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neuromuscular scoliosis

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https://www.readbyqxmd.com/read/28665821/spine-and-thoracic-height-measurements-have-excellent-interrater-and-intrarater-reliability-in-patients-with-early-onset-scoliosis
#1
Nicole Michael, Patrick Carry, Mark Erickson, Nikki Bloch, Steven Gibbons, Courtney O'Donnell, Sumeet Garg
STUDY DESIGN: Reproducibility of measurements OBJECTIVE.: This study investigates the reliability and standard error of measurement of spine and thoracic height radiographic measurements in patients with early onset scoliosis (EOS). SUMMARY OF BACKGROUND DATA: Spine and thoracic height radiographic measurements are often used as a surrogate for pulmonary development in patients with EOS. There is limited literature validating the reliability of spine and thoracic height measurements in the EOS population...
June 29, 2017: Spine
https://www.readbyqxmd.com/read/28662944/skeletal-muscle-channelopathies-rare-disorders-with-common-pediatric-symptoms
#2
Emma Matthews, Arpana Silwal, Richa Sud, Michael G Hanna, Adnan Y Manzur, Francesco Muntoni, Pinki Munot
OBJECTIVE: To ascertain the presenting symptoms of children with skeletal muscle channelopathies to promote early diagnosis and treatment. STUDY DESIGN: Retrospective case review of 38 children with a skeletal muscle channelopathy attending the specialist pediatric neuromuscular service at Great Ormond Street Hospital over a 15-year period. RESULTS: Gait disorder and leg cramps are a frequent presentation of myotonic disorders (19 of 29). Strabismus or extraocular myotonia (9 of 19) and respiratory and/or bulbar symptoms (11 of 19) are common among those with sodium channelopathy...
June 26, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28660205/pneumothoraces-in-collagen-vi-related-dystrophy-a-case-series-and-recommendations-for-management
#3
Kristin L Fraser, Scott Wong, A Reghan Foley, Sameer Chhibber, Carsten G Bönnemann, Daniel J Lesser, Carla Grosmann, Anne Rutkowski
Collagen VI-related dystrophy (collagen VI-RD) is a rare neuromuscular condition caused by mutations in the COL6A1, COL6A2 or COL6A3 genes. The phenotypic spectrum includes early-onset Ullrich congenital muscular dystrophy, adult-onset Bethlem myopathy and an intermediate phenotype. The disorder is characterised by distal hyperlaxity and progressive muscle weakness, joint contractures and respiratory insufficiency. Respiratory insufficiency is attributed to chest wall contractures, scoliosis, impaired diaphragmatic function and intercostal muscle weakness...
April 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28614287/nasal-swab-screening-for-staphylococcus-aureus-in-spinal-deformity-patients-treated-with-growing-rods
#4
Scott J Luhmann, June C Smith
BACKGROUND: Surgical-site infections are one of the most concerning complications in patients treated with growing rods (GR). The purpose of this study was to evaluate the use of preoperative screening for Staphylococcus aureus (SA) for all growing spine procedures, and if this would permit alteration of prophylactic antibiotics to cover the identified resistances. METHODS: All patients were identified who had SA screening during the course of GR treatment. In otal, 34 patients [23 neuromuscular (NMS), 4 congenital, 4 idiopathic scoliosis (IS), and 3 syndromic] were identified who had 111 preoperative screenings [79 lengthenings, 23 insertions, 6 revisions, and 3 conversions to posterior spinal fusions (PSF)]...
June 13, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28565896/effects-of-magnetically-controlled-growing-rods-surgery-on-pulmonary-function-in-young-subjects-with-spinal-muscular-atrophy-type-2-and-other-neuromuscular-scoliosis
#5
Luca Colombo, Carlotta Martini, Chiara Bersanini, Francesca Izzo, Jorge H Villafañe, Pedro Berjano, Claudio Lamartina
BACKGROUND: The aim of this study was to determine the effects of magnetically controlled growing rods surgery (MCGRS) of the scoliosis on pulmonary function in children with neuromuscular scoliosis. METHODS: Seven patients, 85.7% female (mean ± SD age: 6.7±1.2 years), with neuromuscular scoliosis (4 SMA II, 2 congenital myopathies and 1 VACTER syndrome), received MCGRS of the spine in the thoraco-lumbar area. The outcome measures were clinical features and pulmonary function (forced vital capacity [FVC] and forced expiratory volume in 1st second [FEV1], were collected...
