Do Rib-Based Anchors Impair Chest Wall Motion in Early Onset Scoliosis (EOS)?

Purpose: There is a concern in pediatric surgery practice that rib-based fixation may limit chest wall motion in early onset scoliosis (EOS). The purpose of this study is to address the above concern by assessing the contribution of chest wall excursion to respiration before and after surgery. Methods: Quantitative dynamic magnetic resonance imaging (QdMRI) is performed on EOS patients (before and after surgery) and normal children in this retrospective study. QdMRI is purely an image-based approach and allows free breathing image acquisition. Tidal volume parameters for chest walls (CWtv) and hemi-diaphragms (Dtv) were analyzed on concave and convex sides of the spinal curve. EOS patients (1-14 years) and normal children (5-18 years) were enrolled, with an average interval of two years for dMRI acquisition before and after surgery. Results: CWtv significantly increased after surgery in the global comparison including all EOS patients (p < 0.05). For main thoracic curve (MTC) EOS patients, CWtv significantly improved by 50.24% (concave side) and 35.17% (convex side) after age correction (p < 0.05) after surgery. The average ratio of Dtv to CWtv on the convex side in MTC EOS patients was not significantly different from that in normal children (p=0.78), although the concave side showed the difference to be significant. Conclusion: Chest wall component tidal volumes in EOS patients measured via QdMRI did not decrease after rib-based surgery, suggesting that rib-based fixation does not impair chest wall motion in pediatric patients with EOS.

Virtual Growing Child (VGC): A general normative comparative system via quantitative dynamic MRI for quantifying pediatric regional respiratory anomalies with application in thoracic insufficiency syndrome (TIS).

Background: A normative database of regional respiratory structure and function in healthy children does not exist. Methods: VGC provides a database with four categories of regional respiratory measurement parameters including morphological, architectural, dynamic, and developmental. The database has 3,820 3D segmentations (around 100,000 2D slices with segmentations). Age and gender group analysis and comparisons for healthy children were performed using those parameters via two-sided t-testing to compare mean measurements, for left and right sides at end-inspiration (EI) and end-expiration (EE), for different age and gender specific groups. We also apply VGC measurements for comparison with TIS patients via an extrapolation approach to estimate the association between measurement and age via a linear model and to predict measurements for TIS patients. Furthermore, we check the Mahalanobis distance between TIS patients and healthy children of corresponding age. Findings: The difference between male and female groups (10-12 years) behave differently from that in other age groups which is consistent with physiology/natural growth behavior related to adolescence with higher right lung and right diaphragm tidal volumes for females(p<0.05). The comparison of TIS patients before and after surgery show that the right and left components are not symmetrical, and the left side diaphragm height and tidal volume has been significantly improved after surgery (p <0.05). The left lung volume at EE, and left diaphragm height at EI of TIS patients after surgery are closer to the normal children with a significant smaller Mahalanobis distance (MD) after surgery (p<0.05). Interpretation: The VGC system can serve as a reference standard to quantify regional respiratory abnormalities on dMRI in young patients with various respiratory conditions and facilitate treatment planning and response assessment. Funding: The grant R01HL150147 from the National Institutes of Health (PI Udupa).

Assessment of 3D hemi-diaphragmatic motion via free-breathing dynamic MRI in pediatric thoracic insufficiency syndrome.

Purpose: Thoracic insufficiency syndrome (TIS) affects ventilatory function due to spinal and thoracic deformities limiting lung space and diaphragmatic motion. Corrective orthopedic surgery can be used to help normalize skeletal anatomy, restoring lung space and diaphragmatic motion. This study employs free-breathing dynamic MRI (dMRI) and quantifies the 3D motion of each hemi-diaphragm surface in normal and TIS patients, and evaluates effects of surgical intervention. Materials and Methods: In a retrospective study of 149 pediatric patients with TIS and 190 healthy children, we constructed 4D images from free-breathing dMRI and manually delineated the diaphragm at end-expiration (EE) and end-inspiration (EI) time points. We automatically selected 25 points uniformly on each hemi-diaphragm surface, calculated their relative velocities between EE and EI, and derived mean velocities in 13 homologous regions for each hemi-diaphragm to provide measures of regional 3D hemi-diaphragm motion. T-testing was used to compare velocity changes before and after surgery, and to velocities in healthy controls. Results: The posterior-central region of the right hemi-diaphragm exhibited the highest average velocity post-operatively. Posterior regions showed greater velocity changes after surgery in both right and left hemi-diaphragms. Surgical reduction of thoracic Cobb angle displayed a stronger correlation with changes in diaphragm velocity than reduction in lumbar Cobb angle. Following surgery, the anterior regions of the left hemi-diaphragm tended to approach a more normal state. Conclusion: Quantification of regional motion of the 3D diaphragm surface in normal subjects and TIS patients via free-breathing dMRI is feasible. Derived measurements can be assessed in comparison to normal subjects to study TIS and the effects of surgery.

