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https://www.readbyqxmd.com/read/28094603/lethal-mechanisms-in-gastric-volvulus
#1
Kimberley J Omond, Roger W Byard
A 55-year-old wheelchair-bound woman with severe cerebral palsy was found at autopsy to have marked distention of the stomach due to a volvulus. The stomach was viable, and filled with air and fluid and had pushed the left dome of the diaphragm upwards causing marked compression of the left lung with a mediastinal shift to the right (including the heart). There was no evidence of gastric perforation, ischaemic necrosis or peritonitis. Removal of the organ block revealed marked kyphoscoliosis. Histology confirmed the viability of the stomach and biochemistry showed no dehydration...
January 1, 2017: Medicine, Science, and the Law
https://www.readbyqxmd.com/read/28089323/neurophysiological-monitoring-of-lumbar-spinal-nerve-roots-a-case-report-of-postoperative-deficit-and-literature-review
#2
Yuguang Chen, Baoqing P Wang, Junlin Yang, Yaolong Deng
INTRODUCTION: Intraoperative neurophysiological monitoring (IONM) has proven to help reduce the probability of postoperative neurological deficit for spinal deformity correctional surgeries. However, in rare cases new deficits may still happen. We report a surgical case in which the patient had postoperative paralysis. We would like to call for more case reports with postoperative neurological deficits as they present difficult clinical cases. PRESENTATION OF CASE: A 61-year-old male patient with severe thoracolumbar kyphoscoliosis underwent posterior spinal correction and fusion with segmental T10-L5 pedicle screws and rods instrumentation with IONM...
November 19, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28045856/a-multiparametric-alarm-criterion-for-motor-evoked-potential-monitoring-during-spine-deformity-surgery
#3
Martín J Segura, María E Talarico, Mariano A Noel
PURPOSE: This is a pilot study to compare changes in the amplitude, area below the curve, number of phases, duration, and latency of the intraoperative transcranial motor evoked potentials (TcMEP) for early detection of impending spinal cord injury. An empirical ratio calculated by a combination of the above-mentioned parameters was also assessed. METHODS: Intraoperative TcMEP recordings from five patients presenting with neuromuscular kyphoscoliosis, idiopathic scoliosis, achondroplasia and lumbar kyphosis, congenital kyphosis, and achondroplasia with cervical instability were reviewed...
January 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28011632/a-developmental-transcriptomic-analysis-of-pax1-and-pax9-in-embryonic-intervertebral-disc-development
#4
V Sivakamasundari, Petra Kraus, Wenjie Sun, Xiaoming Hu, Siew Lan Lim, Shyam Prabhakar, Thomas Lufkin
Pax1 and Pax9 play redundant, synergistic functions in the patterning and differentiation of the sclerotomal cells that give rise to the vertebral bodies and intervertebral discs (IVD) of the axial skeleton. They are conserved in mice and humans, whereby mutations/deficiency of human PAX1/PAX9 have been associated with kyphoscoliosis. By combining cell-type specific transcriptome and ChIP-sequencing data, we identified the roles of Pax1/Pax9 in cell proliferation, cartilage development and collagen fibrillogenesis, which are vital in early IVD morphogenesis...
December 23, 2016: Biology Open
https://www.readbyqxmd.com/read/27979613/mucopolysaccharidosis-iva-and-glycosaminoglycans
#5
REVIEW
Shaukat Khan, Carlos J Alméciga-Díaz, Kazuki Sawamoto, William G Mackenzie, Mary C Theroux, Christian Pizarro, Robert W Mason, Tadao Orii, Shunji Tomatsu
Mucopolysaccharidosis IVA (MPS IVA; Morquio A: OMIM 253000) is a lysosomal storage disease with an autosomal recessive trait caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to accumulation of specific glycosaminoglycans (GAGs): chondroitin-6-sulfate (C6S) and keratan sulfate (KS). C6S and KS are mainly produced in the cartilage. Therefore, the undegraded substrates are stored primarily in cartilage and in its extracellular matrix (ECM), leading to a direct impact on cartilage and bone development, and successive systemic skeletal dysplasia...
