Pre-op considerations in neuromuscular scoliosis deformity surgery: proceedings of the half day course at the 58th annual meeting of the Scoliosis Research Society.

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.

Social determinants of respiratory health from birth: still of concern in the 21st century?

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.

Chronic respiratory disease in Indigenous peoples: a framework to address inequity and strengthen respiratory health and health care globally.

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.

Consensus-Based Best Practice Guidelines for the Management of Spinal Deformity and Associated Tumors in Pediatric Neurofibromatosis Type 1: Screening and Surveillance, Surgical Intervention, and Medical Therapy.

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.

A multidisciplinary approach to severe bronchopulmonary dysplasia is associated with resolution of pulmonary hypertension.

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.

Does pulmonary function improve after surgical correction of adult idiopathic scoliosis?

PURPOSE: The aim was to investigate pulmonary function after surgical correction of adult idiopathic scoliosis. METHODS: 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%). RESULTS: 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). CONCLUSION: 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.

55th Harrington Lecture: The respiratory impact of EOS and its therapies: where are we and where do we go from here?

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.

Low Preoperative Lung Functions in Children With Early Onset Scoliosis Predict Postoperative Length of Stay.

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.

Association between health-related quality of life outcomes and pulmonary function testing.

INTRODUCTION: Investigations in associations between subjective health-related quality of life (HRQoL) measures and objective clinical assessments in patients with early-onset scoliosis (EOS) are limited. The purpose of this study is to investigate the association between pulmonary function rated by parents and pulmonary function testing (PFT) in patients with EOS. MATERIALS/METHODS: In this cross-sectional study, patients with EOS at any stage of treatment from 2011 to 2018 were identified in 2 registries including 33 centers. Parents' perception of pulmonary function was evaluated using pulmonary function (PF) domain in the Early-Onset Scoliosis 24 item Questionnaire (EOSQ-24). PFT measures included FVC% predicted, FEV1/FVC, and TLC% predicted. All PFT predicted values utilized arm span. PFT and EOSQ-24 questionnaire were completed within 180 days of each other with an average day difference of 26 days. RESULTS: 176 patients (mean age: 10.4 years old, female: 56%) were identified. 33% of patients were of congenital/structural etiology, 27% neuromuscular, 26% syndromic, and 14% idiopathic. Wide variance and lower scores of PF domain were reported by parents at lower FVC% predicted values (< 50%). As FVC% predicted values increased, PFD scores increased with simultaneous decreases in variance with few exceptions. CONCLUSION: More variability and frequent lower pulmonary function values are reported by parents when percent forced vital capacity (FVC%) is < 50%. This likely reflects the degree to which children adapt to restrictive lung disease and the limits on adaptation that occur increasingly as lung function falls below 50% predicted. As a direct linear association with high correlation was expected, more research into the character of what the PF domain is measuring is necessary. LEVEL OF EVIDENCE: IV.

Innovating and adapting in pediatric pulmonology and sleep medicine during the COVID-19 pandemic: ATS pediatric assembly web committee consensus statement for initial COVID-19 virtual response.

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.

Preoperative 6-Minute Walk Performance in Children With Congenital Scoliosis.

BACKGROUND: The 6-minute walk test (6MWT) is used to assess the function of cardiopulmonary and neuromuscular conditions in adults and children. The primary research question was to determine the relationship between 6MWT distance and forced vital capacity (FVC) and the major curve among children with congenital scoliosis with rib anomalies. METHODS: The authors recorded 6MWT distance in meters, FVC as a percentage of predicted normal value using arm span for height (FVC%), and Cobb angle in 20 children (13 girls; average age, 6.7±1.3 y) with congenital scoliosis before outpatient surgical treatment. The 6MWT uses a standardized protocol and measures distance traveled in 6 minutes on a flat surface. The authors then determined the correlation between these measures using linear regression analysis. RESULTS: The Cobb angle of the major curvature was 55.4±20.5 degrees. The type of vertebral anomaly was mixed in 17 cases, formation failure in 2 cases, and segmentation failure in 1 case. The range of rib anomalies was 3.4±3.9 levels; 15 and 5 patients, respectively, had unilateral and bilateral rib anomalies. FVC and FVC% were 0.7±0.2 L and 60%±19%, respectively. The ratio of forced expiratory volume at 1 second to FVC (FEV1/FVC), which indicates obstructive lung disease, was normal at 93%±7%. The 6MWT distance was 386.3±59.4 m, which was ≤10% of the predicted distance for normal children. No child was able to walk the normal distance on the basis of published norms. 6MWT distance was significantly correlated with arm span (ρ=0.46, P=0.04) and major curve (ρ=-0.61, P=0.004), but not with FVC% (ρ=0.17, P=0.49). CONCLUSIONS: The 6MWT distance is a feasible measure of function and is substantially reduced before surgery in children with thoracic congenital scoliosis with rib anomalies. The 6MWT distance was significantly correlated with a major curve but not with FVC%. 6MWT distance is not affected by moderate lung function impairment. LEVEL OF EVIDENCE: Level IV-retrospective cohort study.

