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neuromuscular scoliosis

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https://www.readbyqxmd.com/read/29318413/evaluation-of-postoperative-fever-after-surgical-correction-of-neuromuscular-scoliosis-implication-on-management
#1
Mohamed Abdelhamid Ali Yousef, Scott Rosenfeld
BACKGROUND: Scoliosis is a common deformity in patients with neuromuscular disorders which usually necessitates surgical correction. Patients with neuromuscular scoliosis are characterized by increased incidence of associated medical co-morbidities and higher postoperative complication rate; therefore, these patients are often managed with a wide multidisciplinary care team. Postoperative fever is a frequent complication after surgery which is often routinely investigated using different workup tests to rule out infection...
January 9, 2018: European Spine Journal
https://www.readbyqxmd.com/read/29302566/bridging-the-gap-between-observation-and-brace-treatment-for-adolescent-idiopathic-scoliosis
#2
Eric Chun Pu Chu, Kevin Hsu Kai Huang
Adolescent idiopathic scoliosis (AIS) remains the most common and potentially severe form of scoliosis during the rapid growth period. It is generally accepted that AIS is a multifactorial disease controlled by genetic, hormonal, neuromuscular, and environmental factors. Herein, we describe a case of 12-year-old scoliotic girl with a double curve of initially 26° thoracic and 23° lumbar, seeking chiropractic care. The therapy was provided three times/twice a week for 6 months. Adjustments of the spine through chiropractic, both soreness and mobility, were shown to improve...
April 2017: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/29287818/youth-and-experience-the-effect-of-surgeon-experience-on-outcomes-in-cerebral-palsy-scoliosis-surgery
#3
Patrick J Cahill, Amer F Samdani, Christopher M Brusalis, Todd Blumberg, Jahangir Asghar, Tracey P Bastrom, Saba Pasha, Christian A Refakis, Joshua M Pahys, John M Flynn, Paul D Sponseller
BACKGROUND: Series on the learning curve in spinal deformity surgery have been published, but none has addressed neuromuscular spinal deformity, comprised of arguably the most complex cases. We present the first multi-center analysis of the impact of surgeon experience on neuromuscular spinal deformity surgery. METHODS: A multi-center prospective study of spinal deformity surgery for cerebral palsy (CP) with at least 2 years of follow-up provided the dataset for assessment...
January 2018: Spine Deformity
https://www.readbyqxmd.com/read/29287816/comparison-of-percentile-weight-gain-of-growth-friendly-constructs-in-early-onset-scoliosis
#4
Liam R Harris, Lindsay M Andras, Paul D Sponseller, Charles E Johnston, John B Emans, David L Skaggs
STUDY DESIGN: Multicenter retrospective cohort. OBJECTIVE: To compare improvement in nutritional status seen in early-onset scoliosis (EOS) patients following treatment with various growth-friendly techniques, especially in underweight patients (<20th weight percentile). BACKGROUND: Thoracic insufficiency resulting from EOS can lead to severe cardiopulmonary disease. In this age group, pulmonary function tests are often difficult or impossible to perform...
January 2018: Spine Deformity
https://www.readbyqxmd.com/read/29249551/validation-of-a-clinical-prediction-model-for-the-development-of-neuromuscular-scoliosis-a-multinational-study
#5
Carlo M Bertoncelli, Domenico Bertoncelli, Leonard Elbaum, Michal Latalski, Paola Altamura, Charles Musoff, Virginie Rampal, Federico Solla
BACKGROUND: The objective of this study was to evaluate the performance of a clinical prediction model of neuromuscular scoliosis via external validation. METHODS: We analyzed a series of 120 patients (mean age ± standard deviation, 15.7 ± 1.8 years; range: 12 to 18 years) with cerebral palsy, severe motor disorders, and cognitive impairment with and without neuromuscular scoliosis treated in two specialized units (70 patients from Nice, France, and 50 patients from Lublin, Poland) in a cross-sectional, double-blind study...