November 29, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27977582/spine-malformation-complex-in-3-diverse-syndromic-entities-case-reports
#6
Ali Al Kaissi, Andreas van Egmond-Fröhlich, Sergey Ryabykh, Polina Ochirov, Vladimir Kenis, Jochen G Hofstaetter, Franz Grill, Rudolf Ganger, Susanne Gerit Kircher
RATIONALE: Clinical and radiographic phenotypic characterizations were the base line tool of diagnosis in 3 syndromic disorders in which congenital cervico-thoracic kyphosis was the major deformity. PATIENTS CONCERNS: Directing maximal care toward the radiographic analysis is not only the axial malformation but also toward the appendicular abnormalities was our main concern. We fully documented the diversity of the spine phenotypic malformation complex via the clinical and radiographic phenotypes...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27942940/growth-modulation-and-remodeling-by-means-of-posterior-tethering-technique-for-correction-of-early-onset-scoliosis-with-thoracolumbar-kyphosis
#7
Alaaeldin A Ahmad, Loai Aker, Yahia Hanbali, Aesha Sbaih, Zaher Nazzal
PURPOSE: The aim of this study is to evaluate the role of the non-fusion instrumented procedure with compression adjunct to lengthening by distraction in facilitating spinal modulation of the wedged peak vertebra, in patients with congenital thoracolumbar kyphosis/kyphoscoliosis according to the Hueter-Volkmann law. The authors seek to address the progressive modulation of the most wedged vertebra by analyzing the subjects' pre-operative and latest follow-up sagittal radiograph. METHODS: Ongoing data collection of 14 peak wedged vertebra modulation during surgical management of 13 patients with Type I congenital thoracolumbar kyphosis (5 patients) or kyphoscoliosis (8 patients)...
December 9, 2016: European Spine Journal
https://www.readbyqxmd.com/read/27942184/an-interesting-case-of-renal-amyloidosis
#8
A Hajra, D Bandyopadhyay
In amyloidosis, there is an extracellular deposition of beta-sheet fibrils. Over 25 proteins have been demonstrated to form amyloid. One of them is Ig amyloid light (AL) chains. We are presenting a 40-year-old female who presented with progressive kyphoscoliosis for last 2 years following a minor trauma and bilateral pedal edema for last 3 months. On further investigation, we found that she had a biclonal variety of MM with amyloidosis of kidney leading to massive proteinuria. Very few case reports are available where patient with biclonal variety of MM develop renal amyloidosis...
November 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27935449/multiple-hook-fixation-in-revision-spinal-deformity-surgery-for-patients-with-a-previous-multilevel-fusion-mass-technical-note-and-preliminary-outcomes
#9
Ning Liu, Kirkham B Wood
OBJECTIVE A previous multilevel fusion mass encountered during revision spinal deformity surgery may obscure anatomical landmarks, making instrumentation unworkable or incurring substantial blood loss and operative time. This study introduced a surgical technique of multiple-hook fixation for fixating previous multilevel fusion masses in revision spinal deformity surgeries and then evaluated its outcomes. METHODS Patients with a previous multilevel fusion mass who underwent revision corrective surgery down to the lumbosacral junction were retrospectively studied...
December 9, 2016: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/27930594/revision-surgery-after-pregnancy-in-a-patient-with-congenital-kyphoscoliosis-a-case-report
#10
Zhikun Li, Fei Wang, Wei Xu, Yifan Li, Xiaodong Zhu
RATIONALE: Rod breakage during pregnancy and delivery has never been described in a patient who has undergone surgery for congenital scoliosis (CS). Here, we present an unusual but significant case of revision surgery. PATIENT CONCERNS: A 29-year-old woman presented with low back pain during pregnancy after posterior osteotomy, correction and fusion at T9 to L5 for CS. Radiographs during follow-up, 4 months after the patient gave birth, demonstrated rod breakage...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27927497/acute-renal-infarction-after-lateral-lumbar-interbody-spinal-fusion-for%C3%A2-kyphoscoliosis
#11
Sanjeev J Suratwala, MaryAnne Cronin, Katelyn Kondra, Vincent Leone
STUDY DESIGN: Case report. OBJECTIVES: Case report of acute renal infarction following lateral interbody fusion with posterior instrumentation for adult lumbar kyphoscoliosis. SUMMARY OF BACKGROUND DATA: This is the first reported renal infarction following minimally invasive lateral interbody fusion and posterior spinal instrumentation. METHODS: We report a case of acute renal infarction in a 72-year-old woman following direct lateral interbody fusion (DLIF) with percutaneous pedicle screw and rod fixation from L1 to L5 for correction of kyphoscoliosis...
