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Early-Early-Onset Scoliosis

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https://www.readbyqxmd.com/read/29325333/-biomechanical-effects-on-adjacent-segments-of-different-growing-rod-fixation-in-early-onset-scoliosis
#1
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]
https://www.readbyqxmd.com/read/29323741/stainless-steel-wear-debris-of-a-scoliotic-growth-guidance-system-has-little-local-and-systemic-effect-in-an-animal-model
#2
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
https://www.readbyqxmd.com/read/29319661/observed-length-increases-of-magnetically-controlled-growing-rods-are-lower-than-programmed
#3
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
https://www.readbyqxmd.com/read/29315128/parallel-proximal-fixation-in-rib-based-growing-rod-system-a-novel-approach-to-deal-with-proximal-anchor-migration
#4
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
https://www.readbyqxmd.com/read/29298440/effect-of-posterior-fossa-decompression-for-chiari-malformation-i-on-scoliosis
#5
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
https://www.readbyqxmd.com/read/29287817/metal-ion-release-during-growth-friendly-instrumentation-for-early-onset-scoliosis-a-preliminary-study
#6
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
https://www.readbyqxmd.com/read/29287816/comparison-of-percentile-weight-gain-of-growth-friendly-constructs-in-early-onset-scoliosis
#7
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/29226947/phenotype-expansion-and-development-in-kosaki-overgrowth-syndrome
#8
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
https://www.readbyqxmd.com/read/29212690/quantifying-the-law-of-diminishing-returns-in-magnetically-controlled-growing-rods
#9
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
https://www.readbyqxmd.com/read/29190173/detection-of-bacteria-colonizing-titanium-spinal-implants-in-children
#10
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...
November 30, 2017: Surgical Infections
https://www.readbyqxmd.com/read/29182111/the-effect-of-distraction-based-growth-friendly-spinal-instrumentation-on-growth-in-early-onset-scoliosis
#11
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
https://www.readbyqxmd.com/read/29170849/myh7-mutation-associated-with-two-phenotypes-of-myopathy
#12
Nan Li, Zhe Zhao, Hongrui Shen, Qi Bing, Xuan Guo, Jing Hu
The mutations of MYH7 (slow skeletal/β-cardiac myosin heavy chain) are commonly found in familial hypertrophic/dilated cardiomyopathy, and also can cause Laing early-onset distal myopathy (LDM), myosin storage myopathy (MSM), and congenital myopathy with fiber-type disproportion (CFTD). Here we report two cases whose diagnosis was hereditary myopathy according to clinical feature and muscle pathology analysis. High-throughput genomic sequencing (next generation sequencing) was performed to validate the diagnosis...
November 24, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29164086/a-novel-missense-mutation-of-the-nsd1-gene-associated-with-overgrowth-in-three-generations-of-an-italian-family-case-report-differential-diagnosis-and-review-of-mutations-of-nsd1-gene-in-familial-sotos-syndrome
#13
Gianluigi Laccetta, Francesca Moscuzza, Angela Michelucci, Andrea Guzzetta, Sara Lunardi, Francesca Lorenzoni, Paolo Ghirri
Sotos syndrome (SoS) is characterized by overgrowth of prenatal onset, learning disability, and characteristic facial appearance; it is usually due to haploinsufficiency of NSD1 gene at chromosome 5q35. An Italian child was born at 37 weeks of gestation (weight 2,910 g, 25th-50th centiles; length 50 cm, 75th centile; head circumference 36 cm, 97th centile) showing cryptorchidism on the right side, hypertelorism, dolichocephaly, broad and prominent forehead, and narrow jaw; the pregnancy was worsened by maternal preeclampsia and gestational diabetes, and his mother had a previous history of four early miscarriages...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29064871/best-practice-guidelines-for-surgical-site-infection-prevention-with-surgical-treatment-of-early-onset-scoliosis
#14
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/29049269/long-term-surgical-outcomes-after-early-definitive-spinal-fusion-for-early-onset-scoliosis-with-neurofibromatosis-type-1-at-mean-follow-up-of-14-years
#15
Ryoji Tauchi, Noriaki Kawakami, Michael Anton Castro, Tetsuya Ohara, Toshiki Saito, Kazuaki Morishita, Ippei Yamauchi
BACKGROUND: Several authors mentioned surgical outcome for scoliosis associated with neurofibromatosis type 1 (NF-1). However, no studies have summarized long-term surgical outcomes after surgical treatment of early-onset scoliosis (EOS) with NF-1. The purpose of this study is to evaluate the long-term surgical outcome after early definitive spinal fusion for EOS in NF-1 patients. METHODS: We performed a retrospective review on a cohort of 11 patients diagnosed with EOS associated with NF-1 between 1990 and 2009 in our hospital...
