatively and classified as a normal function (≥ 80%), mild impairment (≥ 65% and < 80%), and moderate impairment (< 65%). Preoperative %FVC and %FEV1 were 85.3% and 85.4%, which were 81.5% and 87.5% at 2 years post-surgery. The preoperative %FVC was mild and moderate in 39 (26.7%) and 12 patients (11.6%), respectively. The %FVC significantly improved (+ 6.2% ± 11.4%, P < 0.001) postoperatively for moderate severity but significantly decreased postoperatively (− 6.4% ± 9.4%, P < 0.001) for normal function. The preoperative %FEV1 was mild and moderate in 27 (18.5%) and 0 patients, respectively. The %FEV1 significantly improved postoperatively (6.3% ± 5.3%, P < 0.001) for mild severity but did not significantly change for normal severity. Twenty-three (15.8%) and 41 (28.1%) patients showed improved ⊿%FVC and ⊿% FEV1 > 5%. Logistic regression analysis showed that preoperative %FVC and %FEV1 severities were independent factors affecting postoperative recovery of %FVC (OR 0.95) and %FEV1 (OR 0.85). Pulmonary function improved in patients with preoperative pulmonary impairment of < 65% in %FVC and < 80% in %FEV1, and the real improvement was limited to patients with severe preoperative impairment.

This chapter addresses the breathing disorders associated with Early onset scoliosis (EOS), the ways to evaluate them, and the role of medical providers in managing children with EOS in resource-limited settings (RLS). EOS is often classified by aetiology, but diagnosis and strategies for care depend on the age at presentation. Several pathophysiologic processes coexist in children with EOS. Spine and chest wall deformities lead to malalignment of the vertebral body, ribs, and sternum. On physical exam, a posterior rib hump is present with or without changes in the anterior chest. One shoulder is often higher than the other. Inspection will also reveal that children use their abdominal muscles to exhale more forcefully at rest; however, this is not a prolonged exhalation but a short push with every breath. The physical examination in young children who are able to run should include an examination after running to hear breath sounds with deeper breathing and to count the respiratory rate.

INTRODUCTION:Children with early onset scoliosis (EOS) undergoing spine surgery often have significant respiratory disease. Preoperative risk assessments that predict an increased length of hospital stay (LOS) for this group have not been previously evaluated.METHODS:A voluntary protocol using preoperative lung function studies began among participants of a multicenter registry in 2016. Preoperative assessments were standardized to include spirometry, blood hemoglobin levels, serum bicarbonate, albumin and prealbumin; radiographic parameters of the spine, C-EOS classification and need for preoperative pulmonary assistance before initial growth friendly device insertion or "definitive" spine fusion. Primary outcome was LOS postoperatively. Data, including age, diagnosis, and type of surgery, was collected prospectively. Secondary outcomes measured included intensive care unit LOS, requirement for new pulmonary assistance on discharge, and pulmonary complications. Groups were compared using the Fisher exact tests.RESULTS:Of 525 children enrolled, 101 (20%) had preoperative spirometry. Median age was 8.9 years [interquartile range (IQR): 4.27]. Etiologies for EOS included 29 neuromuscular (28%), 33 idiopathic (32%), 19 syndromic (19%), and 22 congenital (21%) scoliosis. Eighty (78%) had growing rod (GR) insertions; 23 (22%) had spine fusion SF. Eighteen subjects (17%) were hospitalized ≥7 days (median=9 d); 83 had a LOS <7 days (median=3 d). Percentage of forced vital capacity (FVC%) predicted was inversely associated with LOS ≥7 days with a median of 75.3% (IQR: 41.7) for LOS <7 days and 51.7% (IQR: 41.6) (P=0.02). There were no detectable differences in LOS for other preoperative values.CONCLUSION:FVC predicted ≤50% preoperatively in children undergoing initial growth friendly rod insertion or definitive fusion after growth friendly treatment is associated with an increased risk of postoperative hospital stays ≥7 days. As demonstrated in previous studies, severe restrictive lung disease (FVC% predicted at or below 50%) is associated with increased risk of poorer outcomes for EOS patients.

Coronavirus disease 2019 (COVID‐19) has been an unprecedented and continuously evolving healthcare crisis. Severe Acute Respiratory Syndrome Coronavirus 2 (SARS‐CoV‐2) spread rapidly and initially little was known about the virus or the clinical course for infected children. In the United States of America, the medical response has been regionalized, based on variation in community transmission of the virus and localized outbreaks. Pediatric pulmonary and sleep divisions evolved in response to administrative and clinical challenges. As the workforce transitioned to working remotely, video conferencing technology and multicenter collaborative efforts were implemented to create clinical protocols. The COVID‐19 pandemic challenges the framework of current medical practice but also highlights the dynamic and cooperative nature of pediatric pulmonology and sleep medicine. Our response to this pandemic has laid the groundwork for future challenges.

Recent literature has highlighted the importance of transition from paediatric to adult care for children with chronic conditions. Non-cystic fibrosis bronchiectasis is an important cause of respiratory morbidity in low-income countries and in indigenous children from affluent countries; however, there is little information about adult outcomes of childhood bronchiectasis. We reviewed the clinical course of 31 Alaska Native adults 20–40 years of age from Alaska’s Yukon Kuskokwim Delta with childhood bronchiectasis. In patients with chronic suppurative lung disease, a diagnosis of bronchiectasis was made at a median age of 4.5 years by computerised tomography (68%), bronchogram (26%), and radiographs (6%). The patients had a median of 75 lifetime respiratory ambulatory visits and 4.5 hospitalisations. As children, 6 (19%) experienced developmental delay; as adults 9 (29%) experienced mental illness or handicap. Four (13%) patients were deceased, four (13%) had severe pulmonary impairment in adulthood, 17 (54%) had persistent or intermittent respiratory symptoms, and seven (23%) were asymptomatic. In adulthood, only five were seen by adult pulmonologists and most had no documentation of a bronchiectasis diagnosis. Lack of provider continuity, remote location and co-morbidities can contribute to increased adult morbidity. Improving the transition to adult care starting in adolescence and educating adult providers may improve care of adults with childhood bronchiectasis.