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https://www.readbyqxmd.com/read/29397630/-radiological-analysis-of-coronal-and-sagittal-spinopelvic-parameters-in-patients-with-degenerative-lumbar-kyphoscoliosis
#1
C J Liu, Z Q Zhu, S Duan, K F Wang, W W Xia, S Xu, H Y Liu
Objective: To review and compare radiological parameters between degenerative lumbar kyphoscoliosis (DLKS) and degenerative lumbar kyphosis (DLK), and analyze the relationships between coronal and sagittal deformities and compensatory mechanisms of sagittal balance. Methods: A total of 82 patients with lumbar degenerative deformities were enrolled for our radiographic study at Department of Spinal Surgery, Peking University People's Hospital from January 2016 to May 2017. These patients were divided into two groups: DLKS group (39 patients) with lumbar coronal and sagittal deformities, and DLK group (43 patients) just with lumbar sagittal deformity...
February 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29397629/-comparison-of-clinical-outcome-between-utilized-second-sacral-alar-iliac-and-iliac-screw-on-kyphoscoliosis-patients
#2
Z Liu, C C Tseng, Z H Zhao, J Li, B Wang, Y Yu, B P Qian, X Sun, Y Qiu, Z Z Zhu
Objective: To compare the clinical outcome and health related quality of life(HRQoL)of patients with degenerative spinal deformity who underwent spino-pelvic fixation utilized second sacral alar-iliac(S(2)AI)with patient utilized traditional iliac screw(IS). Methods: Patients diagnosed as degenerative spinal deformity who underwent spino-pelvic fixation utilized either S(2)AI screw or Iliac screw at Department of Spine Surgery of Drum Tower hospital from January 2013 to January 2016 were retrospectively analyzed...
February 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/29334774/three-column-osteotomy-for-adult-spine-deformity-comparison-of-outcomes-and-complications-between-kyphosis-and-kyphoscoliosis
#3
Jun Qiao, Lingyan Xiao, Xu Sun, Zhen Liu, Zezhang Zhu, Bangping Qian, Yong Qiu
BACKGROUND: To compare the surgical outcomes and complications between kyphosis and kyphoscoliosis when using three-column osteotomies. METHODS: Adult spine deformity (ASD) patients with three column osteotomies from March 2005 to December 2014 in our center were retrospectively reviewed. Pre- and postoperative standing postero-anterior and lateral radiographs of the entire spine were obtained. Scoliosis Research Society-22 questionnaire [SRS-22] and Oswestry Disability Index [ODI] were administered preoperatively, postoperatively (surveys within 2 months after surgery), and at final follow-up...
January 15, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29326877/natural-history-of-morquio-a-patient-with-tracheal-obstruction-from-birth-to-death
#4
Caitlin Doherty, Lauren W Averill, Mary Theroux, William G Mackenzie, Christian Pizarro, Robert W Mason, Shunji Tomatsu
Morquio A syndrome (mucopolysaccharidosis IVA, MPS IVA) is a lysosomal storage disease caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase, resulting in systemic accumulation of the partially degraded glycosaminoglycans (GAGs), keratan sulfate and chondroitin-6-sulfate. The accumulation of these GAGs leads to distinguishing features as skeletal dysplasia with disproportionate dwarfism, short neck, kyphoscoliosis, pectus carinatum, tracheal obstruction, coxa valga, genu valgum, and joint laxity...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29280899/surgical-correction-of-severe-kyphoscoliosis-associated-with-crouzon-syndrome-with-serious-postoperative-respiratory-problems-a-case-report
#5
Taro Umezu, Nobuyuki Fujita, Mitsuru Yagi, Osahiko Tsuji, Narihito Nagoshi, Ken Ishii, Masaya Nakamura, Morio Matsumoto, Kota Watanabe
CASE: A 12-year-old girl with Crouzon syndrome presented to our hospital with scoliosis (114°) and kyphosis from T8 to T12 (138°). After she had been in halo-gravity traction for 2 weeks, we performed posterior correction and fusion surgery from T3 to L3, with a posterior vertebral column resection of T10. She experienced postoperative respiratory failure and remained on a ventilator for 4 weeks. With rehabilitation, the respiratory function had recovered by postoperative week 8. At the 2-year follow-up, there was no loss of correction or any other complication...
