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Early-Onset Scoliosis

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https://www.readbyqxmd.com/read/27909129/metallosis-following-implantation-of-magnetically-controlled-growing-rods-in-the-treatment-of-scoliosis-a-case-series
#1
K H Teoh, C von Ruhland, S L Evans, S H James, A Jones, J Howes, P R Davies, S Ahuja
AIMS: We present a case series of five patients who had revision surgery following magnetic controlled growing rods (MGCR) for early onset scoliosis. Metallosis was found during revision in four out of five patients and we postulated a mechanism for rod failure based on retrieval analysis. PATIENTS AND METHODS: Retrieval analysis was performed on the seven explanted rods. The mean duration of MCGR from implantation to revision was 35 months (17 to 46). The mean age at revision was 12 years (7 to 15; four boys, one girl)...
December 2016: Bone & Joint Journal
https://www.readbyqxmd.com/read/27863379/targeted-next-generation-sequencing-identifies-two-novel-mutations-in-sepn1-in-rigid-spine-muscular-dystrophy-1
#2
Yi Dai, Shengran Liang, Yan Huang, Lin Chen, Santasree Banerjee
Rigid spine muscular dystrophy 1 (RSMD1) is a neuromuscular disorder, manifested with poor axial muscle strength, scoliosis and neck weakness, and a variable degree of spinal rigidity with an early ventilatory insufficiency which can lead to death by respiratory failure. Mutations of SEPN1 gene are associated with autosomal recessive RSMD1. Here, we present a clinical molecular study of a Chinese proband with RSMD1. The proband is a 17 years old male, showing difficulty in feeding, delayed motor response, problem in running with frequent fall down, early onset respiratory insufficiency, general muscle weakness and rigid cervical spine...
November 14, 2016: Oncotarget
https://www.readbyqxmd.com/read/27852908/final-fusion-after-growing-rod-treatment-for-early-onset-scoliosis-is-it-really-final
#3
Connie Poe-Kochert, Claire Shannon, Jeff B Pawelek, George H Thompson, Christina K Hardesty, David S Marks, Behrooz A Akbarnia, Richard E McCarthy, John B Emans
BACKGROUND: Final fusion is thought to be the end point for patients with early onset scoliosis following treatment with the use of growing rods. But is it? The purpose of this study was to determine the incidence and cause of any reoperation after final fusion. METHODS: A multicenter database of patients with early onset scoliosis was retrospectively analyzed to identify patients treated with growing rods with a minimum of 2 years of follow-up after final fusion...
November 16, 2016: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/27852502/variability-of-surgical-site-infection-with-veptr-at-eight-centers-a%C3%A2-retrospective-cohort-analysis
#4
Sumeet Garg, Micaela Cyr, Tricia St Hilaire, Tara Flynn, Patrick Carry, Michael Glotzbecker, John T Smith, Jeffrey Sawyer, Joshua Pahys, Scott Luhmann, John M Flynn, Ron El-Hawary, Michael Vitale
STUDY DESIGN: Retrospective review. OBJECTIVES: To describe clinical characteristics and infection rates in modern vertical expandable prosthetic titanium rib (VEPTR) surgery. SUMMARY OF BACKGROUND DATA: Prior studies have demonstrated infection rates from 10% to 30% with VEPTR surgery. METHODS: A retrospective query was done on an institutional review board-approved, multicenter prospectively collected database for patients implanted with VEPTR from 2007 to 2013 at eight sites...
January 2016: Spine Deformity
https://www.readbyqxmd.com/read/27852501/longitudinal-pilot-analysis-of-radiation-exposure-during-the-course-of-growing-rod-treatment-for-early-onset-scoliosis
#5
Gregory M Mundis, Jeff B Pawelek, Edward K Nomoto, Michael W Hennessy, Burt Yaszay, Behrooz A Akbarnia
STUDY DESIGN: Retrospective consecutive case series. OBJECTIVES: To estimate the amount of ionizing radiation (IR) exposure in growing rod (GR) surgery for early-onset scoliosis. SUMMARY OF BACKGROUND DATA: There is substantial evidence of the health hazards attributed to IR exposure. However, no studies have estimated the amount of IR exposure in GR surgery. MATERIALS AND METHODS: A consecutive single-center series of GR patients were retrospectively reviewed...
