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Early-Early-Onset Scoliosis

X Sun, L Xu, Z H Chen, X Chen, C Z Du, S Li, Z Liu, B P Qian, B Wang, Z Z Zhu, Y Qiu
Objective: To evaluate the correction result of traditional dual growing rods on apical vertebral rotation. Methods: This study recruited 19 early-onset scoliosis patients (6 boys and 13 girls) who had received traditional dual growing rods treatment at Department of Spine Surgery, Nanjing Drum Tower Hospital from January 2009 to July 2015. The age at initial surgery was (5.7±1.7)years(range, 3 to 9 years). Measurements of primary curve magnitude, height of T(1)-S(1), apical vertebral translation(AVR), apical vertebral body-rib ratio, apical vertebral rotation, thoracic rotation and rib hump were compared between pre-operatively, post-operatively, and at latest follow-up, through a paired- t test...
March 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
Matthew E Oetgen
Evidence-based medicine (EBM) is a process of decision-making aimed at making the best clinical decisions as they relate to patients' health. The current use of EBM in pediatric spine surgery is varied, based mainly on the availability of high-quality data. The use of EBM is limited in idiopathic scoliosis, whereas EBM has been used to investigate the treatment of pediatric spondylolysis. Studies on early onset scoliosis are of low quality, making EBM difficult in this condition. Future focus and commitment to study quality in pediatric spinal surgery will likely increase the role of EBM in these conditions...
April 2018: Orthopedic Clinics of North America
John M McCarthy, Bonnie M McCann-Crosby, Megan E Rech, Jiani Yin, Chun-An Chen, May A Ali, HaiThuy N Nguyen, Jennifer L Miller, Christian P Schaaf
BACKGROUND: Nonsense and frameshift mutations in the maternally imprinted, paternally expressed gene MAGEL2, located in the Prader-Willi critical region 15q11-15q13, have been reported to cause Schaaf-Yang syndrome (SYS), a genetic disorder that manifests as developmental delay/intellectual disability, hypotonia, feeding difficulties and autism spectrum disorder. Prader-Willi syndrome (PWS) is a genetic disorder characterised by severe infantile hypotonia, hypogonadotrophic hypogonadism, early childhood onset obesity/hyperphagia, developmental delay/intellectual disability and short stature...
March 1, 2018: Journal of Medical Genetics
Johan L Heemskerk, Moyo C Kruyt, Dino Colo, René M Castelein, Diederik H R Kempen
BACKGROUND: There is ongoing controversy about the routine use of magnetic resonance imaging (MRI) preoperatively in patients with presumed idiopathic scoliosis (IS). Routine MRI can help identify possible causes for the deformity and detect anomalies which could complicate deformity surgery. However, routine MRI increases health care costs significantly and may reveal mild variations from normal without clinical relevance, that can still lead to anxiety and influence decision making...
February 14, 2018: Spine Journal: Official Journal of the North American Spine Society
Jonathan Oore, Braydon Connell, Burt Yaszay, Amer Samdani, Tricia St Hilaire, Tara Flynn, Ron El-Hawary
BACKGROUND: Prader-Willi syndrome (PWS) patients can present with scoliosis which can be treated with serial cast correction (SCC) or with growth friendly surgery (GFS). This study's purpose was to describe the results of SCC as well as GFS for PWS patients with early-onset scoliosis (EOS). METHODS: PWS patients were identified from 2 international multicenter EOS databases. Scoliosis, kyphosis, spine height (T1-S1), right/left hemithoracic heights/widths (RHTH, LHTH, RHTW, LHTW) were measured pretreatment, postoperation, and at 2-year follow-up...
February 2, 2018: Journal of Pediatric Orthopedics
Bonnie Lauder, Peter Sinclair, Jane Maguire
BACKGROUND: Chronic childhood disease has a major impact on health related quality of life. Caring for a child with a chronic illness is well documented but the specific experiences of parents who care for children with Early Onset Scoliosis (EOS), a rare but devastating illness, has not been explored. Numerous studies have described the interrelated psychological, financial, social, physical and logistical factors that impact on the experience of the care giver role with various diseases, but in the case of EOS, limited studies have been conducted about the parental experience...