May 31, 2017: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/28546128/predictive-factors-of-intraoperative-cell-salvage-during-pediatric-scoliosis-surgery-cell-saver-during-scoliosis-surgery-in-children
#6
Daphné Michelet, Florence Julien-Marsollier, Julie Hilly, Thierno Diallo, Christophe Vidal, Souhayl Dahmani
INTRODUCTION: blood saving strategy during spinal surgery in children often includes recombinant erythropoietin (rEPO) and antifibrinolytic therapapy (AFT). The aim of this study was to investigate the efficacy of intraoperative blood salvage in decreasing homologous blood transfusion. MATERIAL AND METHODS: using the prospective data from patients operated during a one year period for scoliosis correction, we calculate the predictable hematocrit at day postoperative 1 without the use of blood salvage and compare it to the target hematocrit transfusion according to patient's status...
May 22, 2017: Anaesthesia, Critical Care & Pain Medicine
https://www.readbyqxmd.com/read/28474006/curve-flexibility-in-cerebral-palsy-related-neuromuscular-scoliosis-does-the-intraoperative-prone-radiograph-reveal-more-flexibility-than-preoperative-radiographs
#7
Zubair Chaudry, John T Anderson
BACKGROUND: Spinal flexibility is determined preoperatively by manipulating the spine and assessing, radiographically, to what extent the amount of deformity reduces. Quantifying spinal flexibility is important when determining the approach to the planned operation in order to achieve the most optimal spinal correction and balance. Currently, supine traction radiography is a popular method used in patients with severe, cerebral palsy-related neuromuscular scoliosis. The different methods for determining spinal flexibility have been studied extensively in the adolescent idiopathic scoliosis population...
2017: Scoliosis and Spinal Disorders
https://www.readbyqxmd.com/read/28446219/the-burden-epidemiology-costs-and-treatment-for-duchenne-muscular-dystrophy-an-evidence-review
#8
REVIEW
S Ryder, R M Leadley, N Armstrong, M Westwood, S de Kock, T Butt, M Jain, J Kleijnen
BACKGROUND: Duchenne Muscular Dystrophy (DMD) is a rapidly progressive, lethal neuromuscular disorder, present from birth, which occurs almost exclusively in males. We have reviewed contemporary evidence of burden, epidemiology, illness costs and treatment patterns of DMD. This systematic review adhered to published methods with information also sought from the web and contacting registries. Searches were carried out from 2005 to June 2015. The population of interest was individuals with clearly defined DMD or their carers...
April 26, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28442963/sugammadex-to-reverse-neuromuscular-blockade-and-provide-optimal-conditions-for-motor-evoked-potential-monitoring
#9
Mehdi Trifa, Senthil Krishna, Ajay D'Mello, Mumin Hakim, Joseph Drew Tobias
Sugammadex is a novel pharmacologic agent, which reverses neuromuscular blockade (NMB) via a mechanism that differs completely from acetylcholinesterase inhibitors. By encapsulating rocuronium, sugammadex can provide recovery of neuromuscular function even when there is a profound degree of NMB. We report anecdotal experience with the use of sugammadex to reverse NMB to facilitate intraoperative neurophysiological monitoring (motor evoked potentials) in an adolescent with scoliosis during posterior spinal fusion...
April 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28441316/hemolysis-following-intraoperative-cell-salvage-replacement-in-a-scoliosis-patient-with-sickle-cell-trait-a-case-report
#10
Daniel You, Alejandro Peiro Garcia, Fabio Ferri-de-Barros, David Parsons
STUDY DESIGN: Case Report. OBJECTIVE: To describe a novel presentation of acute renal failure associated with hemolysis following intraoperative cell salvage (ICS) in a neuromuscular scoliosis patient with sickle cell trait (SCT). SUMMARY OF BACKGROUND DATA: Hemolysis associated acute renal failure following ICS in patients with SCT has not been previously reported. Sickle cell disease is regarded as a relative contraindication for ICS due to the risk of red blood cell sickling in the hypoxic cell saver reservoir...