Clinical Issues for Pediatric Pulmonologists Managing Children With Thoracic Insufficiency Syndrome.

Thoracic insufficiency Syndrome (TIS) is a recently coined phrase to describe children with spine and chest wall deformities, inherited and acquired, who have respiratory impairment, and are skeletally immature. This population has both restrictive and less often obstructive lung disease due to changes in spine and rib configuration which reduce lung volume, stiffen the chest wall, and reduce respiratory muscle strength. Although the population is heterogeneous with regard to age of onset, etiology, severity of deformity, and rate of progression of the deformity, there are common issues that arise which can be addressed by pediatric pulmonologists. These are illustrated in this review by using Early Onset Scoliosis as a common form of TIS. The pulmonary issues pertaining to TIS require collaboration with multi-disciplinary teams, particularly spine surgeons, in order to make decisions about non-surgical and surgical strategies, timing of surgery and medical supportive care over time. Pulmonary input about respiratory function should be used in conjunction with structural features of each deformity in order to determine the impact of the deformity and the response to various treatment options. In those patients with residual lung function impairment as young adults, pediatric pulmonologists must also ensure successful transition to adult care.

Bigger is better: larger thoracic height is associated with increased health related quality of life at skeletal maturity.

STUDY DESIGN: Cross sectional OBJECTIVES: The purpose of this study is to evaluate the association between thoracic height and health-related quality of life (HRQoL) at skeletal maturity in patients with EOS. Current literature suggests a minimum thoracic height of 18 cm to 22 cm to avoid poor pulmonary function and related health outcomes. METHODS: Patients with EOS who reached skeletal maturity from 2005 to 2018 were identified in two registries including 32 centers. Thoracic height from T1 to T12 at skeletal maturity and Early Onset Scoliosis 24 Item Questionnaire (EOSQ-24) scores were collected. The EOSQ-24 domains included HRQoL of patients, parental impact, financial impact and patient and parental satisfaction. RESULTS: 469 patients (mean age: 14.9, female: 77.4%) were identified. 29% patients were of congenital etiology, 20.3% neuromuscular, 13.6% syndromic, 34.8% idiopathic, and 2.3% other. When patients were grouped by thoracic height at skeletal maturity, all EOSQ-24 domains increased after a threshold of 18 cm. When stratified by etiology, the 18 cm cutoff held for patients with congenital, neuromuscular and syndromic EOS. The cutoff for idiopathic EOS was 20 cm. For all patients, after the threshold was met, HRQoL continued to improve with increases in thoracic height at skeletal maturity. A subset of 169 patients for which arm span measurements were available was also identified and their thoracic heights were normalized. When grouped by the percentage of expected thoracic height attained, EOSQ-24 domains increased after a threshold of 80%. CONCLUSIONS: Once 18 cm of actual thoracic height or 80% of expected thoracic height is achieved, HRQoL continues to improve as thoracic height increases in skeletally mature patients with non-idiopathic EOS. Patients with idiopathic EOS had a higher threshold, possibly due to their larger average size and higher care giver expectations for HRQoL. LEVEL OF EVIDENCE: Level III.

Persistence and Progression of Airway Obstruction in Children With Early Onset Scoliosis.

