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kyphoscoliosis

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https://www.readbyqxmd.com/read/28985988/a-novel-screwing-method-to-prevent-decubitus-and-skin-ulcer-in-severe-kyphoscoliosis-after-spinal-cord-injury-a-case-report
#1
Shoji Seki, Yoshiharu Kawaguchi, Hayato Mine, Tomoatsu Kimura
No abstract text is available yet for this article.
October 3, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28979012/focal-dermal-hypoplasia-goltz-syndrome-a-cross-sectional-study-from-eastern-india
#2
Sudip Kumar Ghosh, Abhijit Dutta, Sharmila Sarkar, Shanka Subhra Nag, Surajit Kumar Biswas, Prabhakar Mandal
INTRODUCTION: Focal dermal hypoplasia (Goltz syndrome), is an extremely rare genetic disorder characterized by distinct skin manifestations and a wide range of abnormalities involving the ocular, dental, skeletal, urinary, gastrointestinal, cardiovascular, and central nervous systems. The objective of the present series is to emphasize the different typical as well as unusual features of this rare syndrome. METHOLOGY: This cross-sectional observational study was performed over a period of 8 years in a tertiary care hospital of Eastern India...
September 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28965455/single-stage-deformity-correction-in-children-with-neurogenic-kyphoscoliosis-nuances-and-a-proposal-for-risk-stratification
#3
Suhas Udayakumaran, Sajesh K Menon, Chiazor U Onyia, Naveen Tahasildar
OBJECTIVE Neurogenic kyphoscoliosis in pediatric patients has varied causes and diverse management options. The most common management strategy is to stage the orthopedic and neurosurgical aspects of the correction at an interval of 3-6 months from each other. The aim of this study was to report the authors' experience with correction of deformity in children with associated neurological abnormalities requiring intervention as a single-stage surgical treatment. METHODS Of 591 pediatric patients with scoliosis treated at the Amrita Institute of Medical Sciences and Research Centre between January 2001 and January 2011, the authors identified all patients with neurogenic kyphoscoliosis who underwent deformity correction and a neurosurgical procedure in the same surgical session when they were younger than 15 years...
October 2017: Neurosurgical Focus
https://www.readbyqxmd.com/read/28965454/risk-factors-associated-with-short-term-complications-and-mortality-after-pediatric-spinal-arthrodesis
#4
Nancy Abu-Bonsrah, C Rory Goodwin, Gezzer Ortega, Fizan Abdullah, Edward Cornwell, Rafael De la Garza-Ramos, Mari L Groves, Michael Ain, Paul D Sponseller, Daniel M Sciubba
OBJECTIVE Spinal arthrodesis is routinely performed in the pediatric population. However, there is limited information on the short-term outcomes of pediatric patients who have undergone spine fusion. Thus, the authors conducted a retrospective review of the Pediatric National Surgical Quality Improvement Program (NSQIP) database to determine the short-term mortality, complication, reoperation, and readmission rates of pediatric patients who underwent spinal arthrodesis for all indications. METHODS The Pediatric NSQIP database was queried for all patients who underwent spinal arthrodesis between 2012 and 2014...
October 2017: Neurosurgical Focus
https://www.readbyqxmd.com/read/28944085/false-negative-transcranial-motor-evoked-potentials-tcmeps-during-surgery-for-congenital-lumbar-kyphoscoliosis-a-case-report
#5
Masayuki Ohashi, Kei Watanabe, Kenta Furutani, Toru Hirano, Keiichi Katsumi, Hirokazu Shoji, Tatsuki Mizouchi, Naoto Endo
INTRODUCTION: Transcranial motor evoked potential (TcMEP) monitoring is the gold standard for intra-operative neurological monitoring (IOM) of motor pathways during complex spine surgery because of its high sensitivity and specificity. However, although it is very low, the rate of false-negatives in TcMEP monitoring is not zero. Therefore, over-reliance on TcMEP monitoring can cause potentially preventable motor deficits. CASE PRESENTATION: We report a case of motor deficits due to nerve root stretch after surgical correction of a congenital lumbar kyphoscoliosis in a 56-year-old woman...
2017: Spinal Cord Series and Cases
https://www.readbyqxmd.com/read/28918465/congenital-lumbar-kyphosis-with-skin-ulceration-and-osteomyelitis-in-a-myelomeningocele-child-a-case-report
#6
Benoit Vibert, Marco Turati, Pierre-Yves Rabattu, Marco Bigoni, Ahmad Eid, Aurélien Courvoisier
INTRODUCTION: Kyphosis is a frequent problem in children with spina bifida, and this deformity may cause different complications as respiratory insufficiency, bowel dysfunction, and skin ulcers. CASE REPORT: We report on a 13-year-old myelomeningocele male with a lumbar kyphoscoliosis associated to a septic skin ulceration that resulted in an acute sepsis. An X-ray revealed a kyphosis of 110° and a scoliosis of 25° between T9 and L5. The wound and blood cultures showed Staphylococcus aureus colonization, and an appropriate antibiotic therapy was started...