Assessment of Regional Functional Effects of Surgical Treatment in Thoracic Insufficiency Syndrome via Dynamic Magnetic Resonance Imaging.

BACKGROUND: Quantitative regional assessment of thoracic function would enable clinicians to better understand the regional effects of therapy and the degree of deviation from normality in patients with thoracic insufficiency syndrome (TIS). The purpose of this study was to determine the regional functional effects of surgical treatment in TIS via quantitative dynamic magnetic resonance imaging (MRI) in comparison with healthy children. METHODS: Volumetric parameters were derived via 129 dynamic MRI scans from 51 normal children (November 2017 to March 2019) and 39 patients with TIS (preoperatively and postoperatively, July 2009 to May 2018) for the left and right lungs, the left and right hemi-diaphragms, and the left and right hemi-chest walls during tidal breathing. Paired t testing was performed to compare the parameters from patients with TIS preoperatively and postoperatively. Mahalanobis distances between parameters of patients with TIS and age-matched normal children were assessed to evaluate the closeness of patient lung function to normality. Linear regression functions were utilized to estimate volume deviations of patients with TIS from normality, taking into account the growth of the subjects. RESULTS: The mean Mahalanobis distances for the right hemi-diaphragm tidal volume (RDtv) were -1.32 ± 1.04 preoperatively and -0.05 ± 1.11 postoperatively (p = 0.001). Similarly, the mean Mahalanobis distances for the right lung tidal volume (RLtv) were -1.12 ± 1.04 preoperatively and -0.10 ± 1.26 postoperatively (p = 0.01). The mean Mahalanobis distances for the ratio of bilateral hemi-diaphragm tidal volume to bilateral lung tidal volume (BDtv/BLtv) were -1.68 ± 1.21 preoperatively and -0.04 ± 1.10 postoperatively (p = 0.003). Mahalanobis distances decreased after treatment, suggesting reduced deviations from normality. Regression results showed that all volumes and tidal volumes significantly increased after treatment (p < 0.001), and the tidal volume increases were significantly greater than those expected from normal growth for RDtv, RLtv, BDtv, and BLtv (p < 0.05). CONCLUSIONS: Postoperative tidal volumes of bilateral lungs and bilateral hemi-diaphragms of patients with TIS came closer to those of normal children, indicating positive treatment effects from the surgical procedure. Quantitative dynamic MRI facilitates the assessment of regional effects of a surgical procedure to treat TIS. LEVEL OF EVIDENCE: Diagnostic Level II. See Instructions for Authors for a complete description of levels of evidence.

Correlation of Pulmonary Function to Novel Radiographic Parameters of Collapsing Parasol Deformity in Spinal Muscular Atrophy.

Spinal muscular atrophy (SMA) is a neuromuscular disease with manifestations of scoliosis, pulmonary function decline, and, uniquely, collapse of the ribs. Methods to quantify rib deformity and its impact on pulmonary function are sparse. The authors propose new radiographic measurements to quantify the aspect of SMA known as collapsing parasol deformity and correlate these measurements with pulmonary function. Twenty-eight full-spine radiographs of pediatric SMA patients were measured twice by 3 independent investigators, with 2 weeks separating each measurement. Radiographic measurements, demographics, spirometry results, and assisted ventilation rating were obtained. Twenty-one patients with spirometry metrics were assessed to correlate pulmonary function and spinal measurements. The intrarater intraclass correlation coefficient (ICC) for the measurements ranged from 0.706 to 0.99, and the interrater ICC ranged from 0.64 to 0.97. Eighteen of 19 variables had ICC values greater than 0.75 for inter- and intrarater reliability. Twenty-one patients with forced expiratory volume in 1 second and forced vital capacity were assessed in terms of these measurements. Ratio of the concave hemithoracic width at T6/convex hemithoracic width at T6 (P=.004) and ratio of convex vertical rib displacement at the apical rib/concave vertical rib displacement (P=.021) were both significantly correlated with decreased pulmonary function. No significant correlation was found examining the average vertical rib displacement at the apical rib. High inter-and intrarater reliability can be obtained in a variety of spinal measurements of SMA patients. Various measurements are correlated to diminished pulmonary function, specifically variables showing asymmetric changes in the chest cavity. [Orthopedics. 2021;44(2):e287-e293.].