BACKGROUND: Obstructive lung disease occurs in 30% of children with early onset scoliosis (EOS); changes in degree of airway obstruction over time have not been reported. METHODS: Longitudinal patterns of incidental, persistent, and progressive airway obstruction were retrospectively analyzed in a cohort of children with EOS with at least 1 forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) value <85% on serial spirometric assessments over a ≥3-year observation period. The prevalence of clinical features and the severity of coronal and sagittal spine deformities for each group at the beginning and end of the study period were compared. RESULTS: Airway obstruction was incidental in 12 (24%) and persistent in 37 (76%) of 49 children with EOS. Twenty of 37 (54%) of those with persistent obstruction developed progressive airway obstruction. The decline in FEV1/FVC over 6±2 years was insignificant in the incidental group (4%±2%) and the persistent nonprogressive group (7%±4%) but significant in the progressive group (13%±4%, t test; P=0.002). In total, 29% of the 49 children at the onset and 57% at the end of the study had airway obstruction. The incidental, persistent nonprogressive, and progressive groups did not differ with regard to age, diagnosis distribution, or sex. The initial coronal curve size, apex, direction of the curve, and degree of kyphosis were statistically similar among the 3 groups. Coronal curve magnitude inversely correlated with FEV1/FVC at the end but not the beginning of the study (r=-0.19, P=0.002). Six of 19 responded to bronchodilator treatment, suggesting concurrent asthma. Airway obstruction did not relate to restrictive pulmonary abnormalities measured by FVC at first or last timepoints [slope=-0.076 (95% confidence interval, -0.99 to 0.038; P=0.19)]. Changes in degrees of airway obstruction and restrictive lung disease over time did not correlate [slope=-0.125 (95% confidence interval, -0.294 to 0.044; P=0.14)]. CONCLUSIONS: Children with EOS and progressive airway obstruction represent an important subgroup which may require new surgical and nonsurgical treatment strategies to prevent loss of lung function over time.

Adult outcomes of childhood bronchiectasis.

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.

A decade on: Follow-up findings of indigenous children with bronchiectasis.

OBJECTIVE: The sole prospective longitudinal study of children with either chronic suppurative lung disease (CSLD) or bronchiectasis published in the current era was limited to a single center. We sought to extend this study by evaluating the longer-term clinical and lung function outcomes and their associated risk factors in Indigenous children of adolescents from Australia, Alaska, and New Zealand who participated in our previous CSLD or bronchiectasis studies during 2004-2010. METHODS: Between 2015 and 2018, we evaluated 131 out of 180 (72.8%) children of adolescents from the original studies at a single follow-up visit. We administered standardized questionnaires, reviewed medical records, undertook clinical examinations, performed spirometry, and scored available chest computed tomography scans. RESULTS: Participants were seen at a mean age of 12.3 years (standard deviation: 2.6) and a median of 9.0 years (range: 5.0-13.0) after their original recruitment. With increasing age, rates of acute lower respiratory infections (ALRI) declined, while lung function was mostly within population norms (median forced expiry volume in one-second = 90% predicted, interquartile range [IQR]: 81-105; forced vital capacity [FVC] = 98% predicted, IQR: 85-114). However, 43 out of 111 (38.7%) reported chronic cough episodes. Their overall global rating judged by symptoms, including ALRI frequency, examination findings, and spirometry was well (20.3%), stable (43.9%), or improved (35.8%). Multivariable regression identified household tobacco exposure and age at first ALRI-episode as independent risk factors associated with lower FVC% predicted values. CONCLUSION: Under our clinical care, the respiratory outcomes in late childhood or early adolescence are encouraging for these patient populations at high-risk of premature mortality. Prospective studies to further inform management throughout the life course into adulthood are now needed.

The Spinal Structure and Lung Function Relationship in an Untreated Nonambulatory Myelomeningocele Group of Patients.

INTRODUCTION: The relationship between spinal structure and respiratory function has been coined as thoracic insufficiency syndrome and is defined as the inability of the thorax to support normal respiratory function or lung growth. Little is known about what supports this relationship in untreated nonambulatory myelomeningocele patients. METHODS: A prospective cross-sectional study of nonambulatory myelodysplasia patients was performed. Anatomic, radiographic, and functional parameters were evaluated to validate the respiratory-spinal structure relationship. Thirty-one patients diagnosed with nonambulatory myelomeningocele fulfilled the inclusion criteria. RESULTS: The imaging study confirmed the spinal deformity. Lung functions measured in this patient population describe reduced lung volumes by CT lung volume reconstruction, reduced vital capacity by spirometry, and reduced total lung capacity by the nitrogen washout method. Together, these findings suggest moderate restrictive respiratory disease. The blood count study did not show evidence of anemia or other blood disturbances. Echocardiogram analysis did not show pulmonary hypertension in any patient. CONCLUSION: The data validate the relationship between spinal structure and lung function. However, there is no simple structural feature that could help to diagnose thoracic insufficiency syndrome. Thus, the diagnosis continues to be based on a combination of clinical findings and radiological and respiratory function evaluations. LEVEL OF EVIDENCE: Level III.