December 27, 2017: JBJS Case Connector
https://www.readbyqxmd.com/read/29261580/ehlers-danlos-syndrome-type-ivb-and-tracheobronchomegaly
#6
Saniye Girit, Ebru Senol, Yakup Cag, Ozge Karatas, Tamer Baysal
Tracheobronchomegaly (TBM) is a rare disease of unknown etiology, characterized by dilatation of the trachea and main bronchi. Despite its rarity, TBM has been reported to accompany several conditions, particularly Ehlers-Danlos syndrome. Herein, we report on a 15-year-old patient who was diagnosed with TBM on the basis of radiologic and endoscopic findings and with Ehlers-Danlos syndrome type IVB presenting with marfanoid features, severe kyphoscoliosis, increased skin elasticity, and ongoing hypotonia since the neonatal period...
January 2018: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/29260614/neuroimaging-findings-in-pallister-killian-syndrome
#7
Emil Jernstedt Barkovich, Tarannum Musvee Lateef, Matthew T Whitehead
Pallister-Killian syndrome (PKS) is a rare chromosomal duplication disorder caused by additional copies of the short arm of chromosome 12 (12p). Clinically PKS is characterized by craniofacial dysmorphism with neonatal frontotemporal alopecia, hypertelorism, and low-set ears as well as kyphoscoliosis, severe intellectual disability, epilepsy, and abnormal muscle tone. Comprehensive high-resolution brain MR findings of PKS in childhood have not been previously illustrated in the medical literature. We present detailed neuroimaging findings from a child with PKS and thoroughly review previously reported structural brain abnormalities in this patient population...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/29260090/a-recurrent-non-penetrant-sequence-variant-p-arg266cys-in-growth-differentiation-factor-3-gdf3-in-a-female-with-unilateral-anophthalmia-and-skeletal-anomalies
#8
Tanya Bardakjian, Max Krall, Di Wu, Richard Lao, Paul Ling-Fung Tang, Eunice Wan, Sarina Kopinsky, Adele Schneider, Pui-Yan Kwok, Anne Slavotinek
Purpose: The genetic causes of anophthalmia, microphthalmia and coloboma remain poorly understood. Missense mutations in Growth/Differentiation Factor 3 (GDF3) gene have previously been reported in patients with microphthalmia, iridial and retinal colobomas, Klippel-Feil anomaly with vertebral fusion, scoliosis, rudimentary 12th ribs and an anomalous right temporal bone. We used whole exome sequencing with a trio approach to study a female with unilateral anophthalmia, kyphoscoliosis and additional skeletal anomalies...
September 2017: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29236131/trends-in-incidence-and-long-term-outcomes-of-myelomeningocele-in-british-columbia
#9
Taylor North, Alexander Cheong, Paul Steinbok, Julia Ae Radic
PURPOSE: Myelomeningocele is typically a disabling condition that results in neurologic, orthopedic, and urologic morbidity. The aim of this study was to examine the trends over time in both incidence and outcomes of myelomeningocele (MMC) in British Columbia (BC). METHODS: A retrospective chart review was performed of all children with MMC followed in the British Columbia Children's Hospital (BCCH) Spinal Cord Clinic between 1971 and 2016. The incidence of new MMC cases and the long-term outcomes of MMC were compared between two 10-year cohorts...
December 13, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29033731/ultrasound-guided-continuous-spinal-anesthesia-for-cesarean-section-in-a-parturient-with-scoliosis-corrected-with-harrington-s-rod-surgery
#10
Amer Majeed, Iftikhar Ahmed, Ghadah Jamaan Alkahtani, Nasser Abdullah Altahtam
With rapid improvement in healthcare in Saudi Arabia, increasing number of women with surgically corrected kyphoscoliosis are likely to present for cesarean section (CS) or vaginal delivery requiring anesthesia or analgesia. Despite the surgical correction, these patients have poor cardiopulmonary reserves which increase the risks associated with general anesthesia. Whereas altered vertebral anatomy from previous surgery and the presence of metal work in spine make performing of regional anesthesia (RA) difficult and unpredictable, we report anesthetic management of such a patient who underwent CS using continuous spinal anesthesia technique...
October 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29033379/is-vertebral-column-resection-vcr-necessary-in-correcting-severe-and-rigid-thoracic-kyphoscoliosis-a-single-institution-surgical-experience
#11
Fan Hengwei, Li Xueshi, Huang Zifang, Sui Wenyuan, Lang Chuandong, Yang Jingfan, Yang Junlin
BACKGROUND: Three-column spinal osteotomies were developed to address severe and stiff kyphoscolisis. However, the optimal choice of osteotomy in these patients is still unclear. This retrospective study aims to compare the outcomes and complications between grade 4 and grade 5 osteotomies in patients with severe and stiff thoracic kyphoscoliosis. METHODS: Forty-five patients with severe and stiff thoracic kyphoscoliosis who underwent three-column osteotomy in the thoracic spine were classified into 2 groups according to the grade of osteotomy...
October 12, 2017: World Neurosurgery
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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