November 20, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29244696/selective-spinal-fusion-for-neuromuscular-scoliosis-in-a-patient-with-pompe-disease-a-case-report-and-review-of-the-literature
#6
Atsushi Tanida, Shinji Tanishima, Tokumitsu Mihara, Aya Narita, Yoshihiro Maegaki, Hideki Nagashima
CASE: A 16-year-old girl with Pompe disease underwent surgery for scoliosis. She had been able to walk without any assistance, and kept her balance by swinging her waist. Therefore, we performed posterior selective spinal correction and fusion to avoid any adverse effects on walking ability that could occur with immobilization of the lumbosacral spine. After surgery, she was highly satisfied with her ability to perform the activities of daily living. CONCLUSION: For nonambulatory patients with scoliosis and Pompe disease, long fusion from the upper thoracic spine to the pelvis is generally required...
January 2017: JBJS Case Connector
https://www.readbyqxmd.com/read/29227363/psychosocial-factors-and-surgical-outcomes-in-adult-spinal-deformity-do-dementia-patients-have-more-complications
#7
Courtney S Toombs, Justin C Paul, Baron S Lonner
STUDY DESIGN: Retrospective analysis of a prospectively collected, national inpatient hospital database. OBJECTIVE: We aimed to investigate comorbid psychiatric disorders in the ASD population. We hypothesized that a high incidence of comorbid psychiatric disorders in ASD would negatively impact perioperative outcomes. SUMMARY OF BACKGROUND DATA: Adult spinal fusion (ASF) patients suffer from severe back pain and often depression. Psychiatric comorbidities in the adult spinal deformity (ASD) population are not well understood, despite the apparent psychological effects of spinal deformity-related self-image...
December 8, 2017: Spine
https://www.readbyqxmd.com/read/29196274/neuromuscular-diseases-diagnosis-and-management
#8
REVIEW
P Mary, L Servais, R Vialle
Neuromuscular diseases (NMDs) affect the peripheral nervous system, which includes the motor neurons and sensory neurons; the muscle itself; or the neuromuscular junction. Thus, the term NMDs encompasses a vast array of different syndromes. Some of these syndromes are of direct relevance to paediatric orthopaedic surgeons, either because the presenting manifestation is a functional sign (e.g., toe walking) or deformity (e.g., pes cavus or scoliosis) suggesting a need for orthopaedic attention or because orthopaedic abnormalities requiring treatment develop during the course of a known NMD...
November 28, 2017: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/29189537/bipolar-sealer-devices-used-in-posterior-spinal-fusion-for-neuromuscular-scoliosis-reduce-blood-loss-and-transfusion-requirements
#9
Christina K Hardesty, Zachary L Gordon, Connie Poe-Kochert, Jochen P Son-Hing, George H Thompson
BACKGROUND: Reducing perioperative blood loss and the need for transfusions in patients undergoing spinal surgery is especially important for those with neuromuscular disorders. These patients require extensive spino-pelvic exposure and are often medically fragile. We have used Amicar to decrease blood loss since 2001. As an effort to further reduce blood loss and transfusions, we use a bipolar sealer device (Aquamantys) as an adjunct to electrocautery. We present the results of our first 64 neuromuscular patients to show the efficacy of the device...
November 16, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29189528/incidence-and-diagnostic-evaluation-of-postoperative-fever-in-pediatric-patients-with-neuromuscular-disorders
#10
Mohamed A A Yousef, Darius Dranginis, Scott Rosenfeld
BACKGROUND: Children with neuromuscular disorders have a significant chance of developing scoliosis and/or hip dislocation during childhood and adolescence and often undergo surgical reconstruction. Because of their high rate of medical comorbidities, these patients are at higher risk of postoperative complications and are therefore often comanaged, along with orthopaedics, by pediatric medicine and critical care teams. Fever during the postoperative stay is a frequent occurrence and often prompts extensive diagnostic workup which increases the cost and burden on the health system but have unclear effects on the care of the patient...