Clinical course of chronic suppurative lung disease and bronchiectasis in Alaska Native children.

INTRODUCTION: Alaska Native (AN) children from the Yukon Kuskokwim (YK) Delta region have high rates of chronic suppurative lung disease (CSLD), including bronchiectasis. We characterized the clinical progress of an AN adolescent cohort with CSLD/bronchiectasis, and estimated bronchiectasis prevalence trends in this region. METHODS: The original cohort comprised 41 AN children (originally aged 0.5-8 years) with CSLD/bronchiectasis, recruited between 2005 and 2008, with follow-up in 2015-2016. Clinical assessments, lung function, radiography, medical chart review, and spirometry were obtained. We also conducted data queries of bronchiectasis diagnoses in YK individuals born between 1990 and 2010 to estimate prevalence. RESULTS: Thirty-four (83%) of the original cohort aged 7.3-17.6 years were reviewed, of whom 14 (41%) had high-resolution computed tomography (HRCT)-confirmed bronchiectasis, eight (24%) had no evidence of bronchiectasis on HRCT scans, while 12 (35%) had not undergone HRCT scans. Annual lower respiratory tract infection (LRTI) frequency decreased with age, although 27 (79%) still had respiratory symptoms, including all with HRCT-confirmed bronchiectasis, who were also more likely than those without confirmed bronchiectasis to have recent wheeze (80 vs 25%, P = 0.005), auscultatory crackles (60 vs 0%, P < 0.001), and lower mean forced expiratory volume in 1-second/forced vital capacity ratio (73 vs 79%, P = 0.03). The bronchiectasis prevalence for YK AN people born during 2000-2009 was 7 per 1000 births, which was lower than previously reported. CONCLUSION: Despite reduced LRTI frequency, most AN children with CSLD/bronchiectasis had symptoms/signs of underlying lung disease as they entered adolescence. Close clinical follow-up remains essential for managing these patients as they transition to adulthood.

Early-Onset Spinal Deformity in Skeletal Dysplasias: A Multicenter Study of Growth-Friendly Systems.

PURPOSE: Severe, early-onset spinal deformity is common in patients with skeletal dysplasia. These deformities often present at young ages and are associated with significant pulmonary dysfunction. The objective of this study is to verify the effectiveness of growth-friendly spinal instrumentation systems in promoting growth in patients with skeletal dysplasia and early-onset kyphoscoliosis. METHODS: A retrospective, multicenter comparative cohort study was performed. Twenty-three patients identified to have a skeletal dysplasia (SKD) were evaluated for diagnosis, age at treatment, gender, and type of growing rod construct (spine vs. rib constructs). Patients were matched by age and construct type with similarly treated patients with early-onset scoliosis (CON) without skeletal dysplasia. Radiographic parameters including maximum coronal and sagittal Cobb angle with levels, T1-S1 height, and T1-T12 height were measured. RESULTS: T1-T12 (12.8 vs. 15.2 cm, p = .01) and T1-S1 (21.2 vs. 24.5 cm, p = .05) heights were significantly shorter for the SKD group at implantation, and kyphosis tended to be more severe in children with SKD (p = .80 and .07, respectively). Kyphosis did not improve with treatment. Scoliosis improved (p < .01), and ΔT1-T12 and ΔT1-S1 significantly increased in both groups (p < .01). Complication rates were similar between the two groups; however, patients with SKD had more intraoperative monitoring changes and hardware failures (p < .005). CONCLUSION: Although patients with SKD start with shorter spine lengths, gains in spine length appear to be comparable to other forms of EOS. Neuromonitoring changes and implant failures are more common in the SKD group. SIGNIFICANCE: The effectiveness of growth-friendly techniques in promoting growth in early-onset spinal deformities in patients with skeletal dysplasia has not been previously studied. We report the first comprehensive review of this topic. Growth-friendly techniques are an appropriate treatment option in this patient population.