September 2016: Spine Deformity
https://www.readbyqxmd.com/read/27927496/lumbosacral-junctional-failures-after-long-spinal-fusion-for-adult-spinal-deformity-which-vertebra-is-the-preferred-distal-instrumented-vertebra
#12
Tatsuya Yasuda, Tomohiko Hasegawa, Yu Yamato, Sho Kobayashi, Daisuke Togawa, Tomohiro Banno, Hideyuki Arima, Shin Oe, Yukihiro Matsuyama
STUDY DESIGN: A retrospective study. OBJECT: To investigate the rate of lumbosacral junction complication after long corrective spinal fusion for degenerative kyphoscoliosis cases with different lower instrumented vertebra (LIV) of L5, S1 (non-iliac group), and ilium (iliac group). SUMMARY OF BACKGROUND DATA: In long spinal fusion, high mechanical stress is concentrated at the fusion ends, especially at the lumbosacral junction. There are conflicting views regarding the selection of lower instrumented vertebra in long spinal fusion for adult spinal deformity...
September 2016: Spine Deformity
https://www.readbyqxmd.com/read/27905128/ehlers-danlos-syndrome-related-to-fkbp14-mutations-detailed-cutaneous-phenotype
#13
A C Bursztejn, M Baumann, D Lipsker
In 2012, a new Ehlers-Danlos (ED) variant, characterized by severe progressive kyphoscoliosis, neonatal myopathy and hearing loss, with normal urinary lysylpyridinoline to hydroxylysylpyridinoline ratio and most often a recurrent homozygous mutation in the FKBP14 gene, was reported. Because one of the major affected tissues in ED syndrome is the skin, recognition of the cutaneous features of this newly recognized EDS variant is important. We describe the cutaneous phenotype of an adolescent girl harbouring the recurrent homozygous FKBP14 mutation...
January 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/27896274/halo-gravity-traction-is-associated-with-reduced-bone-mineral-density-of-patients-with-severe-kyphoscoliosis
#14
Xiao Han, Weixiang Sun, Yong Qiu, Leilei Xu, Shifu Sha, Benlong Shi, Huang Yan, Zhen Liu, Zezhang Zhu
Background. Halo gravity traction (HGT) is one of the most commonly used perioperative techniques for the treatment of severe kyphoscoliosis. This study was to explore the influence of HGT on the BMD of these patients. Methods. Patients with severe kyphoscoliosis treated by preoperative HGT for at least 2 months were included. Patients' BMD were assessed by dual-energy X-ray absorptiometry at lumbar spine (LS, L2-L4) and femur neck (FN) of the nondominant side. The weight and duration of traction, as well as baseline characteristics, were recorded...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27858241/repeated-posterior-dislocation-of-total-hip-arthroplasty-after-spinal-corrective-long-fusion-with-pelvic-fixation
#15
Hiroki Furuhashi, Daisuke Togawa, Hiroshi Koyama, Hironobu Hoshino, Tatsuya Yasuda, Yukihiro Matsuyama
PURPOSE: Several reports have indicated that anterior dislocation of total hip arthroplasty (THA) can be caused by spinal degenerative changes with excessive pelvic retroversion. However, no reports have indicated that posterior dislocation can be caused by fixed pelvic anteversion after corrective spine surgery. We describe a rare case experiencing repeated posterior THA dislocation that occurred at 5 months after corrective spinal long fusion with pelvic fixation. METHODS: A 64-year-old woman had undergone bilateral THA at 13 years before presenting to our institution...
November 17, 2016: European Spine Journal
https://www.readbyqxmd.com/read/27852504/bilateral-rib-based-distraction-to-the-pelvis-for-the-management-of-congenital-gibbus-deformity-in-the-growing-child-with-myelodysplasia
#16
John T Smith
BACKGROUND: Congenital gibbus deformity of the spine associated with myelodysplasia is a challenging problem in the growing child and is commonly associated with skin breakdown and chronic infection. Surgical solutions including kyphectomy, flap closure of the skin, and early spinal fusion are associated with a high rate of complications and, ultimately, a short trunk due to stoppage of spinal growth. The purpose of this article is to describe our early results in using a rib-based distraction to the pelvis without vertebral resection and fusion to manage this deformity...
