Early-onset scoliosis (EOS) describes a wide array of diagnoses and deformities exposed to growth. This potentially life-threatening condition is still 1 of the biggest challenges in pediatric ...orthopaedics. The enlightenment of Bob Campbell’s thoracic insufficiency syndrome concept and the negative impact of the earlier short and straight spine fusion approach on respiratory function and survival have fueled the evolution of EOS care. Despite all the progress made, growth-friendly spine surgery remains to be a burden to patients and caregivers. Even down-sized implants and remote-controlled noninvasive rod expansions do not omit unexpected returns to the operating roomfailures of foundations, rod breakage, difficulties to keep the sagittal balance, progressive transverse plane deformities, stiffening, and the need for final instrumented fusion are still common. However, past experience and the current multitude of surgical strategies and implants have sharpened the decision-making process, patients with thoracic insufficiency syndrome require earliest possible vertical expandable prosthetic titanium ribs application. Flexible deformities below 60 degrees, with normal spinal anatomy and without thoracic involvement, benefit from serial Mehta casting which revived as a long available but not-used strategy. In case of progression, standard double growing rods or—if available, affordable, and applicable—magnetically controlled motorized rods provide deformity control and growth promotion. Shilla growth-guiding technique is a less costly alternative. Its lack of stiff lengthening boxes or actuators may be beneficial in difficult deformities. Anterior convex flexible tethering promises benefits of sparing the trunk muscles and keeping mobility. However, this step towards a true nonfusion concept has yet to stand the test of broad clinical application.
•We report an update on standards of care recommendations for spinal muscular atrophy.•The paper provides a review of the recent literature.•Expert opinion is provided where there was not enough ...published evidence.
Spinal muscular atrophy (SMA) is a severe neuromuscular disorder due to a defect in the survival motor neuron 1 (SMN1) gene. Its incidence is approximately 1 in 11,000 live births. In 2007, an International Conference on the Standard of Care for SMA published a consensus statement on SMA standard of care that has been widely used throughout the world. Here we report a two-part update of the topics covered in the previous recommendations. In part 1 we present the methods used to achieve these recommendations, and an update on diagnosis, rehabilitation, orthopedic and spinal management; and nutritional, swallowing and gastrointestinal management. Pulmonary management, acute care, other organ involvement, ethical issues, medications, and the impact of new treatments for SMA are discussed in part 2.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
This retrospective study analyses 23 children treated with vertical expandable prosthetic titanium rib (VEPTR) for correction of non-congenital early onset spine deformities. After the index ...procedure (IP), the device was lengthened at 6-month intervals. The average (av) age at the time of IP was 6.5 years (1.11–10.5). The av follow-up time was 3.6 years (2–5.8). Diagnosis included 1 early onset idiopathic scoliosis, 11 neuromuscular, 2 post-thoracotomy scoliosis, 1 Sprengel deformity, 2 hyperkyphosis, 1 myopathy and 5 syndromic. Surgeries (187) included 23 IPs, av 6.5 (4–10) device expansions per patient (149) and 15 unplanned surgeries. 23 complications (0.13 per surgery) included 10 skin sloughs, 5 implant dislocations, 2 rod breakages and 6 infections. Coronal Cobb angle was av 68° (11°–111°), at follow-up av 54° (0°–105°). Pelvic obliquity was av 33° (13°–60°), at follow-up av 16° (0°–42°). T1 tilt was av 29° (5°–84°), two remained unchanged, the remainder improved 10°–68°. Sagittal plane: All but two had stable profiles, two hyperkyphosis of 110°/124° improved to 56°/86°. Space available for lung ratio was less than 90% in ten before the IP, improved in nine and deteriorated in one. Originally designed for thoracic insufficiency syndromes related to rib and vertebral anomalies, VEPTR proved to be a valuable alternative to dual growing rods for non-congenital early onset spine deformities. The complication rate was lower, the control of the sagittal plane and the pelvic obliquity was as good, but the correction of the coronal plane deformity was less than growing rods. However, VEPTR’s spine-sparing approach might provoke less spontaneous spinal fusion and ease the final correction at maturity.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Whole-body lifting strategies could be derived from conventional video recordings using the Stoop-Squat-Index, which quantifies the ratio between trunk forward lean and lower extremity joint flexion ...from 0 (full squat) to 100 (full stoop). The purpose of this study was to compare Stoop-Squat-Indices derived from conventional video recordings to those from a three-dimensional marker-based motion capture system and to evaluate interrater and intrarater reliability of the video-based approach.
