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neuromuscular scoliosis

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https://www.readbyqxmd.com/read/28905003/an-immediate-effect-of-pnf-specific-mobilization-on-the-angle-of-trunk-rotation-and-the-trunk-pelvis-hip-angle-range-of-motion-in-adolescent-girls-with-double-idiopathic-scoliosis-a-pilot-study
#1
A Stępień, K Fabian, K Graff, M Podgurniak, A Wit
BACKGROUND: Impairment of spine rotation is a key concept in several theories explaining the pathogenesis and progression of scoliosis. In previous studies, a more limited range of motion in scoliotic girls compared to their non-scoliotic peers was noted. The Trunk-Pelvis-Hip Angle measurement is a test used to assess the range of motion in the trunk-pelvis-hip complex in the transverse plane. The aim of this study was to assess an immediate effect of Proprioceptive Neuromuscular Facilitation specific mobilization (mPNF) on the angle of trunk rotation and Trunk-Pelvis-Hip Angle range of motion in adolescent girls with double scoliosis...
2017: Scoliosis and Spinal Disorders
https://www.readbyqxmd.com/read/28887279/sacral-alar-iliac-fixation-in-children-with-neuromuscular-scoliosis-minimum-5-year-follow-up
#2
Amit Jain, Brian T Sullivan, Anne Kuwabara, Khaled Kebaish, Paul D Sponseller
OBJECTIVE: The aim of our study was to investigate the 5-year outcomes of children with neuromuscular scoliosis treated with sacral-alar-iliac screws. METHODS: We reviewed clinical and radiographic records of patients ≤18 years old treated by 1 pediatric orthopaedic surgeon for neuromuscular scoliosis with spinal fusion using sacral-alar-iliac pelvic anchors. Thirty-eight patients, with a minimum 5-year radiographic follow-up (6.0 ± 1.2 years), were studied. Mean patient age was 13 ± 2...
September 5, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28885083/risk-factors-for-progressive-neuromuscular-scoliosis-requiring-posterior-spinal-fusion-after-selective-dorsal-rhizotomy
#3
Vijay M Ravindra, Michael T Christensen, Kaine Onwuzulike, John T Smith, Kyle Halvorson, Douglas L Brockmeyer, Marion L Walker, Robert J Bollo
OBJECTIVE Selective dorsal rhizotomy (SDR) via limited laminectomy is an effective treatment of lower-extremity spasticity in the pediatric population. Children with spasticity are also at risk for neuromuscular scoliosis; however, specific risk factors for progressive spinal deformity requiring posterior spinal fusion (PSF) after SDR are unknown. The authors' goal was to identify potential risk factors. METHODS The authors performed a retrospective cohort study of patients who underwent SDR via limited laminectomy between 2003 and 2014 and who had at least 1 year of follow-up...
September 8, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28870838/comparison-of-s2-alar-and-traditional-iliac-screw-pelvic-fixation-for-pediatric-neuromuscular-deformity
#4
Mark C Lee, Casey Jarvis, Matthew J Solomito, Jeffrey Thomson
Background Context Many pelvic fixation options exist for posterior spinal fusion of pediatric neuromuscular scoliosis, including standard iliac screws (SIS) or a more recently introduced S2-Alar (S2A) technique. However, little data exists comparing the clinical and radiographic outcomes of these techniques. Purpose Identify differences in clinical and radiographic outcomes for pediatric neuromuscular scoliosis patients treated with standard iliac screw (SIS) or S2-Alar (S2-A) pelvic fixation. Study Design/Setting Retrospective cohort study at a pediatric orthopaedic clinic...