September 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28885330/fractional-curve-progression-with-maintenance-of-fusion-mass-in-congenital-scoliosis-an-18-year-follow-up-of-a-case-report
#7
Dong-Gune Chang, Jae Hyuk Yang, Se-Il Suk, Seung-Woo Suh, Jin-Hyok Kim, Seung-Joo Lee, Ki-Ho Na, Jung-Hee Lee
RATIONALE: The management of congenital scoliosis concentrates on early diagnosis and proper surgical treatment before the development of severe deformities. Decision making regarding the appropriate fusion levels, proper surgical treatment, and reduction amount of kyphoscoliosis is very important but difficult in the treatment of congenital scoliosis, especially in young children. PATIENT CONCERNS: We report an 11-year follow-up of revision surgery for fractional curve progression after combined anterior and posterior fusion without hemivertebra resection using pedicle screw fixation (PSF) in congenital kyphoscoliosis at age 4 years (a total 18-year follow-up)...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28822231/preoperative-halo-gravity-traction-for-severe-thoracic-kyphoscoliosis-patients-from-tibet-radiographic-correction-pulmonary-function-improvement-nursing-and-complications
#8
Xiaojin Li, Liwen Zeng, Xiaoyin Li, Xuexia Chen, Caixia Ke
BACKGROUND This study investigated the outcomes of preoperative HGT as an adjunct treatment for severe thoracic kyphoscoliosis, its role in radiographic correction, and pulmonary function improvement, together with nursing strategy and incidence of complications. MATERIAL AND METHODS Eleven patients with a mean age of 18.8 years were retrospectively reviewed. Inclusion criteria were: patients with severe kyphoscoliosis (coronal Cobb angle and kyphosis angle ≥80°); duration of HGT ≥8 weeks; patients undergoing HGT for at least 12 h per day; traction weight no less than 40% of body weight; and patients not receiving physical therapies...
August 19, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28777491/identification-of-stac3-variants-in-non-native-american-families-with-overlapping-features-of-carey-fineman-ziter-syndrome-and-moebius-syndrome
#9
Aida Telegrafi, Bryn D Webb, Sarah M Robbins, Carlos E Speck-Martins, David FitzPatrick, Leah Fleming, Richard Redett, Andreas Dufke, Gunnar Houge, Jeske J T van Harssel, Alain Verloes, Angela Robles, Irini Manoli, Elizabeth C Engle, Ethylin W Jabs, David Valle, John Carey, Julie E Hoover-Fong, Nara L M Sobreira
Horstick et al. (2013) previously reported a homozygous p.Trp284Ser variant in STAC3 as the cause of Native American myopathy (NAM) in 5 Lumbee Native American families with congenital hypotonia and weakness, cleft palate, short stature, ptosis, kyphoscoliosis, talipes deformities, and susceptibility to malignant hyperthermia (MH). Here we present two non-Native American families, who were found to have STAC3 pathogenic variants. The first proband and her affected older sister are from a consanguineous Qatari family with a suspected clinical diagnosis of Carey-Fineman-Ziter syndrome (CFZS) based on features of hypotonia, myopathic facies with generalized weakness, ptosis, normal extraocular movements, cleft palate, growth delay, and kyphoscoliosis...
August 4, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28757364/ehlers-danlos-syndrome-kyphoscoliotic-type-due-to-lysyl-hydroxylase-1-deficiency-in-two-children-without-congenital-or-early-onset-kyphoscoliosis
#10
Fleur S van Dijk, Grazia M S Mancini, Alessandra Maugeri, Jan M Cobben
We report two children with Ehlers Danlos, kyphoscoliotic type confirmed by Lysyl Hydroxylase 1 deficiency due to bi-allelic PLOD1 mutations (kEDS-PLOD1) who were initially thought to have either a diagnosis of classical EDS (cEDS) or a neuromuscular disorder due to absence of (congenital) scoliosis. As the two patients reported here illustrate, patients with kEDS-PLOD1 do not always have a kyphoscoliosis present at birth or in the first year of life, neither do they necessarily develop kyphoscoliosis later in infancy...