April 24, 2017: Spine
https://www.readbyqxmd.com/read/28318781/spinal-fusion-in-a-patient-with-fukuyama-congenital-muscular-dystrophy
#11
Kaori Hino, Mitsumasa Fukuda, Tadao Morino, Tadanori Ogata, Masanori Ito, Eiichi Ishii
Many studies have evaluated surgical treatments for spinal deformities in patients with neuromuscular disease. However, few reports have described patients with Fukuyama congenital muscular dystrophy (FCMD). A 13-year-old boy with FCMD was unable to sit for long periods or sleep in the supine position because of progressive scoliosis. His Cobb angle worsened from 27° to 41° in 5months. He underwent standard posterior spinal fusion and pedicle-screw-alone fixation from T5 to S1. Postoperatively, his Cobb angle improved from 41° to 25° without exacerbation for 2years...
March 16, 2017: Brain & Development
https://www.readbyqxmd.com/read/28314995/rate-of-complications-due-to-neuromuscular-scoliosis-spine-surgery-in-a-30-years-consecutive-series
#12
Francesco Turturro, Antonello Montanaro, Cosma Calderaro, Luca Labianca, Vincenzo Di Sanzo, Andrea Ferretti
PURPOSE: The aim of this study was to evaluate the rate of intraoperative and postoperative complications in a large series of patients affected by neuromuscular scoliosis. METHODS: It was a monocentric retrospective study. In this study have been considered complications those events that significantly affected the course of treatment, such as getting the hospital stay longer, or requiring a subsequent surgical procedure, or corrupting the final result of the treatment...
March 17, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28296737/incidence-influencing-factors-and-prognostic-impact-of-intraoperative-massive-blood-loss-in-adolescents-with-neuromuscular-scoliosis-a-strobe-compliant-retrospective-observational-analysis
#13
Rui Jia, Na Li, Bi-Yun Xu, Wei Zhang, Xiao-Ping Gu, Zheng-Liang Ma
Factors influencing massive blood loss for neuromuscular scoliosis (NMS) patients.Despite advances in surgical and anesthetic techniques, scoliosis surgery is still associated with intraoperative massive blood loss, which can result in postoperative mortality and morbidity. The aim of this study was to assess the incidence, influencing factors, and prognostic impact of intraoperative massive blood loss in adolescents with NMS.A retrospective review of adolescents who underwent posterior spinal instrumentation and fusion for NMS was performed...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28273239/correlation-between-the-degree-of-correction-of-neuromuscular-scoliosis-and-patient-quality-of-life
#14
David Gonçalves Nordon, Ariel Falbel Lugão, Lucas Castrillon Carmo Machado, Raphael Martus Marcon, Alexandre Fogaça Cristante, Tarcísio Eloy Pessoa de Barros, Olavo Biraghi Letaif
OBJECTIVE: There are few data on patient satisfaction with surgery for the correction of neuromuscular scoliosis or on the correlation between patient satisfaction and the degree of curve correction achieved by surgery. Our aim was to determine the correlations between both patient satisfaction and perception of quality of life and the degree of curve correction. METHODS: We interviewed 18 patients and administered a questionnaire that collected social and economic data and information about functional ability, comorbidities and satisfaction...
February 1, 2017: Clinics
https://www.readbyqxmd.com/read/28240718/a-peripherally-inserted-central-catheter-line-inserted-the-day-before-surgery-decreases-the-time-from-induction-to-incision-for-spinal-deformity-surgery-and-safely-provides-central-venous-access-during-surgery-a-pilot-study
#15
Anne E Stuedemann, Richard M Schwend, Valorie K Thomas, Julia M Leamon, Tammy S Lightner
Pediatric patients undergoing surgery for spinal deformity may benefit from central venous access to provide intraoperative monitoring and fluid resuscitation. For pediatric surgical patients requiring central access, we hypothesized that placing a peripherally inserted central catheter (PICC) line preoperatively should decrease time from induction of anesthesia to incision and result in improved patient safety and decreased operating room charges. This was a retrospective, nonrandomized, and case comparison study...