October 18, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29049087/raising-mean-arterial-pressure-alone-restores-20-of-intraoperative-neuromonitoring-losses
#16
Joshua Yang, David L Skaggs, Priscella Chan, Suken A Shah, Michael G Vitale, Geraldine Neiss, Nicholas Feinberg, Lindsay M Andras
STUDY DESIGN: Multicenter prospective. OBJECTIVE: To assess the effect of intraoperative interventions in restoring intraoperative neuromonitoring (IONM) signals in pediatric spine surgery. SUMMARY OF BACKGROUND DATA: No prior studies have prospectively examined the rate of return of IONM signals by increasing blood pressure (BP) alone. METHODS: Patients undergoing posterior spinal deformity surgery were enrolled at their preoperative appointment...
October 18, 2017: Spine
https://www.readbyqxmd.com/read/29016427/comparison-of-intended-lengthening-of-magnetically-controlled-growing-rods-ultrasound-versus-x-ray
#17
Mutlu Cobanoglu, Suken A Shah, Peter Gabos, Kenneth Rogers, Petya Yorgova, Geraldine Neiss, Leslie Grissom, William G Mackenzie
BACKGROUND: In the treatment of early onset scoliosis (EOS), there has been a trend to use magnetically controlled growing rods (MCGR) in order to reduce the number of surgeries. To confirm the amount of lengthening, spine radiographs were required. Recently, ultrasound (US) has been added to monitor lengthening of MCGR to avoid radiation exposure. Our aim was to determine whether US is as accurate as plain radiography (x-ray) in determining the amount of length achieved at individual MCGR lengthening episodes...
October 9, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28984540/evolution-of-the-postoperative-sagittal-spinal-profile-in-early-onset-scoliosis-is-there-a-difference-between-rib-based-and-spine-based-growth-friendly-instrumentation
#18
Zhonghui Chen, Song Li, Yong Qiu, Zezhang Zhu, Xi Chen, Liang Xu, Xu Sun
OBJECTIVE Although the vertical expandable prosthetic titanium rib (VEPTR) and growing rod instrumentation (GRI) encourage spinal growth via regular lengthening, they can create different results because of their different fixation patterns and mechanisms in correcting scoliosis. Previous studies have focused comparisons on coronal plane deformity with minimal attention to the sagittal profile. In this retrospective study, the authors aimed to compare the evolution of the sagittal spinal profile in early-onset scoliosis (EOS) treated with VEPTR versus GRI...
October 6, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28960046/a-mild-form-of-cog5-defect-showing-early-childhood-onset-friedreich-s-ataxia-like-phenotypes-with-isolated-cerebellar-atrophy
#19
Young Ok Kim, Misun Yun, Jae Ho Jeong, Seong Min Choi, Seul Kee Kim, Woong Yoon, Chungoo Park, Yeongjin Hong, Young Jong Woo
Progressive cerebellar ataxias are rare diseases during childhood, especially under 6 years of age. In a single family, three affected siblings exhibited Friedreich's-ataxia-like phenotypes before 2 years of age. They had progressive cerebellar atrophy, intellectual disability, and scoliosis. Although their phenotypes were similar to those observed in patients with autosomal recessive cerebellar ataxias, other phenotypes (e.g., seizure, movement disorders, ophthalmologic disturbance, cardiomyopathy, and cutaneous disorders) were not noted in this family...
November 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28926385/graduation-protocol-after-growing-rod-treatment-removal-of-implants-without-new-instrumentation-is-not-a-realistic-approach
#20
Ismail Aykut Kocyigit, Z Deniz Olgun, H Gokhan Demirkiran, Mehmet Ayvaz, Muharrem Yazici
BACKGROUND: The growing rod remains an effective option in the treatment of early-onset scoliosis, and has previously been likened to an internal brace. While details of growing-rod treatment have been largely agreed on, the appropriate end point remains a matter of controversy. A decision was made in 2004, at the beginning of growing-rod treatment at our institution, to remove longitudinal instrumentation when the period of lengthening concluded and, similar to discontinuing a brace, leave the spine free...
September 20, 2017: Journal of Bone and Joint Surgery. American Volume
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