September 1, 2017: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/28842365/spinal-deformity-changes-in-children-with-long-term-vertical-expandable-prosthetic-titanium-rib-veptr-treatment
#5
Andrea S Gantner, Lena Braunschweig, Konstantinos Tsaknakis, Heiko M Lorenz, Anna K Hell
BACKGROUND CONTEXT: In several studies, vertical expandable prosthetic titanium rib (VEPTR) implants have shown good scoliosis control in children with the longest reported follow-up (f/u) of 3.6 years. For growing rods, recent studies suggest a decreased efficiency of correction starting just after that time. To our knowledge, no long-term results of children with VEPTR treatment are available. PURPOSE: To evaluate spinal deformity in scoliotic children and to investigate correction potential of VEPTR implants at several time points of treatment, particularly after long-term f/u...
August 22, 2017: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/28837459/the-crankshaft-phenomenon
#6
Robert F Murphy, James F Mooney
The crankshaft phenomenon, a progressive rotational and angular spinal deformity that can occur after posterior spinal surgery, has been reported in pediatric patients with idiopathic, congenital, and neuromuscular scoliosis. In the skeletally immature patient, the crankshaft phenomenon is thought to occur secondary to continued growth of the anterior elements of the spine after solid posterior spinal fusion. The condition has also been reported in the setting of newer, so-called growth-friendly posterior distraction-based spinal instrumentation...
September 2017: Journal of the American Academy of Orthopaedic Surgeons
https://www.readbyqxmd.com/read/28777284/outcomes-of-primary-posterior-spinal-fusion-for-scoliosis-in-spinal-muscular-atrophy-clinical-radiographic-and-pulmonary-outcomes-and-complications
#7
Joshua B Holt, Lori A Dolan, Stuart L Weinstein
BACKGROUND: Spinal muscular atrophy (SMA) is a progressive neuromuscular disease commonly including progressive scoliosis resulting in severe deformity and negatively affecting pulmonary function. Surgical correction and stabilization of this progressive deformity is generally recommended; however, the timing and method of surgical fixation remains controversial. METHODS: Retrospective review of clinical, radiographic, and pulmonary function data from 16 children with SMA and surgically treated scoliosis between 1985 and 2013...
August 2, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28757364/ehlers-danlos-syndrome-kyphoscoliotic-type-due-to-lysyl-hydroxylase-1-deficiency-in-two-children-without-congenital-or-early-onset-kyphoscoliosis
#8
Fleur S van Dijk, Grazia M S Mancini, Alessandra Maugeri, Jan M Cobben
We report two children with Ehlers Danlos, kyphoscoliotic type confirmed by Lysyl Hydroxylase 1 deficiency due to bi-allelic PLOD1 mutations (kEDS-PLOD1) who were initially thought to have either a diagnosis of classical EDS (cEDS) or a neuromuscular disorder due to absence of (congenital) scoliosis. As the two patients reported here illustrate, patients with kEDS-PLOD1 do not always have a kyphoscoliosis present at birth or in the first year of life, neither do they necessarily develop kyphoscoliosis later in infancy...
October 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28754454/clinical-spectra-of-neuromuscular-manifestations-in-patients-with-lipodystrophy-a-multicenter-study
#9
Gulcin Akinci, Haluk Topaloglu, Tevfik Demir, Ayca Ersen Danyeli, Beril Talim, Fatma Ela Keskin, Pinar Kadioglu, Enez Talip, Canan Altay, Guzin Fidan Yaylali, Habib Bilen, Banu Nur, Leyla Demir, Huseyin Onay, Baris Akinci
Lipodystrophy is a heterogeneous group of disorders characterized by loss of adipose tissue. Here, we report on clinical spectra of neuromuscular manifestations of Turkish patients with lipodystrophy. Seventy-four patients with lipodystrophy and 20 healthy controls were included. Peripheral sensorimotor neuropathy was a common finding (67.4%) in lipodystrophic patients with diabetes. Neuropathic foot ulcers were observed in 4 patients. Drop foot developed in 1 patient with congenital generalized lipodystrophy type 1...
June 1, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28665821/spine-and-thoracic-height-measurements-have-excellent-interrater-and-intrarater-reliability-in-patients-with-early-onset-scoliosis
#10
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
#11
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...
September 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28660205/pneumothoraces-in-collagen-vi-related-dystrophy-a-case-series-and-recommendations-for-management
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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