February 24, 2017: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/28177173/perioperative-considerations-in-infantile-neuroaxonal-dystrophy
#16
Jina L Sinskey, Robert S Holzman
Infantile neuroaxonal dystrophy is a rare neurological disorder that is universally fatal with life expectancy under 10 years. A 10-year-old boy with infantile neuroaxonal dystrophy and severe neuromuscular scoliosis underwent posterior spinal fusion following halo traction. He was successfully extubated to bilevel positive airway pressure on postoperative day 3 and discharged home on postoperative day 11. Infantile neuroaxonal dystrophy presents several perioperative challenges including concerns for difficult intubation and respiratory dysfunction...
March 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28156208/image-guided-intrathecal-baclofen-pump-catheter-implantation-a-technical-note-and-case-series
#17
Shenandoah Robinson, Faith C Robertson, Hormuzdiyar H Dasenbrock, Cormac P O'Brien, Charles Berde, Horacio Padua
OBJECTIVE Medically refractory spasticity and dystonia are often alleviated with intrathecal baclofen (ITB) administration through an indwelling catheter inserted in the lumbar spine. In patients with cerebral palsy, however, there is a high incidence of concomitant neuromuscular scoliosis. ITB placement may be technically challenging in those who have severe spinal deformity or who have undergone prior instrumented thoracolumbar fusion. Although prior reports have described drilling through the lumbar fusion mass with a high-speed bur, as well as IT catheter implantation at the foramen magnum or cervical spine, these approaches have notable limitations...
May 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28141685/veptr-implantation-to-treat-children-with-early-onset-scoliosis-without-rib-abnormalities-early-results-from-a-prospective-multicenter-study
#18
Ron El-Hawary, Muayad Kadhim, Michael Vitale, John Smith, Amer Samdani, John M Flynn
BACKGROUND: Thoracoplasty and insertion of vertical expandable prosthetic titanium rib (VEPTR) for thoracic and spine distraction has been found to be effective in the treatment of early-onset scoliosis (EOS) with ribs anomalies and congenital vertebral anomalies. The aim of this study was to evaluate the efficacy of VEPTR in preventing further progression of scoliosis without impeding spinal growth in children with progressive EOS without rib abnormalities. METHODS: This is a prospective, multicenter, observational cohort study...
January 30, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28119879/influence-of-the-proprioceptive-neuromuscular-facilitation-exercise-programs-on-idiopathic-scoliosis-patient-in-the-early-20s-in-terms-of-curves-and-balancing-abilities-single-case-study
#19
Byung-Ki Lee
The purpose of this study was to determine the influence of the proprioceptive neuromuscular facilitation (PNF) exercise programs for idiopathic scoliosis with a female patient in the early 20s in terms of her spinal curve and balancing abilities. The study subject was selected among 21-year-old female college students. There were no particular activities that the subject could not perform, but patient complained of difficulty in maintaining the standing position for a prolonged time. Patient chest X-ray results showed S-shaped curves tilted towards the left or right in the lumbar spine, thoracic spine, and cervical spine areas...
December 2016: Journal of Exercise Rehabilitation
https://www.readbyqxmd.com/read/28045856/a-multiparametric-alarm-criterion-for-motor-evoked-potential-monitoring-during-spine-deformity-surgery
#20
Martín J Segura, María E Talarico, Mariano A Noel
PURPOSE: This is a pilot study to compare changes in the amplitude, area below the curve, number of phases, duration, and latency of the intraoperative transcranial motor evoked potentials (TcMEP) for early detection of impending spinal cord injury. An empirical ratio calculated by a combination of the above-mentioned parameters was also assessed. METHODS: Intraoperative TcMEP recordings from five patients presenting with neuromuscular kyphoscoliosis, idiopathic scoliosis, achondroplasia and lumbar kyphosis, congenital kyphosis, and achondroplasia with cervical instability were reviewed...
January 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
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