October 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28746122/anterior-and-posterior-vertebral-column-resection-versus-posterior-only-technique-a-comparison-of-clinical-outcomes-and-complications-in-congenital-kyphoscoliosis
#11
Gokhan Demirkiran, Ozgur Dede, Emre Karadeniz, Deniz Olgun, Mehmet Ayvaz, Muharrem Yazici
STUDY DESIGN: Retrospective cohort. OBJECTIVE: The purpose of this study is to compare the anterior-posterior surgery to posterior-only vertebra resection for congenital kyphoscoliosis in pediatric patients. SUMMARY OF BACKGROUND DATA: Vertebral column resection is a very powerful correction technique mainly used in the treatment of severe and rigid spinal deformities. The technique can be applied with combined anterior and posterior vertebral column resection (APVCR) or posterior-only vertebral column resection (PVCR) approaches...
August 2017: Clinical Spine Surgery
https://www.readbyqxmd.com/read/28741218/asymmetric-pedicle-subtractionosteotomy-apso-guided-by-a-3d-printed-model-to-correct-a-combined-fixed-sagittal-and-coronal-imbalance
#12
Pierre-Pascal Girod, S Hartmann, P Kavakebi, J Obernauer, M Verius, C Thomé
Surgical correction of fixed thoracolumbar deformity is usually achieved by estimating the preoperatively planned correction angles during surgery and is therefore prone to inaccuracy. This is particularly problematic in biplanar deformities. To overcome these difficulties, 3D model for planning, preparation, and simulation of an asymmetric pedicle subtraction osteotomy (aPSO) was printed and used to realign coronal and sagittal balance in case of rigid degenerative kyphoscoliosis. A 59-year-old woman presented with severe back pain and spinal claudication and was diagnosed with a rigid kyphoscoliosis with multilevel spinal stenosis...
July 24, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28669484/vertebral-anomalies-in-craniofacial-microsomia-a-systematic-review
#13
REVIEW
R W Renkema, C J J M Caron, I M J Mathijssen, E B Wolvius, D J Dunaway, C R Forrest, B L Padwa, M J Koudstaal
Craniofacial microsomia (CFM) is characterized by a heterogeneous underdevelopment of the facial structures arising from the first and second branchial arches, but extracraniofacial malformations such as vertebral anomalies also occur. This systematic review provides an overview of the literature on the types and prevalence of vertebral anomalies found in patients with CFM. A systematic search was conducted. Data on the number of patients, patient characteristics, types and prevalence of vertebral anomalies, and other associations between CFM and vertebral anomalies were extracted from the articles identified...
October 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28667723/kyphoscolitic-type-of-ehlers-danlos-syndrome-with-prenatal-stroke
#14
Meriem Zahed-Cheikh, Barthélémy Tosello, Stéphanie Coze, Catherine Gire
BACKGROUND: The kyphoscoliotic type of Ehlers-Danlos syndrome (EDS type VIA) is an autosomal recessive disorder characterized by connective tissue dysplasia. CASE CHARACTERISTICS: We report two children with perinatal stroke; accompanied by neonatal joint hypermobility, hypotonia; and early development of kyphoscoliosis. OUTCOME: Molecular analysis revealed a PLOD1 gene mutation. Our definitive diagnosis was a EDS VIA. MESSAGE: Prenatal brain stroke is a rare clinical feature of EDSVIA...
June 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28617417/a-cohort-of-17-patients-with-kyphoscoliotic-ehlers-danlos-syndrome-caused-by-biallelic-mutations-in-fkbp14-expansion-of-the-clinical-and-mutational-spectrum-and-description-of-the-natural-history
#15
Cecilia Giunta, Matthias Baumann, Christine Fauth, Uschi Lindert, Ebtesam M Abdalla, Angela F Brady, James Collins, Jahannaz Dastgir, Sandra Donkervoort, Neeti Ghali, Diana S Johnson, Ariana Kariminejad, Johannes Koch, Marius Kraenzlin, Nayana Lahiri, Bernarda Lozic, Adnan Y Manzur, Jenny E V Morton, Jacek Pilch, Rebecca C Pollitt, Gudrun Schreiber, Nora L Shannon, Glenda Sobey, Anthony Vandersteen, Fleur S van Dijk, Martina Witsch-Baumgartner, Johannes Zschocke, F Michael Pope, Carsten G Bönnemann, Marianne Rohrbach
PurposeIn 2012 we reported in six individuals a clinical condition almost indistinguishable from PLOD1-kyphoscoliotic Ehlers-Danlos syndrome (PLOD1-kEDS), caused by biallelic mutations in FKBP14, and characterized by progressive kyphoscoliosis, myopathy, and hearing loss in addition to connective tissue abnormalities such as joint hypermobility and hyperelastic skin. FKBP14 is an ER-resident protein belonging to the family of FK506-binding peptidyl-prolyl cis-trans isomerases (PPIases); it catalyzes the folding of type III collagen and interacts with type III, type VI, and type X collagens...