January 2016: Spine Deformity
https://www.readbyqxmd.com/read/27831994/pseudarthrosis-repair-using-autologous-cultured-osteoblasts-in-complex-type-1-neurofibromatosis-spinal-deformity-a-case-report-and-review-of-the-literature
#17
Young-Hoon Kim, Genriech N Reoyan, Kee-Yong Ha, Chul-Kyu Kim
STUDY DESIGN: A case report and literature review. OBJECTIVE: To report a late dislocation of the vertebra caused by progressive dural ectasia combined with type-1 neurofibromatosis (NF-1) and the clinical results of pseudarthrosis repairs using autologous cultured osteoblasts. SUMMARY OF BACKGROUND DATA: NF-1 is a well-known genetic disorder that is commonly characterized by spinal deformities including kyphoscoliosis. Late dislocation of the vertebra resulting from progressive dural ectasia after surgical correction of NF-1 scoliosis is a very rare occurrence, and pseudarthrosis frequently develops after surgical intervention for this complex spinal deformity...
November 15, 2016: Spine
https://www.readbyqxmd.com/read/27771677/cerebrotendinous-xanthomatosis-without-skin-changes-diagnostic-delay-and-confirmation-by-genetic-analysis
#18
Shilpa D Kulkarni, Meenal Garg, Rafat Sayed
BACKGROUND: Cerebrotendinous xanthomatosis is an inherited lipid storage disease manifesting with infantile onset diarrhea, cataracts, xanthomas and adult-onset neurological dysfunction with cerebellar signs and neuropathy. CASE CHARACTERISTICS: 10-year-old boy presented with progressive ataxia, neuropathy and cataracts. Over 6 years, he developed dementia, kyphoscoliosis with worsening ataxia, and neuropathy. OUTCOME: Sterol analysis and CYP27A1 sequencing confirmed the diagnosis...
October 8, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27766191/vertebral-column-resection-for-kyphoscoliosis-in-a-patient-with-ehlers-danlos-syndrome-an-intraoperative-neurophysiological-monitoring-alert
#19
Faisal R Jahangiri, Sami Al Eissa, Samir Sayegh, Fahad Al Helal, Shomoukh A Al-Sharif, Monerah M Annaim, Sheryar Muhammad, Tanweer Aziz
A 16-year-old male patient with Ehler-Danlos syndrome (EDS) and a back deformity since birth presented with severe kyphoscoliosis. The patient was neurologically intact but had respiratory and cardiac insufficiencies. A two-stage vertebral column resection (VCR) at T9-T10 with multiple level fusion with multimodality intraoperative neurophysiological monitoring (IONM) was planned.  During the first stage, pedicle screws were placed at multiple spinal levels above and below the VCR level. Upper and lower somatosensory evoked potentials (SSEP), transcranial electrical motor evoked potentials (TCeMEP), and electromyography were monitored continuously and showed no significant changes...
August 31, 2016: Curēus
https://www.readbyqxmd.com/read/27758814/unusual-case-of-globe-perforation-the-brittle-cornea-without-systemic-manifestations
#20
Shilpa Ajit Joshi, Shalomith Uppapalli, Pranav More, Madan Deshpande
Brittle cornea syndrome is a rare generalised connective tissue disorder with ocular features like keratoglobus or keratoconus, severe corneal thinning and a high risk of perforation. Various authors in different case reports and case series have brought out the fact that brittle cornea is a disorder with characteristic systemic manifestations such as deafness, joint hypermobility, hyperelasticity of skin, kyphoscoliosis and dental abnormalities alongwith ophthalmic features. We report a case of globe perforation following trivial trauma, in an individual with brittle cornea without any extraocular manifestations, posing a challenge in the diagnosis and dilemma in surgical repair of cornea, restoration of globe integrity and visual rehabilitation...
October 7, 2016: BMJ Case Reports
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