Respiratory Trajectories in Type 2 and 3 Spinal Muscular Atrophy in the iSMAC Cohort Study.

OBJECTIVE: To describe the respiratory trajectories and their correlation with motor function in an international pediatric cohort of patients with type 2 and nonambulant type 3 spinal muscular atrophy (SMA). METHODS: This was an 8-year retrospective observational study of patients in the International SMA Consortium (iSMAc) natural history study. We retrieved anthropometrics, forced vital capacity (FVC) absolute, FVC percent predicted (FVC%P), and noninvasive ventilation (NIV) requirement. Hammersmith Functional Motor Scale (HFMS) and revised Performance of Upper Limb (RULM) scores were correlated with respiratory function. We excluded patients in interventional clinical trials and on nusinersen commercial therapy. RESULTS: There were 437 patients with SMA: 348 with type 2 and 89 with nonambulant type 3. Mean age at first visit was 6.9 (±4.4) and 11.1 (±4) years. In SMA type 2, FVC%P declined by 4.2%/y from 5 to 13 years, followed by a slower decline (1.0%/y). In type 3, FVC%P declined by 6.3%/y between 8 and 13 years, followed by a slower decline (0.9%/y). Thirty-nine percent with SMA type 2% and 9% with type 3 required NIV at a median age 5.0 (1.8-16.6) and 15.1 (13.8-16.3) years. Eighty-four percent with SMA type 2% and 80% with type 3 had scoliosis; 54% and 46% required surgery, which did not significantly affect respiratory decline. FVC%P positively correlated with HFMS and RULM scores in both subtypes. CONCLUSIONS: In SMA type 2 and nonambulant type 3, lung function declines differently, with a common leveling after age 13 years. Lung and motor function correlated in both subtypes. Our data further define the milder SMA phenotypes and provide information to benchmark the long-term efficacy of new treatments for SMA.

Understanding Respiratory Restrictions as a Function of the Scoliotic Spinal Curve in Thoracic Insufficiency Syndrome: A 4D Dynamic MR Imaging Study.

BACKGROUND: Over the past 100 years, many procedures have been developed for correcting restrictive thoracic deformities which cause thoracic insufficiency syndrome. However, none of them have been assessed by a robust metric incorporating thoracic dynamics. In this paper, we investigate the relationship between radiographic spinal curve and lung volumes derived from thoracic dynamic magnetic resonance imaging (dMRI). Our central hypothesis is that different anteroposterior major spinal curve types induce different restrictions on the left and right lungs and their dynamics. METHODS: Retrospectively, we included 25 consecutive patients with thoracic insufficiency syndrome (14 neuromuscular, 7 congenital, 4 other) who underwent vertical expandable prosthetic titanium rib surgery and received preimplantation and postimplantation thoracic dMRI for clinical care. We measured thoracic and lumbar major curves by the Cobb measurement method from anteroposterior radiographs and classified the curves as per Scoliosis Research Society (SRS)-defined curve types. From 4D dMRI images, we derived static volumes and tidal volumes of left and right lung, along with left and right chest wall and left and right diaphragm tidal volumes (excursions), and analyzed their association with curve type and major curve angles. RESULTS: Thoracic and lumbar major curve angles ranged from 0 to 136 and 0 to 116 degrees, respectively. A dramatic postoperative increase in chest wall and diaphragmatic excursion was seen qualitatively. All components of volume increased postoperatively by up to 533%, with a mean of 70%. As the major curve, main thoracic curve (MTC) was associated with higher tidal volumes (effect size range: 0.7 to 1.0) than thoracolumbar curve (TLC) in preoperative and postoperative situation. Neither MTC nor TLC showed any meaningful correlation between volumes and major curve angles preoperatively or postoperatively. Moderate correlations (0.65) were observed for specific conditions like volumes at end-inspiration or end-expiration. CONCLUSIONS: The relationships between component tidal volumes and the spinal curve type are complex and are beyond intuitive reasoning and guessing. TLC has a much greater influence on restricting chest wall and diaphragm tidal volumes than MTC. Major curve angles are not indicative of passive resting volumes or tidal volumes. LEVEL OF EVIDENCE: Level II-diagnostic.