November 16, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29169929/compound-heterozygous-ryr1-mutations-in-a-preterm-with-arthrogryposis-multiplex-congenita-and-prenatal-cns-bleeding
#11
Florian Brackmann, Matthias Türk, Nils Gratzki, Oliver Rompel, Heinz Jungbluth, Rolf Schröder, Regina Trollmann
RYR1 mutations, the most common cause of non-dystrophic neuromuscular disorders, are associated with the malignant hyperthermia susceptibility (MHS) trait as well as congenital myopathies with widely variable clinical and histopathological manifestations. Recently, bleeding anomalies have been reported in association with certain RYR1 mutations. Here we report a preterm infant born at 32 weeks gestation with arthrogryposis multiplex congenita due to compound heterozygous, previously MHS-associated RYR1 mutations, with additional signs of prenatal hemorrhage...
September 28, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29145830/differences-in-vertebral-morphology-around-the-apical-vertebrae-between-neuromuscular-scoliosis-and-idiopathic-scoliosis-in-skeletally-immature-patients-a-three-dimensional-morphometric-analysis
#12
Takahiro Makino, Yusuke Sakai, Masafumi Kashii, Shota Takenaka, Kazuomi Sugamoto, Hideki Yoshikawa, Takashi Kaito
BACKGROUND: Recent morphological analyses of vertebrae in patients with scoliosis have revealed three-dimensional (3D) deformities in the vertebral bodies. However, it remains controversial whether these deformities are secondary changes caused by asymmetrical vertebral loading or primary changes caused by aberrant asymmetrical vertebral growth. Furthermore, the difference in vertebral morphology between scoliosis with different pathogeneses remains unclear. This study was aimed to investigate the difference in the coronal asymmetry of vertebral bodies between neuromuscular scoliosis (NS) in Duchenne muscular dystrophy (DMD) and idiopathic scoliosis (IS) using in vivo 3D analysis...
November 16, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29118959/congenital-myasthenic-syndrome-due-to-dok7-mutations-in-a-family-from-chile
#13
Jorge A Bevilacqua, Marian Lara, Jorge Díaz, Mario Campero, Jessica Vázquez, Ricardo A Maselli
Congenital myasthenic syndromes (CMS) are neuromuscular transmission disorders caused by mutations in genes encoding neuromuscular junction proteins. A 61-year-old female and her older sister showed bilateral ptosis, facial and proximal limb weakness, and scoliosis since childhood. Another female sibling had milder signs, while other family members were asymptomatic. Facial nerve repetitive stimulation in the proband showed decrement of muscle responses. Single fiber EMG revealed increased jitter and blocking...
June 27, 2017: European Journal of Translational Myology
https://www.readbyqxmd.com/read/29101470/pancreatic-fracture-a-rare-complication-following-scoliosis-surgery
#14
Mélodie Juricic, Kalitha Pinnagoda, Walid Lakhal, Jérome Sales De Gauzy, Olivier Abbo
STUDY DESIGN: Grand Round case report. OBJECTIVE: We report a pancreatic fracture associated with Wirsung duct disruption, following a scoliosis surgery in a cerebral palsy adolescent. Spinal fusion surgery is the standard treatment for severe neuromuscular scoliosis. Many complications such as digestive ones account for its complexity. Postoperative acute pancreatitis is well described, although its pathophysiology remains unclear. To our knowledge, pancreatic fracture following scoliosis correction has never been described to date...
November 3, 2017: European Spine Journal
https://www.readbyqxmd.com/read/29095413/segmental-pedicle-screw-instrumentation-and-fusion-only-to-l5-in-the-surgical-treatment-of-flaccid-neuromuscular-scoliosis
#15
Masashi Takaso, Toshiyuki Nakazawa, Takayuki Imura, Michinari Fukuda, Kazuhisa Takahashi, Seiji Ohtori
STUDY DESIGN: A retrospective cohort study was performed. OBJECTIVE: The purpose of this study was to determine the efficacy and safety of stopping segmental pedicle screw instrumentation constructs at L5 in the treatment of neuromuscular scoliosis. SUMMARY OF BACKGROUND DATA: Duchenne muscular dystrophy and spinal muscular atrophy are flaccid neuromuscular disorders in which gradual deterioration is the hallmark and have a lot of characteristics in common despite differences in etiology...
