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Chiari malformation type 1

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https://www.readbyqxmd.com/read/28291422/comparison-of-posterior-fossa-volumes-and-clinical-outcomes-after-decompression-of-chiari-malformation-type-i
#1
Siri Sahib S Khalsa, Alan Siu, Tiffani A DeFreitas, Justin M Cappuzzo, John S Myseros, Suresh N Magge, Chima O Oluigbo, Robert F Keating
OBJECTIVE Previous studies have indicated an association of Chiari malformation Type I (CM-I) and a small posterior fossa. Most of these studies have been limited by 2D quantitative methods, and more recent studies utilizing 3D methodologies are time-intensive with manual segmentation. The authors sought to develop a more automated tool to calculate the 3D posterior fossa volume, and correlate its changes after decompression with surgical outcomes. METHODS A semiautomated segmentation program was developed, and used to compare the pre- and postoperative volumes of the posterior cranial fossa (PCF) and the CSF spaces (cisterna magna, prepontine cistern, and fourth ventricle) in a cohort of pediatric patients with CM-I...
February 17, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28291403/a-rat-model-of-chronic-syringomyelia-induced-by-epidural-compression-of-the-lumbar-spinal-cord
#2
Ji Yeoun Lee, Shin Won Kim, Saet Pyoul Kim, Hyeonjin Kim, Jung-Eun Cheon, Seung-Ki Kim, Sun Ha Paek, Dachling Pang, Kyu-Chang Wang
OBJECTIVE There has been no established animal model of syringomyelia associated with lumbosacral spinal lipoma. The research on the pathophysiology of syringomyelia has been focused on Chiari malformation, trauma, and inflammation. To understand the pathophysiology of syringomyelia associated with occult spinal dysraphism, a novel animal model of syringomyelia induced by chronic mechanical compression of the lumbar spinal cord was created. METHODS The model was made by epidural injection of highly concentrated paste-like kaolin solution through windows created by partial laminectomy of L-1 and L-5 vertebrae...
February 17, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28217389/management-of-parturients-in-active-labor-with-arnold-chiari-malformation-tonsillar-herniation-and-syringomyelia
#3
Ramsis F Ghaly, Tatiana Tverdohleb, Kenneth D Candido, Nebojsa Nick Knezevic
BACKGROUND: Arnold-Chiari malformation Type 1 (ACM-1) in parturients is a topic of ongoing discussion between obstetricians and anesthesiologists. The primary unanswered question remains; How should the anesthesia provider proceed with labor analgesia and anesthesia for cesarean section when confronted with an advanced, asymptomatic, or minimally symptomatic case of ACM-1 during labor? CASE DESCRIPTION: A 24-year-old, ASA II, G1P0 full-term parturient presented to Labor and Delivery for vaginal delivery...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28156254/population-based-description-of-familial-clustering-of-chiari-malformation-type-i
#4
Diana Abbott, Douglas Brockmeyer, Deborah W Neklason, Craig Teerlink, Lisa A Cannon-Albright
OBJECTIVE A population-based genealogical resource with linked medical data was used to define the observed familial clustering of Chiari malformation Type I (CM-I). METHODS All patients with CM-I were identified from the 2 largest health care providers in Utah; those patients with linked genealogical data were used to test hypotheses regarding familial clustering. Relative risks (RRs) in first-, second-, and third-degree relatives were estimated using internal cohort-specific CM-I rates; the Genealogical Index of Familiality (GIF) test was used to test for an excess of relationships between all patients with CM-I compared with the expected distribution of relationships for matched control sets randomly selected from the resource...
February 3, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28052881/headache-outcomes-in-children-undergoing-foramen-magnum-decompression-for-chiari-i-malformation
#5
Saba Raza-Knight, Kshitij Mankad, Prab Prabhakar, Dominic Thompson
OBJECTIVE: A common symptom of Chiari I malformation (CIM) is headache, which is diagnosed using non-validated criteria from the International Headache Society (IHS). CIM-associated headaches should resolve following neurosurgical treatment of the malformation by foramen magnum decompression (FMD). We aimed to validate the IHS criteria and determine (1) the efficacy of FMD in treating headache and (2) whether duraplasty confers an advantage over simple bony decompression in the treatment of this symptom...
January 4, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27921214/external-validity-of-the-chiari-severity-index-and-outcomes-among-pediatric-chiari-i-patients-treated-with-intra-or-extra-dural-decompression
#6
Jared M Pisapia, Maxwell B Merkow, Danielle Brewington, Rosemary E Henn, Leslie N Sutton, Phillip B Storm, Gregory G Heuer
INTRODUCTION: Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. METHODS: We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children's hospital...