February 5, 2018: Journal of Clinical Nursing
Nicole Michael, Claire Palmer, John T Smith, Zhaoxing Pan, Tricia St Hilaire, Jessica Morgan, Victoria Heagy, Sumeet Garg
BACKGROUND: Growth-friendly surgery has high complication rates. The Complication Severity Score for growth-friendly surgery was developed to maintain consistency while reporting complications as part of research in this rapidly evolving field. This study evaluates the interrater and intrarater reliability of this complication classification system. METHODS: After Institutional Review Board approval, complications during treatment for early onset scoliosis were identified from a prospectively collected database...
January 31, 2018: Journal of Pediatric Orthopedics
Anna K Hell, Katharina Groenefeld, Konstantinos Tsaknakis, Lena Braunschweig, Heiko M Lorenz
Spine-based fixation of magnetically controlled lengthening devices has been successfully performed for children with early-onset scoliosis. However, spinal manipulation may lead to ossifications, stiffness, and autofusion as previously described. To avoid these problems, a surgical technique combining bilateral externally controlled magnetic device implantation with a rib cradle and pelvic hook fixation was introduced by us in 2011. By using a bilateral single-rib or double-rib cradle fixation and a pelvic hook, the magnetic device is percutaneously inserted...
January 23, 2018: Clinical Spine Surgery
Adrian Gardner, Alistair Beaven, David Marks, Jonathan Spilsbury, Jwalant Mehta, Matthew Newton Ede
Background: This study is a single centre retrospective review of prospectively collected data. The 'law of diminishing returns' describes the number of lengthening episodes that a traditional growth rod (TGR) can undergo before stiffness across the construct prevents further increases in overall length. It is unclear whether this will affect MCGR when used in the management of early onset scoliosis (EOS). Methods: A retrospective review of prospectively collected data on the experience of MCGR lengthening in a heterogenous cohort of children with EOS from a single centre...
December 2017: Journal of Spine Surgery (Hong Kong)
Y Hai, A X Pan, Y G Li, L Zang, S Yuan
Objective: To analyse the biomechanical effects on adjacent segments of different growing-rod (GR) fixation in early onset scoliosis through a finite element analysis method. Methods: A severe early-onset scoliosis patient was selected and the pre-operation and post-GR-operation (Upper instrumented levels: T(4), T(5). Lower instrumented levels: L(3), L(4)) whole spine 3-dimentional CT scan data were collected to build the finite models. Based on the different models, biomechanical differences on adjacent segments were analysed...
December 26, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Vaneet Singh, Jeremy Rawlinson, Nadim Hallab
Options to treat early-onset scoliosis include guided-growth systems with sliding action between rods and pedicle screws. The wear was previously measured in an in vitro test, and in this in vivo rabbit model, we evaluated the local and systemic biological response to the stainless steel debris. Compared to the previous study, a relatively higher volume of representative wear particles with a median particle size of 0.84 µm were generated. Bolus dosages were injected into the epidural space at L4-L5 for a minimum of 36 rabbits across three treatment groups (negative control, 1...
January 11, 2018: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
Sarah E Gilday, Mark S Schwartz, Donita I Bylski-Austrow, David L Glos, Lindsay Schultz, Sara O'Hara, Viral V Jain, Peter F Sturm
BACKGROUND: Magnetically controlled growing rods (MCGRs) are increasingly used in the treatment of early onset scoliosis (EOS). Few studies have reported whether desired lengthening can reliably be achieved, or if prior spine instrumentation and large tissue depths affect lengthening. In this clinical study of EOS patients, it was hypothesized that increases in rod length would equal programmed increases, patients with prior spine instrumentation would lengthen less than patients without prior surgery, and larger tissue depths would decrease lengthening success...
January 9, 2018: Journal of Pediatric Orthopedics
John Heflin, Michelle Welborn, Norman Ramirez-Lluch, Ivan Iriarte, Ron El-Hawary, Graham T Fedorak, John T Smith
STUDY DESIGN: Retrospective case control OBJECTIVE.: To report on differences in implant failure rates and complications requiring reoperation in children with early-onset scoliosis (EOS) treated with rib-based distraction utilizing four proximal fixation points in either a parallel or in-line configuration. SUMMARY OF BACKGROUND DATA: Proximal anchor failure continues to be a significant problem in growth-friendly surgery using rib-based distraction to treat children with EOS...