Comparison of T1-S1 Spine Height of Postoperative Rib-based Implant Patients With Age-matched Peers.

BACKGROUND: Severe early-onset scoliosis leads to deficient spine height, thoracic growth inhibition, and ultimately pulmonary compromise. Rib-based growing instrumentation seeks to correct thoracic deformities, in part by correcting the spinal deformity, adding height, increasing thoracic volume, and allowing for continual spinal growth until maturity. However, the amount of growth in these patients relative to their peers is unknown. METHODS: Sixty patients who had undergone surgical intervention for the treatment of early-onset scoliosis were assessed via radiographic measurements of coronal T1-S1 height and major curve angle before implantation and again at most recent follow-up (minimum 2 years). T1-S1 measurements were then compared with age-matched peers to assess growth differences. Clinical information was examined for relevant parameters. RESULTS: The average age of our cohort at initial surgery and most recent follow-up was 4.4±3.8 and 10.0±4.4 years old, respectively. In this patient set, there was an average increase in T1-S1 height of 13.1±11.1 mm per year, with the majority of growth occurring in the first 2 years following implantation, and improvement in a major curve from 68±8 to 53±7 degrees. Overall, 77% of patients saw improvement in the major curve at most recent follow-up. Furthermore, a statistically significant greater percent of expected growth was seen in congenital compared with neuromuscular scoliosis (P<0.001). In addition, a weak negative correlation was observed between a number of surgical lengthenings and T1-S1 growth. CONCLUSIONS: Rib-based implant intervention has been shown to improve the major curve, but only improves growth potential to around 80% of expected growth. Scoliosis diagnosis type also influences growth rate potential, with congenital scoliosis patients being surgically treated earlier in life and having a growth rate approaching that of a healthy individual. LEVEL OF EVIDENCE: Level III-Case control.

Comprehensive Wound Risk Stratification of Rib-Based Distraction Instrumentation Procedures.

STUDY DESIGN: Single-center retrospective analysis of a prospectively collected registry. OBJECTIVES: Identify factors predictive of rib-based distraction (RBD) instrumentation wound complication. Create a risk stratification model for RBD instrumentation wound complication. SUMMARY OF BACKGROUND DATA: RBD instrumentation procedures have a high rate of wound complications, often requiring unplanned operative treatment. Currently, there is a relative lack of understanding of RBD complication risk factors compared with the comprehensive understanding of complication risk factors for other spine surgeries. METHODS: Between January 2011 and September 2015, patients treated with RBD instrumentation at a single institution were analyzed for risk factors associated with surgical wound complications that resulted in unplanned operative treatment. Univariate logistic regression determined predictors of wound complication and multivariate regression determined independent predictive factors; α = 0.10. RESULTS: A total of 122 patients aged 0-18 years underwent 140 implant surgeries in which 22 resulted in complications: 18 (82%) infectious and 4 (18%) noninfectious. Mean age at surgery was 5.2 years. Univariate analysis showed a correlation between wound complication rates and the following: male gender (p = .097), diapered patient with lower back incision (p = .004), bilateral procedure (p = .008), more than three incisions (p = .011), left iliac incision (p = .097), right iliac incision (p = .009), patient age ≤4 years (p = .10), and operative time >150 minutes (p = .079). Multivariate analysis identified the following independent predictors: age ≤ 4 years (p = .002), male gender (p = .04), number of skin incisions (p = .001), left iliac incision (p = .018), and nutritionally challenged (p = .044). The multivariate model predicted wound complications with an area under the receiver operating characteristic curve of 0.88. CONCLUSIONS: Knowledge of risk factors for RBD instrumentation wound complications can be used to construct patient risk models. This can identify patients at higher risk for complications and influence clinical decision making. LEVEL OF EVIDENCE: Level II.

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.

Quantitative dynamic MRI (QdMRI) Volumetric Analysis of Pediatric Patients with Thoracic Insufficiency Syndrome.