January 2016: Spine Deformity
https://www.readbyqxmd.com/read/27831988/magnetic-controlled-growing-rods-as-a-treatment-of-early-onset-scoliosis-early-results-with-two-patients
#6
Ahmed Majid Heydar, Serdar Şirazi, Murat Bezer
STUDY DESIGN: Prospective unicentral nonrandomized study. OBJECTIVE: To evaluate the safety and effectivity profile of magnetic controlled growing rods (MCGR) in patients with early onset scoliosis (EOS). SUMMARY OF BACKGROUND DATA: Conventional growing rods are the most commonly used growth sparring devices in the treatment of EOS, as this technique requires repeated surgical operations for lengthening; it is associated with high rate of complications and increased costs...
November 15, 2016: Spine
https://www.readbyqxmd.com/read/27824791/how-does-hyperkyphotic-early-onset-scoliosis-respond-to-growing-rod-treatment
#7
Zhonghui Chen, Yong Qiu, Zezhang Zhu, Song Li, Xi Chen, Xu Sun
BACKGROUND: Growing rods (GRs) pose a higher risk of kyphosis gain while improving coronal deformity with intermittent distractions in early-onset scoliosis (EOS), but in turn, increased kyphosis may also affect GR correction outcomes. However, there is a paucity of data regarding the effect of GRs on the sagittal spinal profiles in hyperkyphotic EOS. Thus, we aim to demonstrate how hyperkyphotic EOS responds to GRs treatment. METHODS: Forty patients treated with GRs for EOS were included and categorized into a normal kyphosis (N) group [20 degrees≤thoracic kyphosis (TK)≤50 degrees] or hyperkyphosis group (K) group (TK≥50 degrees)...
November 7, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/27817139/three-dimensional-reconstruction-using-stereoradiography-for-evaluating-adult-spinal-deformity-a-reproducibility-study
#8
Emmanuelle Ferrero, Renaud Lafage, Shaleen Vira, Pierre-Yves Rohan, Jonathan Oren, Edward Delsole, Pierre Guigui, Frank Schwab, Virginie Lafage, Wafa Skalli
PURPOSE: In addition to the sagittal alignment, impact of transverse plane parameters (TPP) and rotatory subluxation on patients reported outcomes were highlighted. One of the hypotheses for genesis of degenerative scoliosis is disc degeneration with increased axial vertebral (AVR) and intervertebral rotation (AIR). Therefore, TPP analysis at early stage of the scoliosis seems of particular interest. This study aims at assessing reliability of tridimensional (3D) reconstructions of adult spinal deformity (ASD) patients...
November 5, 2016: European Spine Journal
https://www.readbyqxmd.com/read/27798426/advances-in-growing-rods-treatment-for-early-onset-scoliosis
#9
Senol Bekmez, Ozgur Dede, Muharrem Yazici
PURPOSE OF REVIEW: The goal of treatment in early onset scoliosis is to correct the deformity while allowing the thoracic growth for optimal cardiopulmonary functions. Growing rods treatment is a distraction-based, growth-friendly method that is commonly used in treatment of early onset scoliosis with its specific indications. Magnetically controlled growing rods (MCGR) method has been introduced to avoid morbidity of recurrent lengthening procedures. In this review, recent developments in traditional growing rods and MCGR are summarized...