January 8, 2018: Spine
Silky Chotai, Jade Basem, Stephen Gannon, Michael Dewan, Chevis N Shannon, John C Wellons, Christopher M Bonfield
BACKGROUND/AIMS: Scoliosis is common in patients with Chiari malformation-I (CM-I). This study examined the change in scoliosis severity after posterior fossa decompression (PFD) for CM-I. METHODS: We conducted a retrospective review at a single tertiary center for children undergoing PFD with untreated scoliosis, and identified 17 patients with complete follow-up data and imaging. RESULTS: Overall, scoliosis improved in 7 (41.2%) patients, worsened in 9 (52...
January 4, 2018: Pediatric Neurosurgery
Caglar Yilgor, Ayaz Efendiyev, Filiz Akbiyik, Gokhan Demirkiran, Alpaslan Senkoylu, Ahmet Alanay, Muharrem Yazici
BACKGROUND: Metal ions released from spinal instruments can cause localized debris and distribute systemically to settle on distant organs. Children with early-onset deformities live with metallic implants for a substantial amount of time. No research focused on metal distribution in growth-friendly instrumentations. The aim of this study was to compare age-matched growing rod (GR) and magnetically controlled growing rod (MCGR) groups to noninstrumented controls. METHODS: The study was designed as a multicenter, prospective, cross-sectional case series...
January 2018: Spine Deformity
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
P Gawliński, M Pelc, E Ciara, S Jhangiani, E Jurkiewicz, T Gambin, A Różdżyńska-Świątkowska, M Dawidziuk, Z H C Akdemir, D Lys Guilbride, D Muzny, J R Lupski, M Krajewska-Walasek
We expand the KOGS phenotype by over 70% to include 24 unreported KOGS symptoms, in a first male patient, the third overall associated with the PDGFRB c.1751C>G, p.(Pro584Arg) mutation. 18 of these symptoms are unique to our patient, the remaining 6 are shared with other patients. Of the 24 unreported features overall, 6 show marked phenotype evolution and varying time of onset. The triangular face detected at 14 months and long palpebral fissures with lateral ectropion at 4 years are present in other members of the cohort...
December 11, 2017: Clinical Genetics
A Ahmad, T Subramanian, P Panteliadis, J Wilson-Macdonald, D A Rothenfluh, C Nnadi
AIMS: Magnetically controlled growing rods (MCGRs) allow non-invasive correction of the spinal deformity in the treatment of early-onset scoliosis. Conventional growing rod systems (CGRS) need repeated surgical distractions: these are associated with the effect of the 'law of diminishing returns'. The primary aim of this study was to quantify this effect in MCGRs over sequential distractions. PATIENTS AND METHODS: A total of 35 patients with a maximum follow-up of 57 months were included in the study...
December 2017: Bone & Joint Journal
Leonie Wagner, Lena Braunschweig, Helmut Eiffert, Konstantinos Tsaknakis, Dirk Kamin, Elisa D'Este, Philipp K Messer, Anna Kathrin Hell, Heiko M Lorenz
BACKGROUND: Bacterial colonization of spinal implants may cause severe complications in patients with early-onset scoliosis. Correct diagnosis and detection of microbiologic formation is crucial to prevent delayed infections caused by bacterial colonization. The purposes of this study were to estimate the rate and risk factors of colonization of vertical expandable prosthetic titanium rib (VEPTR) implants in children and to compare the different methods for detecting microbiologic formation on the spinal implants...
January 2018: Surgical Infections
M B Balioğlu, Y Atici, A Albayrak, D Kargin, Y E Akman, M A Kaygusuz
The present study assessed the advantages and disadvantages of growth-friendly spinal instrumentation surgery for early-onset scoliosis in 17 patients who underwent this surgery with a minimum 2-year follow-up. The mean number of lengthening procedures was three, initial age at which surgery was performed was 108.1 ± 30.2 months, and follow-up duration was 40.6 ± 16.6 months. Spinal height (T1-S1 and T1-T12), lung space available, major Cobb angle for scoliosis, maximum thoracic kyphosis, lumbar lordosis, shoulder and pelvic balance, and coronal and sagittal balance were assessed preoperatively and at the last follow-up...
December 2016: Acta Orthopaedica Belgica
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