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Early-Early-Onset Scoliosis

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
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
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
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
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
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
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
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
Armand Laumen, Toon De Backer
No abstract text is available yet for this article.
July 29, 2016: Acta Chirurgica Belgica
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
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
Gui-Jun Xu, Xin Fu, Peng Tian, Jian-Xiong Ma, Xin-Long Ma
BACKGROUND: The growing rod technique was applied in the treatment of early onset scoliosis (EOS) with promising outcomes and many complications at the same time. We reviewed data from literatures to compare the results of single growing rods with dual growing rods to achieve a clear understanding of this technique. METHODS: PubMed, Embase, MEDLINE, ScienceDirect, CNKI, Wanfang Data, and CQVIP were searched electronically until March 2016 using "growing rod" and "early onset scoliosis" as major search terms...
2016: Journal of Orthopaedic Surgery and Research
Nolan M Wessell, Jeffrey E Martus, Matthew A Halanski, Brian Snyder, Walter Truong
BACKGROUND: Early-onset scoliosis (EOS) affects roughly 1 to 2 out of 10,000 live births per year. Because this subset of patients has a yet to achieve a majority of their skeletal growth, a number of treatment challenges need to be addressed before surgical intervention. If left untreated, EOS can cause a number of problems throughout the patient's lifespan, particularly in regards to the growth of the thorax and pulmonary development. A wide variety of surgical systems and techniques are available to the treating surgeon...
July 11, 2016: Journal of Pediatric Orthopedics
John H T Waldhausen, Greg Redding, Klane White, Kit Song
PURPOSE: This report describes complications using the vertical expandable prosthetic titanium rib (VEPTR) for thoracic insufficiency syndrome (TIS) at a single center. METHODS: This is a prospective cohort evaluating 65 patients with rib-rib and rib-spine VEPTR devices for TIS placed between 10/2001 and 11/2014, for children with spinal or chest wall deformity. Patients were classified using the early onset scoliosis classification system (C-EOS). RESULTS: 65 patients are available for follow up...
June 26, 2016: Journal of Pediatric Surgery
Andrea Angius, Paolo Uva, Insa Buers, Manuela Oppo, Alessandro Puddu, Stefano Onano, Ivana Persico, Angela Loi, Loredana Marcia, Wolfgang Höhne, Gianmauro Cuccuru, Giorgio Fotia, Manila Deiana, Mara Marongiu, Hatice Tuba Atalay, Sibel Inan, Osama El Assy, Leo M E Smit, Ilyas Okur, Koray Boduroglu, Gülen Eda Utine, Esra Kılıç, Giuseppe Zampino, Giangiorgio Crisponi, Laura Crisponi, Frank Rutsch
Crisponi syndrome (CS)/cold-induced sweating syndrome type 1 (CISS1) is a very rare autosomal-recessive disorder characterized by a complex phenotype with high neonatal lethality, associated with the following main clinical features: hyperthermia and feeding difficulties in the neonatal period, scoliosis, and paradoxical sweating induced by cold since early childhood. CS/CISS1 can be caused by mutations in cytokine receptor-like factor 1 (CRLF1). However, the physiopathological role of CRLF1 is still poorly understood...
July 7, 2016: American Journal of Human Genetics
Amit Jain, Paul D Sponseller, John M Flynn, Suken A Shah, George H Thompson, John B Emans, Jeff B Pawelek, Behrooz A Akbarnia
BACKGROUND: Definitive "final" surgical fusion is the common end point for growing-rod treatment of early-onset scoliosis. However, final surgical fusion may be unnecessary for patients who have reached skeletal maturity with good alignment. This study characterizes patients who completed growing-rod treatment but did not undergo final surgical fusion. METHODS: Using a multicenter early-onset-scoliosis database, we identified 167 patients who received growing-rod treatment from 1995 through 2010, reached skeletal maturity, and had a minimum 2-year follow-up after their last surgery...
July 6, 2016: Journal of Bone and Joint Surgery. American Volume
X Zheng, Y Qiu, X Sun, B P Qian, B Wang, Y Yu, Z Z Zhu
OBJECTIVE: To evaluate the efficacy of a growth-guidance growing rod in an established porcine scoliosis model via the Cobb angle correction and the continued spinal growth. METHODS: Immature pigs (age: 6 weeks old, weight: 6-8 kg) were instrumented and tethered using a three separate incisions fashion.After considerable scoliosis was induced, the pigs were randomly assigned to an experiment group (EG) and a sham group (SG). In EG, the growing rod was implanted and the pigs were euthanized 8 weeks postoperatively; while in SG, the whole instrumentations were only removed and the pigs were followed up over a 8-week period...
June 21, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Edmund Choi, Burt Yazsay, Gregory Mundis, Pooria Hosseini, Jeff Pawelek, Ahmet Alanay, Haluk Berk, Kenneth Cheung, Gokhan Demirkiran, John Ferguson, Tiziana Greggi, Ilkka Helenius, Guido La Rosa, Alpaslan Senkoylu, Behrooz A Akbarnia
BACKGROUND: Traditional growing rods have a reported wound and implant complication rate as high as 58%. It is unclear whether the use of magnetically controlled growing rods (MCGR) will affect this rate. This study was performed to characterize surgical complications following MCGR in early onset scoliosis. METHODS: A multicenter retrospective review of MCGR cases was performed. Inclusion criteria were: (1) diagnosis of early onset scoliosis of any etiology; (2) 10 years and younger at time of index surgery; (3) preoperative major curve size >30 degrees; (4) preoperative thoracic spine height <22 cm...
June 18, 2016: Journal of Pediatric Orthopedics
Yingsong Wang, Noriaki Kawakami, Taichi Tsuji, Tetsuya Ohara, Yoshitaka Suzuki, Toshiki Saito, Ayato Nohara, Ryoji Tauchi, Kazuki Kawakami
STUDY DESIGN: A retrospective study. OBJECTIVE: To investigate whether PJK or obvious proximal junctional angle (PJA) changes in the sagittal plane develops following short fusion in children younger than 10 years of age with congenital scoliosis, and to investigate the possible risk factors. SUMMARY OF BACKGROUND DATA: Proximal junctional kyphosis (PJK) following long spinal fusion in adolescents and adults is a serious postoperative complication...
June 20, 2016: Clinical Spine Surgery
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