Thirty healthy participants lifted a 5-kg box under different conditions (freestyle, squat, stoop). Kinematic data were recorded using a Vicon motion capture system (serving as reference standard) and an iPad camera. Stoop-Squat-Indices over the entire lifting cycle were derived separately from both approaches. Agreement was assessed using mean differences (video minus motion capture) and limits of agreement. Reliability was investigated by calculating intraclass correlation coefficients (ICC) and minimal detectable changes (MDC) over the course of the lifting cycle. Systematic errors were identified with Statistical Parametric Mapping-based T-tests.
Systematic errors between the video-based and the motion capture-based approach were observed among all conditions. Mean differences in Stoop-Squat-Indices over the lifting cycle ranged from −6.9 to 3.2 (freestyle), from −1.8 to 5.3 (squat) and from −2.8 to −1.1 (stoop). Limits of agreement were lower when the box was close to the floor, and higher towards upright standing. Reliability of the video-based approach was excellent for most of the lifting cycle, with ICC above 0.995 and MDC below 3.5.
These findings support using a video-based assessment of Stoop-Squat-Indices to quantify whole-body lifting strategy in field.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Virtual reality (VR), coupled with motion tracking, can investigate walking in a controlled setting while applying various walking challenges. The purpose of this review was to summarize the evidence ...on consequences of VR on biomechanical gait parameters in children with cerebral palsy. MEDLINE, Embase and Web of Science were searched. Among 7.574 studies, screened by two independent reviewers, seven studies were included, analyzing treadmill (
= 6) or overground walking (
= 1) under VR. Most frequently reported were the spatiotemporal parameters walking speed, stride length, step width, stance phase, and the kinematic parameters range of knee flexion and peak ankle dorsiflexion. However, methodological approaches and reporting of the results were inconsistent among studies. This review reveals that VR can complement information gained from clinical gait analysis. However, this is still an emerging field of research and there is limited knowledge on the effect of VR on gait parameters, notably during overground walking.
Study Design. A multicenter retrospective review of consecutive series of patients. Objective. Long-term experience with using the magnetically controlled growing rods (MCGR) to treat patients with ...deformity in the growing spine to the conclusion of treatment with posterior spine fusion. Summary of Background Data. MCGR treatment for growing spine gained popularity with paucity of long-term follow up data. We hypothesized that final fusion might be more effective in bringing additional correction of the spine deformity after treatment with MCGR than that reported after traditional growing rods (TGR) due to less scarring and auto-fusion. Methods. Retrospective review of 47 patients with varied etiology, treated between 2011 and 2017 which graduated treatment were followed in five academic medical centers for average of 50 months (range, 10–88). Results. The initial mean coronal deformity of 69.6° (95% CI 65–74) was corrected to 40° (95% CI 36–40) immediately after the MCGR implantation but progressed to 52.8° (95% CI 46–59) prior to the final surgery ( P < 0.01). Nevertheless, thoracic spine growth (T1–T12 height) improved from 187.3 mm (95% CI 179–195) following index surgery to 208.9 mm (95% CI 199–218) prior to final fusion ( P < 0.01). Significant correction and spinal length were obtained at final fusion, but metallosis was a frequent observation (47%, 22/47). The average growth rate was 0.5 mm/month (95% CI 0.3–0.6). The overall complication rate within our cohort was 66% (31/47) with 45% (21/47) of unplanned returns to the operating theater. 32% (15/47) of the patients had an implant related complication. Unplanned surgery was highly correlated with thoracic kyphosis greater than 40° (OR 5.42 95% CI 1.3–23). Conclusion. Treatment of growing spine deformities with MCGR provides adequate control of spine deformity it is comparable to previously published data about TGR. The overall high complications rate over time and specifically implant related complications. Level of Evidence: 4
The authors – who all practice in high-volume paediatric (neuro- and spine) orthopaedic units – highlight the current anatomical, biomechanical and surgical understanding of these deformities and the ...practical implications for conservative and surgical strategies.1-5 The natural history of spine deformities in patients with cerebral palsy is closely linked to their gross motor functioning. ...caregivers must be broadly informed at an early stage of the deformity that conservative therapy is usually a ‘time buyer’ but is unlikely to prevent progression and a surgical intervention in the mid- and long term. ...recently the sagittal spinal profile and the orientation of the pelvis have not gained as much attention in patients with neuromuscular spine deformities as compared to otherwise healthy ambulators.
Although the relevance of understanding spinal kinematics during functional activities in patients with complex spinal deformities is undisputed among researchers and clinicians, evidence using skin ...marker-based motion capture systems is still limited to a handful of studies, mostly conducted on healthy subjects and using non-validated marker configurations. The current study therefore aimed to explore the validity of a previously developed enhanced trunk marker set for the static measurement of spinal curvature angles in patients with main thoracic adolescent idiopathic scoliosis. In addition, the impact of inaccurate marker placement on curvature angle calculation was investigated.