The lack of standardizable objective diagnostic measurement techniques is a major hurdle in the assessment and treatment of pediatric patients with thoracic insufficiency syndrome (TIS). The aim of this paper is to explore quantitative dynamic MRI (QdMRI) volumetric parameters derived from thoracic dMRI in pediatric patients with TIS and the relationships between dMRI parameters and clinical measurements. 25 TIS patients treated with vertical expandable prosthetic titanium rib (VEPTR) surgery are included in this retrospective study. Left and right lungs at end-inspiration and end-expiration are segmented from constructed 4D dMRI images. Lung volumes and excursion (or tidal) volumes of the left/right chest wall and hemi-diaphragms are computed. Commonly used clinical parameters include thoracic and lumbar Cobb angles and respiratory measurements from pulmonary function testing (PFT). 200 3D lungs in total (left & right, pre-operative & post-operative, end-inspiration & end-expiration) are segmented for analysis. Our analysis indicates that change of resting breathing rate (RR) following surgery is negatively correlated with that of QdMRI parameters. Chest wall tidal volumes and hemi-diaphragm tidal volumes increase significantly following surgery. Clinical parameter RR reduced after surgical treatment with P values around 0.06 but no significant differences were found on other clinical parameters. The significant increase in post-operative tidal volumes suggests a treatment-related improvement in lung capacity. The reduction of RR following surgery shows that breathing function is improved. The QdMRI parameters may offer an objective marker set for studying TIS, which is currently lacking.

An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age.

Although pulmonary function testing plays a key role in the diagnosis and management of chronic pulmonary conditions in children under 6 years of age, objective physiologic assessment is limited in the clinical care of infants and children less than 6 years old, due to the challenges of measuring lung function in this age range. Ongoing research in lung function testing in infants, toddlers, and preschoolers has resulted in techniques that show promise as safe, feasible, and potentially clinically useful tests. Official American Thoracic Society workshops were convened in 2009 and 2010 to review six lung function tests based on a comprehensive review of the literature (infant raised-volume rapid thoracic compression and plethysmography, preschool spirometry, specific airway resistance, forced oscillation, the interrupter technique, and multiple-breath washout). In these proceedings, the current state of the art for each of these tests is reviewed as it applies to the clinical management of infants and children under 6 years of age with cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheeze, using a standardized format that allows easy comparison between the measures. Although insufficient evidence exists to recommend incorporation of these tests into the routine diagnostic evaluation and clinical monitoring of infants and young children with cystic fibrosis, bronchopulmonary dysplasia, or recurrent wheeze, they may be valuable tools with which to address specific concerns, such as ongoing symptoms or monitoring response to treatment, and as outcome measures in clinical research studies.

Lung function distinguishes preschool children with CF from healthy controls in a multi-center setting.

RATIONALE: Conducting clinical trials in cystic fibrosis (CF) preschoolers has been limited by lack of sensitive lung function measures performed across sites. OBJECTIVES: (1) Assess feasibility and short-term reproducibility of spirometry, forced oscillometry (FO), and inductance plethysmography (IP) in a multi-center preschool population; (2) compare ability of each technique to differentiate lung function of CF preschoolers and controls; (3) evaluate longitudinal changes in lung function; (4) estimate sample sizes for future trials. METHODS: A longitudinal, multi-center study of CF preschoolers was conducted utilizing standardized equipment, rigorous site training, and centralized lung function data review. CF subjects participated in up to four study visits 6 months apart, plus a 2-week reproducibility visit. Controls had one study visit. RESULTS: Ninety-three CF subjects and 87 controls participated. Acceptability rates were lowest for spirometry (55%) and highest for IP (77%). Spirometry success increased with age and having a prior acceptable measurement. FEV(1) , FEV(0.5) , and FEF(25-75) were lower for CF subjects than for controls; spirometric z-scores declined with age. IP measures of thoracoabdominal asynchrony were greater for CF subjects than for controls. FO indices did not distinguish CF from controls. FEV(1) and FEV(0.5) are able to detect the smallest treatment effect for a given sample size. CONCLUSIONS: Spirometry appears more sensitive than IP or FO for detecting lung disease in CF preschoolers; spirometric indices decline with age. Future trials using spirometry should include a run-in period for training and require acceptable data prior to enrollment. However, near-normal spirometric measurements in CF preschoolers may lead to difficulty detecting a treatment effect.

Analysis of the associations between lung function and clinical features in preschool children with cystic fibrosis.