December 5, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27857798/not-all-cases-of-nyctalopia-are-benign-unusual-and-serendipitous-presentation-of-arnold-chiari-type-1-malformation-at-a-pediatric-tertiary-care-center
#7
Kailash Chandra Patra, Abhijeet Prakash Kirtane
The Arnold-Chiari Type 1 malformation (CM1) is a rare congenital abnormality characterized by ectopia or caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical spine, resulting in crowding at the craniocervical junction. It seldom presents in childhood with symptoms and a normal neurological examination. More typically, CM1 presents in young adult women with neurological symptoms including a headache, cervical pain, cranial nerve palsies, neurosensory deficit, and ataxia. Ocular manifestations associated with Chiari I include third and sixth cranial nerve paresis and convergence/divergence abnormalities...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27798986/chiari-related-hydrocephalus-assessment-of-clinical-risk-factors-in-a-cohort-of-297-consecutive-patients
#8
Jian Guan, Jay Riva-Cambrin, Douglas L Brockmeyer
OBJECTIVE Patients treated for Chiari I malformation (CM-I) with posterior fossa decompression (PFD) may occasionally and unpredictably develop postoperative hydrocephalus. The clinical risk factors predictive of this type of Chiari-related hydrocephalus (CRH) are unknown. The authors' objective was to evaluate their experience to identify risk factors that may predict which of these patients undergoing PFD will develop CRH after surgery. METHODS The authors performed a retrospective clinical chart review of all patients who underwent PFD surgery and duraplasty for CM-I at the Primary Children's Hospital in Utah from June 1, 2005, through May 31, 2015...
November 2016: Neurosurgical Focus
https://www.readbyqxmd.com/read/27743249/the-association-between-the-pulse-pressure-gradient-at-the-cranio-cervical-junction-derived-from-phase-contrast-magnetic-resonance-imaging-and-invasively-measured-pulsatile-intracranial-pressure-in-symptomatic-patients-with-chiari-malformation-type-1
#9
Radek Frič, Erika Kristina Lindstrøm, Geir Andre Ringstad, Kent-André Mardal, Per Kristian Eide
BACKGROUND: In symptomatic Chiari malformation type 1 (CMI), impaired intracranial compliance (ICC) is associated with an increased cranio-spinal pulsatile pressure gradient. Phase-contrast magnetic resonance imaging (MRI) represents a non-invasive modality for the assessment of the pulse pressure gradient at the cranio-cervical junction (CCJ). We wished to explore how the MRI-derived pulse pressure gradient (MRI-dP) compares with invasively measured pulsatile intracranial pressure (ICP) in CMI, and with healthy controls...
October 14, 2016: Acta Neurochirurgica
https://www.readbyqxmd.com/read/27665041/hospitalization-cost-model-of-pediatric-surgical-treatment-of-chiari-type-1-malformation
#10
Sandi K Lam, Rory R Mayer, Thomas G Luerssen, I Wen Pan
OBJECTIVES: To develop a cost model for hospitalization costs of surgery among children with Chiari malformation type 1 (CM-1) and to examine risk factors for increased costs. STUDY DESIGN: Data were extracted from the US National Healthcare Cost and Utilization Project 2009 Kids' Inpatient Database. The study cohort was comprised of patients aged 0-20 years who underwent CM-1 surgery. Patient charges were converted to costs by cost-to-charge ratios. Simple and multivariable generalized linear models were used to construct cost models and to determine factors associated with increased hospital costs of CM-1 surgery...
September 21, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27651871/surgical-outcome-of-adult-idiopathic-chiari-malformation-type-1
#11
Woon Tak Yuh, Chi Heon Kim, Chun Kee Chung, Hyun-Jib Kim, Tae-Ahn Jahng, Sung Bae Park
OBJECTIVE: The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. METHODS: Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33...
September 2016: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/27611205/letter-to-the-editor-evoked-potentials-and-chiari-malformation-type-1
#12
Ori Barzilai, Jonathan Roth, Akiva Korn, Shlomi Constantini
No abstract text is available yet for this article.