October 27, 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27695352/cost-analysis-of-magnetically-controlled-growing-rods-compared-with-traditional-growing-rods-for-early-onset-scoliosis-in-the-us-an-integrated-health-care-delivery-system-perspective
#10
David W Polly, Stacey J Ackerman, Karen Schneider, Jeff B Pawelek, Behrooz A Akbarnia
PURPOSE: Traditional growing rod (TGR) for early-onset scoliosis (EOS) is effective but requires repeated invasive surgical lengthenings under general anesthesia. Magnetically controlled growing rod (MCGR) is lengthened noninvasively using a hand-held magnetic external remote controller in a physician office; however, the MCGR implant is expensive, and the cumulative cost savings have not been well studied. We compared direct medical costs of MCGR and TGR for EOS from the US integrated health care delivery system perspective...
2016: ClinicoEconomics and Outcomes Research: CEOR
https://www.readbyqxmd.com/read/27689140/flexible-growing-rods-a-biomechanical-pilot-study-of-polymer-rod-constructs-in-the-stability-of-skeletally-immature-spines
#11
Donita I Bylski-Austrow, David L Glos, Anne C Bonifas, Max F Carvalho, Matthew C Coombs, Peter F Sturm
BACKGROUND: Surgical treatments for early onset scoliosis (EOS) correct curvatures and improve respiratory function but involve many complications. A distractible, or 'growing rod,' implant construct that is more flexible than current metal rod systems may sufficiently correct curves in small children and reduce complications due to biomechanical factors. The purpose of this pilot study was to determine ranges of motion (ROM) after implantation of simulated growing rod constructs with a range of clinically relevant structural properties...
2016: Scoliosis and Spinal Disorders
https://www.readbyqxmd.com/read/27662388/spinal-instrumentation-in-growing-children-retards-the-natural-development-of-pelvic-incidence
#12
Senol Bekmez, Halil Gokhan Demirkiran, Ozgur Dede, Yunus Atici, Mehmet Bulent Balioglu, Moyo Kruyt, Timothy Ward, Muharrem Yazici
BACKGROUND: Pelvic incidence increases gradually throughout growth until skeletal maturity. Growing rod instrumentation has been suggested to have a stabilizing effect on the development of the normal sagittal spinal alignment. The purpose of this study is to determine the effect of fixed sagittal plane caused by dual growing rod instrumentation on the natural progression of sagittal spinopelvic parameters in children with idiopathic or idiopathic-like early onset scoliosis. METHODS: Hospital records of children with growing rod instrumentation from 4 separate institutions were reviewed retrospectively...
September 22, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/27618868/gosr2-a-progressive-myoclonus-epilepsy-gene
#13
Leanne M Dibbens, Guido Rubboli
GOSR2-associated PME is associated with a homozygous mutation in GOSR2 (c.430G>T, p.Gly144Trp), a Golgi vesicle transport gene. The functional effect of this mutation is a loss of function that results in failure of the GOSR2 protein to localize to the cis-Golgi. The main clinical features of the GOSR2-associated PME are early-onset ataxia, areflexia, action myoclonus and seizures, scoliosis, elevated creatine kinase levels, relative preservation of cognitive function until the late stages of the disease, and relentless disease course...
September 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/27587527/the-use-of-magnetically-controlled-growing-rods-to-treat-children-with-early-onset-scoliosis-early-radiological-results-in-19-children
#14
W Thompson, C Thakar, D J Rolton, J Wilson-MacDonald, C Nnadi
AIMS: We undertook a prospective non-randomised radiological study to evaluate the preliminary results of using magnetically-controlled growing rods (MAGEC System, Ellipse technology) to treat children with early-onset scoliosis. PATIENTS AND METHODS: Between January 2011 and January 2015, 19 children were treated with magnetically-controlled growing rods (MCGRs) and underwent distraction at three-monthly intervals. The mean age of our cohort was 9.1 years (4 to 14) and the mean follow-up 22...