OBJECTIVE: To analyze cross-sectional and longitudinal associations between lung function measures and clinical features in a cohort of preschool children with cystic fibrosis (CF). METHODS: Longitudinal eight-center observational study of children with CF aged 36-60 months at enrollment, who underwent semiannual pulmonary function tests (PFTs) for up to 2 years consisting of spirometry (all 8 sites), forced oscillometry (FO, 5 sites), and measures of thoracoabdominal asynchrony using respiratory inductive plethysmography (IP, 5 sites). RESULTS: Ninety-three subjects were enrolled; 181 acceptable spirometry measurements from 71 subjects, 128 FO from 47 subjects, and 142 IP from 50 subjects were available for analysis. Cross sectional analyses did not detect an association between any PFT parameter at enrollment and Pseudomonas aeruginosa (Pa) status, CF gene mutation class, Wisconsin cough score, Shwachman score, environmental tobacco smoke exposure, family history of asthma, or nutritional indices. In longitudinal analyses, Pa infection within 6 months preceding enrollment was associated with a significantly greater rate of decline in z-scores for forced expiratory flow between 25 and 75% of forced vital capacity (FEF(25-75) ) (-1.3 vs. -0.4 Z scores/year, P = 0.024) and greater thoracoabdominal asynchrony measured by IP (mean phase angle difference 4.6°, P = 0.004). No other significant longitudinal associations were observed. CONCLUSIONS: Prior Pa infection is associated with a greater rate of decline in FEF(25-75) z-score and mild thoracoabdominal asynchrony in preschool children with CF. In this multicenter US study, significant associations between other lung function measures and clinical features were not detected.

Structure-respiration function relationships before and after surgical treatment of early-onset scoliosis.

BACKGROUND: Spine and chest wall deformities in children with early onset scoliosis (EOS) frequently impair respiratory function and postnatal growth of the lung. While a relationship between deformity and such impairment has been reported in children with adolescent idiopathic scoliosis it is not well understood in children with early-onset scoliosis (EOS). QUESTIONS/PURPOSES: We therefore describe (1) the preoperative relation between Cobb angle and forced vital capacity (FVC) in infants with EOS; (2) how changes in Cobb angle before and after surgery relate to changes in lung ventilation and perfusion in the right and left lungs. METHODS: We measured FVC in 10 children with EOS < 3 years old using the raised volume rapid thoracic compression (RVRTC) technique and correlated them with Cobb angles. We then measured right lung contributions to total lung ventilation and perfusion using lung scans before and 4 to 57 months after placement of vertical expandable prosthetic titanium ribs (VEPTRs) in 15 children with EOS and correlated changes in right lung function with postoperative changes in Cobb angles. RESULTS: In children 4 to 57 months of age, preoperative FVC (mean value, 83%; range, 63%-109% of predicted values) did not correlate with Cobb angles (mean value, 56º; range, 14°-120º). In children 1.8 to 11.5 years old, right lung ventilation and perfusion were abnormal in eight and seven children, respectively, but neither ventilation nor perfusion predictably normalized despite reductions in Cobb angle postoperatively. CONCLUSIONS: The data extend the age range of children with EOS whose Cobb angles correlate poorly with FVC preoperatively. The data are also consistent with reports that reduced Cobb angles after VEPTR insertion do not correlate with postoperative changes in respiratory function.

Lung function in 3-5-year-old children with cystic fibrosis.

It is well established that the lung disease of CF can occur early in life and may progress through the preschool years when accurate lung function assessment has been challenging to perform. We hypothesized that respiratory inductive plethysmography (RIP) and spirometry could be effectively performed in 3-6-year-old children and could be used to assess both longitudinal changes in lung function and the acute changes that occur during exacerbation of pulmonary disease. Both RIP and spirometry were equally feasible; however, the success rate for spirometry gradually increased with age to become higher than that for RIP in the 6-year-old subjects. Forty-four subjects were studied longitudinally and demonstrated significant increases in FVC, FEV(1), and FEV(0.5), but not in FEF(25-75) or RIP variables. There were significant differences in FVC, FEV(1), and phase angle (a measure of thoracoabdominal asynchrony) during exacerbations of lung disease. Although both RIP and spirometry were able to show differences in lung function in subjects with acute clinical worsening, spirometry was more robust in demonstrating change in lung function longitudinally and in children who had an exacerbation of lung disease.

Assessment and management of fatigue and dyspnea in pediatric palliative care.

Fatigue is one of the most prevalent symptoms in patients with a life-threatening illness. Untreated, fatigue can impair quality of life and prohibit addressing practical needs, psychosocial and spiritual distress, and opportunities for growth and closure at life's end. To this end addressing fatigue is a crucial component of the provision of effective palliative care. Dyspnea is the sensation of breathlessness. The challenge in treating it, however, is that it can come from various different abnormalities so understanding the underlying disorder and the acute abnormality are critical. With that understanding several different treatments can be offered to treat the cause of the dyspnea or palliate the symptom itself.