October 31, 2017: Spine
https://www.readbyqxmd.com/read/29067653/clinical-manifestations-and-overall-management-strategies-for-duchenne-muscular-dystrophy
#16
Takeshi Tsuda
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder that causes progressive weakness and wasting of skeletal muscular and myocardium in boys due to mutation of dystrophin. The structural integrity of each individual skeletal and cardiac myocyte is significantly compromised upon physical stress due to the absence of dystrophin. The progressive destruction of systemic musculature and myocardium causes affected patients to develop multiple organ disabilities, including loss of ambulation, physical immobility, neuromuscular scoliosis, joint contracture, restrictive lung disease, obstructive sleep apnea, and cardiomyopathy...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29064871/best-practice-guidelines-for-surgical-site-infection-prevention-with-surgical-treatment-of-early-onset-scoliosis
#17
Michael P Glotzbecker, Tricia A St Hilaire, Jeff B Pawelek, George H Thompson, Michael G Vitale
BACKGROUND: Postoperative surgical site infection (SSI) is unfortunately a commonly encountered complication in the surgical treatment of children with Early Onset Scoliosis (EOS). There is documented variation in the treatment of this patient population. Previous work building consensus for the approach to high risk patients (eg, neuromuscular etiology) has been promising. The goal of the current study is to apply similar principles to develop consensus-based guidelines for the treatment of patients with EOS...
October 23, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29052036/rapidly-increasing-incidence-in-scoliosis-surgery-over-14%C3%A2-years-in-a-nationwide-sample
#18
Johan von Heideken, Maura D Iversen, Paul Gerdhem
PURPOSE: Severe scoliosis is primarily managed with surgery. This cohort study describes the incidence of surgically treated scoliosis among Swedish youth and young adults, stratified by age, sex, scoliosis type, and surgical approach and identifies changes in incidence rate and hospital length of stay (LOS), infections requiring re-surgery and mortality within 90 days. METHODS: Swedish youth, 0-21 years, (n = 3062) with a diagnostic code for scoliosis and spine surgery between 2000 and 2013 were selected from the National Patient Register...
October 19, 2017: European Spine Journal
https://www.readbyqxmd.com/read/29051055/skeletal-impairment-in-pierson-syndrome-is-there-a-role-for-laminin%C3%AE-2-in-bone-physiology
#19
Camille Beaufils, Delphine Farlay, Irma Machuca-Gayet, Alice Fassier, Martin Zenker, Caroline Freychet, Edith Bonnelye, Aurélia Bertholet-Thomas, Bruno Ranchin, Justine Bacchetta
INTRODUCTION: Pierson syndrome is caused by a mutation of LAMB2, encoding for laminin β2. Clinical phenotype is variable but usually associates congenital nephrotic syndrome (CNS) and ocular abnormalities. Neuromuscular impairment has also been described. METHODS: We report on a 15-year old girl, suffering from Pierson Syndrome, who developed severe bone deformations during puberty. This patient initially displayed CNS and microcoria, leading to the clinical diagnosis of Pierson syndrome...
October 16, 2017: Bone
https://www.readbyqxmd.com/read/29050720/does-intrathecal-baclofen-therapy-increase-prevalence-and-or-progression-of-neuromuscular-scoliosis
#20
Kevin R Walker, Susan A Novotny, Linda E Krach
STUDY DESIGN: Retrospective, case-matched review. OBJECTIVES: Compare a group of individuals with cerebral palsy (CP) who had intrathecal baclofen (ITB) pumps to a group of individuals with CP who did not have ITB pumps in order to determine if there was a difference in the prevalence of new-onset neuromuscular scoliosis, an increased rate of progression of preexisting neuromuscular scoliosis, or an increased rate of posterior spine fusion surgery in skeletally immature individuals with CP who had ITB pumps...
November 2017: Spine Deformity
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