Ten patients (Cobb angle: 44.4±17.7 degrees) were equipped with radio-opaque markers on selected spinous processes and underwent a standard biplanar radiographic examination. Subsequently, radio-opaque markers were replaced with retro-reflective markers and the patients were measured statically using a Vicon motion capture system. Thoracolumbar/lumbar and thoracic curvature angles in the sagittal and frontal planes were calculated based on the centers of area of the vertebral bodies and radio-opaque markers as well as the three-dimensional position of the retro-reflective markers. To investigate curvature angle estimation accuracy, linear regression analyses among the respective parameters were used. The impact of inaccurate marker placement was explored using linear regression analyses among the radio-opaque marker- and spinous process-derived curvature angles.
The results demonstrate that curvatures angles in the sagittal plane can be measured with reasonable accuracy, whereas in the frontal plane, angles were systematically underestimated, mainly due to the positional and structural deformities of the scoliotic vertebrae. Inaccuracy of marker placement had a greater impact on thoracolumbar/lumbar than thoracic curvature angles. It is suggested that spinal curvature measurements are included in marker-based clinical gait analysis protocols in order to enable a deeper understanding of the biomechanical behavior of the healthy and pathological spine in dynamic situations as well as to comprehensively evaluate treatment effects.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
IntroductionChronic postsurgical pain (CPSP) is defined as pain that persists after a surgical procedure and has a significant impact on quality of life. Previous studies show the importance of ...psychological factors in CPSP, yet the majority of studies focused solely on negative emotions. This longitudinal observational study aims to broaden this knowledge base by examining the role of emotional state, emotion variability, emotion regulation and emotion differentiation on the child and the parent level for the development CPSP, and to describe pain and emotion-related trajectories following surgery.Methods and analysisWe intend to include 280 children and adolescents aged 8–18 years with a planned orthopaedic surgery and their parents. A total of five assessment time points is planned: 3 weeks before surgery (baseline), 2 weeks after surgery (post) and 3 months (follow-up (FU) 1), 6 months and 12 months after surgery. At baseline and post only, children and parents are asked to complete a daily diary thrice a day for a week where they rate their current emotional state and their pain severity (children only). Emotional state ratings will be used to calculate indices of emotion variability, emotion regulation and emotion differentiation. Children and parents will complete questionnaires at each time point, including measures on quality of life, social support, sleep, and symptoms of anxiety and depression.To predict development of CPSP, generalised linear regression models will be used, resulting in ORs and 95% CIs. Pearson product-moment correlations between predictors and outcomes will be evaluated at each time point. The primary outcome of the prediction model is CPSP at FU1. For the trajectory analysis, the classification method K-means for longitudinal data will be used to determine clusters in the data.Ethics and disseminationThe Ethics Committee of the Canton of Zurich, Switzerland, has approved the study (ID: 2023-01475). Participants will be compensated, and a dissemination workshop will be held.Trial registration numberNCT05816174.
BACKGROUND:The final strategy for graduates from growth-sparing surgery is challenging. The purpose of this study was to evaluate the radiographic outcome and complications of patients with early ...onset scoliosis (EOS) who have graduated from vertical expandable prosthetic titanium rib (VEPTR) treatment, either undergoing final fusion surgery or following a nonfusion approach.
METHODS:Final treatment for VEPTR graduates was divided in “VEPTR in situ without final fusion,” “removal of VEPTR without final fusion,” and “removal of VEPTR with instrumented final fusion.” Radiographic evaluations included main coronal Cobb angle and main kyphosis pre and post VEPTR implantation, at the end of implant lengthening, after final fusion (if applicable), and at latest follow-up. Complications during VEPTR treatment and in case of final fusion were reported.
RESULTS:In total, 34 VEPTR graduates were included; 17 underwent final fusion surgery, and 17 followed a nonfusion strategy. Average coronal Cobb angle before VEPTR implantation was 70±23 degrees (range, 21 to 121 degrees), and 65±22 degrees (range, 17 to 119 degrees) at latest follow-up. Average main kyphosis angle was 53±27 degrees (range, 6 to 137 degrees) before VEPTR, and 69±34 degrees (range, 10 to 150 degrees) at latest follow-up. There was a 41% complication rate with final fusion surgery.
CONCLUSIONS:There is a high complication rate during VEPTR treatment and with final fusion surgery. The stiffness of the spine and thorax allow for only limited correction when performing a final instrumented spondylodesis. Avoiding final fusion may be a viable alternative in case of good coronal and sagittal alignment.
LEVEL OF EVIDENCE:Level IV—therapeutic.
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