September 2016: Bone & Joint Journal
https://www.readbyqxmd.com/read/27499651/new-developments-in-the-treatment-of-early-onset-spinal-deformity-role-of-the-shilla-growth-guidance-system
#15
REVIEW
Sean M Morell, Richard E McCarthy
Early-onset scoliosis is a complex condition with multiple facets. The goal of treating any spinal deformity is to improve the condition of the patient with the least intervention necessary. A system that allows for continuation of natural spinal growth while correcting the deformity should be the goal of treating this complex condition. The SHILLA growth guidance system allows for continued growth of the pediatric spine while correcting and guiding the apex and guiding the future growth of the curvature. The system involves selective fusion across the apex of the curvature, and minimally invasive instrumentation is then used above and below the apex to allow for continued growth of the spine...
2016: Medical Devices: Evidence and Research
https://www.readbyqxmd.com/read/27497928/magnetically-controlled-growing-rod-in-early-onset-scoliosis-a-minimum-of-2-year-follow-up
#16
Baran Yılmaz, Murat Şakir Ekşi, Semra Işik, Emel Ece Özcan-Ekşi, Zafer Orkun Toktaş, Deniz Konya
PURPOSE: The magnetically controlled growing rod technique decreases complications, costs, and loss of functionality by avoiding successive surgical corrections every 6 months in patients with early-onset scoliosis. However, only a few studies have presented clinical experience with the magnetically controlled growing rod. In this study we aimed to present our single-center experience in patients with early-onset scoliosis who underwent the magnetically controlled growing rod technique and follow-up for at least 2 years...
2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27485408/a-new-early-onset-neuromuscular-disorder-associated-with-kyphoscoliosis-peptidase-ky-deficiency
#17
Carola Hedberg-Oldfors, Niklas Darin, Mia Olsson Engman, Zacharias Orfanos, Christer Thomsen, Peter F M van der Ven, Anders Oldfors
We describe a new early-onset neuromuscular disorder due to a homozygous loss-of-function variant in the kyphoscoliosis peptidase gene (KY). A 7.5-year-old girl with walking difficulties from 2 years of age presented with generalized muscle weakness; mild contractures in the shoulders, hips and feet; cavus feet; and lordosis but no scoliosis. She had previously been operated with Achilles tendon elongation. Whole-body MRI showed atrophy and fatty infiltration in the calf muscles. Biopsy of the vastus lateralis muscle showed variability in fiber size, with some internalized nuclei and numerous very small fibers with variable expression of developmental myosin heavy chain isoforms...
August 3, 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27470156/early-onset-scoliosis-a-comprehensive-guide-from-the-oxford-meetings
#18
Armand Laumen, Toon De Backer
No abstract text is available yet for this article.
July 29, 2016: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/27469082/a-brief-history-and-review-of-modern-casting-techniques-in-early-onset-scoliosis
#19
Ozgur Dede, Peter F Sturm
PURPOSE: Body casts have a long history in the treatment of spinal deformity. Currently the use of body casts is limited to early onset scoliosis. Here, we aim to provide a brief narrative of the evolution of cast application for the management of spinal deformity. METHODS: A history of cast application is provided with a brief review of the orthopedic literature. The current indications for cast application and the authors' preferred technique are described. RESULTS: Serial casting is an effective treatment method for early onset scoliosis...
October 2016: Journal of Children's Orthopaedics
https://www.readbyqxmd.com/read/27435487/incidence-of-proximal-junctional-kyphosis-with-magnetic-expansion-control-rods-in-early-onset-scoliosis
#20
P Inaparthy, J C Queruz, D Bhagawati, C Thakar, T Subramanian, C Nnadi
BACKGROUND: The introduction of magnetic expansion control growth rods for the surgical management of EOS has gained popularity. However, there are no published studies on the incidence of proximal junctional kyphosis (PJK) using this technique. PURPOSE: The aim of this study is to report the incidence of PJK following treatment with magnetic growth rods in EOS. METHODS: Retrospective review of data from 21 cases (12 males, 9 females) over 3 years...
October 2016: European Spine Journal
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