VEPTR: Are We Reducing Respiratory Assistance Requirements?

Nossov, Sarah B.; Curatolo, Evan; Campbell, Robert M.; Mayer, Oscar H.; Garg, Sumeet; Cahill, Patrick J.

doi:10.1097/bpo.0000000000000986

Cited by 20 publications

(6 citation statements)

References 30 publications

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“…Although changes in Cobb angle after treatment of scoliosis have been studied extensively (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15), their relationships to tidal volumes to our knowledge have not been examined. In this study, we found only one significant association for Cobb angle, namely lumbar Cobb angle measurement with LHTV.…”

Section: Discussionmentioning

confidence: 99%

Quantitative Dynamic Thoracic MRI: Application to Thoracic Insufficiency Syndrome in Pediatric Patients

Tong¹,

Udupa²,

McDonough³

et al. 2019

Radiology

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Background: Available methods to quantify regional dynamic thoracic function in thoracic insufficiency syndrome (TIS) are limited. Purpose: To evaluate the use of quantitative dynamic MRI to depict changes in regional dynamic thoracic function before and after surgical correction of TIS. Materials and Methods: Images from free-breathing dynamic MRI in pediatric patients with TIS (July 2009-August 2015) were retrospectively evaluated before and after surgical correction by using vertical expandable prosthetic titanium rib (VEPTR). Eleven volumetric parameters were derived from lung, chest wall, and diaphragm segmentations, and parameter changes before versus after operation were correlated with changes in clinical parameters. Paired analysis from Student t test on MRI parameters and clinical parameters was performed to detect if changes (from preoperative to postoperative condition) were statistically significant. Results: Left and right lung volumes at end inspiration and end expiration increased substantially after operation in pediatric patients with thoracic insufficiency syndrome, especially right lung volume with 22.9% and 26.3% volume increase at end expiration (P = .001) and end inspiration (P = .002), respectively. The average lung tidal volumes increased after operation for TIS; there was a 43.8% and 55.3% increase for left lung tidal volume and right lung tidal volume (P , .001 for both), respectively. However, clinical parameters did not show significant changes from pre-to posttreatment states. Thoracic and lumbar Cobb angle were poor predictors of MRI tidal volumes (chest wall, diaphragm, and left and right separately), but assisted ventilation rating and forced vital capacity showed moderate correlations with tidal volumes (chest wall, diaphragm, and left and right separately). Conclusion: Vertical expandable prosthetic titanium rib operation was associated with postoperative increases in all components of tidal volume (left and right chest wall and diaphragm, and left and right lung tidal volumes) measured at MRI. Clinical parameters did not demonstrate improvements in postoperative tidal volumes.

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Section: Discussionmentioning

confidence: 99%

Quantitative Dynamic Thoracic MRI: Application to Thoracic Insufficiency Syndrome in Pediatric Patients

Tong¹,

Udupa²,

McDonough³

et al. 2019

Radiology

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“…There is data that among children with TIS due to EOS who require invasive or non-invasive mechanical ventilation for all or some part of the day/night. Twenty-four percent of 77 children in one series were able to reduce their respiratory support after spine surgery (36).…”

Section: Specific Pulmonary Questions Relevant To Children With Tis Amentioning

confidence: 97%

Clinical Issues for Pediatric Pulmonologists Managing Children With Thoracic Insufficiency Syndrome

Redding

2020

Front. Pediatr.

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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 multidisciplinary 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.

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“…134 The effect on pulmonary function metrics after spine surgery is variable; postoperative decrease in pulmonary function (e.g., from 61.9%-63.9% predicted FVC preoperatively to 56.3%-57.6% postoperatively), increase, and no change have all been reported. 22,23,30,31,74,[135][136][137][138][139][140] Postoperative reduction in the rate of decline of pulmonary function (e.g., from FVC decline of 5.31% per year preoperatively to 1.77% per year postoperatively) has also been described. Even with variable FVC outcomes, healthrelated quality of life pulmonary function subscores improve after growth-friendly instrumentation 140 (example in Figure 2).…”

Section: Chest Deformity Thoracic Insufficiency and Pulmonary Healthmentioning

confidence: 99%

“…[17][18][19][20][21][22][23] Hypotonic spinal curves continuously progress through childhood, and because conservative management is often unable to halt curve progression, spinal instrumentation is frequently indicated to preserve truncal balance, respiratory function, and quality of life. 17,[22][23][24][25][26][27][28][29][30][31][32] Though treatment of spinal deformity is well-established, operative management of hip instability has not generally been recommended based on the historically high probability of recurrent instability. [33][34][35] While hip instability is common in this population, hip subluxation rarely causes pain, and surgically stabilized hips often re-dislocate.…”

Section: Introductionmentioning

confidence: 99%

Best Practices for the Orthopaedic Care of Children with Spinal Muscular Atrophy: A Consensus Statement from the European Neuromuscular Centre Standard of Care Orthopaedic Working Group

Vitale¹,

Roye²,

Bloom³

et al. 2022

Journal of the Pediatric Orthopaedic Society of North America

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Purpose: Spinal muscular atrophy (SMA) is a genetic disease resulting in orthopaedic problems that warrant intervention. Recent genetic therapies appear to improve the natural course of this disease, prompting care providers to rethink treatment strategies. Insufficient high-quality evidence exists to guide optimal intervention in this new landscape. To address this, the European Neuromuscular Centre (ENMC) tasked an orthopaedic subgroup to update previous recommendations. Materials and Methods: A consensus statement on orthopaedic treatment of patients with SMA undergoing genetic therapy was sought via Delphi method. After review of the orthopaedic literature, three iterative surveys were administered from 2015-2016 to worldwide experts in orthopaedic care of children with SMA. Surveys identified important topics for optimal management, solicited responses regarding management and evaluation of identified topics, and clarified decision-making strategies and indications for evaluation and management, respectively. In light of new evidence of the long-term effects of gene therapy on SMA patients, an additional review of literature was used to contextualize the consensus recommendations. Results: Thirteen experts on orthopaedic management of SMA identified treatment of spine deformity, hip instability, and joint contractures as issues of importance. Experts recommended instrumentation in spinal deformity for major curves greater than 50 degrees, while monitoring pain, respiratory function, kyphosis, pelvic obliquity, functional status, trunk imbalance, and rib deformity. Preferential use of magnetically controlled growth constructs and performance of final fusion with skipped levels to accommodate intrathecal access were also endorsed. Additionally, experts advocated for surgical correction of hip instability and/or contractures that cause pain or functional impairment. Conclusions: Practice guidelines for management of scoliosis, hip instability and joint contractures were created to aid clinical decision-making in the era of genetic therapy.

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VEPTR: Are We Reducing Respiratory Assistance Requirements?

Abstract: Level III-prognostic.

Cited by 20 publications

References 30 publications

Quantitative Dynamic Thoracic MRI: Application to Thoracic Insufficiency Syndrome in Pediatric Patients

Quantitative Dynamic Thoracic MRI: Application to Thoracic Insufficiency Syndrome in Pediatric Patients

Clinical Issues for Pediatric Pulmonologists Managing Children With Thoracic Insufficiency Syndrome

Best Practices for the Orthopaedic Care of Children with Spinal Muscular Atrophy: A Consensus Statement from the European Neuromuscular Centre Standard of Care Orthopaedic Working Group

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