June 15, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28602981/three-dimensional-translations-following-posterior-three-column-spinal-osteotomies-for-the-correction-of-severe-and-stiff-kyphoscoliosis
#16
Xueshi Li, Zifang Huang, Yaolong Deng, Hengwei Fan, Wenyuan Sui, Chongwen Wang, Junlin Yang
BACKGROUND CONTEXT: Posterior three-column spinal osteotomies were shown to be effective to treat severe and stiff kyphoscoliosis. Translations at the site of osteotomy after deformity correction were commonly seen intraoperatively, which might cause potential neurologic deficits. However, this phenomenon was not thoroughly discussed in the current literature. PURPOSE: This study aimed to evaluate the three-dimensional (3D) translations at the three-column osteotomy site and their effects on neurologic outcome in the surgical correction of severe and stiff kyphoscoliosis...
June 7, 2017: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/28578109/reconstruction-of-thoracic-spine-using-a-personalized-3d-printed-vertebral-body-in-adolescent-with-t9-primary-bone-tumor
#17
Wen Jie Choy, Ralph J Mobbs, Ben Wilcox, Steven Phan, Kevin Phan, Chester E Sutterlin
BACKGROUND: Neurosurgery and spine surgery have the potential to benefit from the use of 3-dimensional printing (3DP) technology due to complex anatomic considerations and the delicate nature of surrounding structures. We report a procedure that uses a 3D-printed titanium T9 vertebral body implant post T9 vertebrectomy for a primary bone tumor. CASE DESCRIPTION: A 14-year-old female presented with progressive kyphoscoliosis and a pathologic fracture of the T9 vertebra with sagittal and coronal deformity due to a destructive primary bone tumor...
September 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28537042/safety-and-efficacy-of-percutaneous-nephrolithotomy-in-patients-with-severe-skeletal-deformities
#18
Seyed Habibollah Mousavi-Bahar, Shahriar Amirhasani, Maede Mohseni, Rezgar Daneshdoost
PURPOSE: Treatment of renal calculi in patients with severe skeletal deformities can be challenging. We present our experience in order to provide an assessment of technical difficulties, associated complications, and outcomes of percutaneous nephrolithotomy (PCNL) as a treatment option in this special patient group. MATERIALS AND METHODS: Our study included eight patients treated with PCNL for renal stones. All had severe skeletal deformities including six with severe kyphoscoliosis, one with osteogenesis imperfecta, and another withrickets...
May 23, 2017: Urology Journal
https://www.readbyqxmd.com/read/28532908/the-safety-and-efficiency-of-pvcr-without-anterior-support-applied-in-treatment-of-yang-type-a-severe-thoracic-kyphoscoliosis
#19
Wen-Yuan Sui, Zi-Fang Huang, Yao-Long Deng, Heng-Wei Fan, Jing-Fan Yang, Fo-Bao Li, Jun-Lin Yang
OBJECTIVE: To assess the clinical and radiographic outcomes of posterior vertebral column resection (PVCR) without anterior support in treatment of Yang type A severe rigid thoracic kyphoscoliosis. METHODS: The records of 27 Yang type A severe thoracic kyphoscoliosis patients treated with PVCR without anterior support from January 2010 to September 2013 were analyzed retrospectively. Intraoperative multimodal neurophysiological monitoring was conducted in all patients...
August 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28488683/progressive-hereditary-spastic-paraplegia-caused-by-a-homozygous-ky-mutation
#20
Yuval Yogev, Yonatan Perez, Iris Noyman, Anwar Abu Madegem, Hagit Flusser, Zamir Shorer, Eugene Cohen, Leonid Kachko, Analia Michaelovsky, Ruth Birk, Arie Koifman, Max Drabkin, Ohad Wormser, Daniel Halperin, Rotem Kadir, Ohad S Birk
Twelve individuals of consanguineous Bedouin kindred presented with autosomal recessive progressive spastic paraplegia evident as of age 0-24 months, with spasticity of lower limbs, hyperreflexia, toe walking and equinus deformity. Kyphoscolisois was evident in older patients. Most had atrophy of the lateral aspects of the tongue and few had intellectual disability. Nerve conduction velocity, electromyography and head and spinal cord magnetic resonance imaging were normal in tested subjects. Muscle biopsy showed occasional central nuclei and fiber size variability with small angular fibers...
August 2017: European Journal of Human Genetics: EJHG
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