February 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/27546932/mosaic-trisomy-8-detected-by-fibroblasts-cultured-of-skin
#13
Gustavo Giraldo, Ana M Gómez, Lina Mora, Fernando Suarez-Obando, Olga Moreno
INTRODUCTION: Mosaic trisomy 8 or "Warkany's Syndrome" is a chromosomopathy with an estimated prevalance of 1:25,000 to 1:50,000, whose clinical presentation has a wide phenotypic variability. CASE DESCRIPTION: Patient aged 14 years old with antecedents of global retardation of development, moderate cognitive deficit and hypothyroidism of possible congenital origin. CLINICAL FINDINGS: Physical examination revealed palpebral ptosis, small corneas and corectopia, hypoplasia of the upper maxilla and prognathism, dental crowding, high-arched palate, anomalies of the extremities such as digitalization of the thumbs, clinodactyly and bilateral shortening of the fifth finger, shortening of the right femur, columnar deviation and linear brown blotches that followed Blaschko's lines...
June 30, 2016: Colombia Médica: CM
https://www.readbyqxmd.com/read/27535630/congenital-spondylolytic-spondylolisthesis-of-c2-vertebra-associated-with-atlanto-axial-dislocation-chiari-type-i-malformation-and-anomalous-vertebral-artery-case-report-with-review-literature
#14
Jayesh Sardhara, Sindgikar Pavaman, Kuntal Das, Arun Srivastava, Anant Mehrotra, Sanjay Behari
BACKGROUND: Congenital spondylolytic spondylolisthesis of C2 vertebra resulting from deficient posterior element of the axis is rarely described in the literature. CASE DESCRIPTION: We describe a unique case of agenesis of posterior elements of C2 with craniovertebral junction anomalies consisting of osseous, vascular, and soft tissue anomalies. A 26-year-old man presented with symptoms of upper cervical myelopathy of 12 months' duration. A computed tomography scan of the cervical spine including the craniovertebral junction revealed spondylolisthesis of C2 over C3, atlantoaxial dislocation, occipitalization of the atlas, hypoplasia of the odontoid, and cleft posterior C1 arch...
November 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27500008/neurofibromatosis-type-1-and-chiari-type-1-malformation-a-case-report-and-literature-review-of-a-rare-association
#15
Marianne Pozetti, Telmo Augusto Barba Belsuzarri, Natalia C B Belsuzarri, Naira B Seixas, João F M Araujo
BACKGROUND: The association between neurofibromatosis type 1 (NF-I) and Chiari I malformation (CMI) is rare, and not many studies are reported in the literature. Performing magnetic resonance imaging (MRI) in patients with NF-1 is essential because several cases of Chiari type I are completely asymptomatic. We emphasize the need for inclusion of Chiari I as diagnosis in association with NF-1. CASE DESCRIPTION: The patient was a 51-year-old black man who presented with complaints of pain and decreased motion and sensibility of his hands, wrists, and forearms, along with progressive dysarthria...
2016: Surgical Neurology International
https://www.readbyqxmd.com/read/27422274/hemifacial-spasm-associated-with-type-1-chiari-malformation-a-retrospective-study-of-13-cases
#16
Ning Li, Wei-Guo Zhao, Chun-Hua Pu, Wen-Lei Yang
Hemifacial spasm (HFS) associated with type 1 Chiari malformation is particularly uncommon and is limited to isolated case report. The aims of this study were to report the clinical correlates of patients who had simultaneously HFS and type 1 Chiari malformation and to present the outcome of these patients treated with microvascular decompression (MVD) surgery. We retrospectively evaluated 13 patients who had simultaneously HFS and type 1 Chiari malformation among 675 HFS patients. Clinical features and radiological findings were collected from each patient and analyzed...
July 15, 2016: Neurosurgical Review
https://www.readbyqxmd.com/read/27418995/unusual-ventilatory-response-to-exercise-in-patient-with-arnold-chiari-type-1-malformation-after-posterior-fossa-decompression
#17
Keely Smith, Ana M Gomez-Rubio, Tomika S Harris, Lauren E Brooks, Ricardo A Mosquera
We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27392442/thirty-day-outcomes-for-posterior-fossa-decompression-in-children-with-chiari-type-1-malformation-from-the-us-nsqip-pediatric-database
#18
Aditya Vedantam, Rory R Mayer, Kristen A Staggers, Dominic A Harris, I-Wen Pan, Sandi K Lam
PURPOSE: The multicenter National Surgical Quality Improvement Program-Pediatric (NSQIP-P) database maintained by the American College of Surgeons was used to describe 30-day outcomes following Chiari type 1 decompression in children and to identify risk factors for readmission, reoperation, and perioperative complications. METHODS: We identified patients aged 0-18 years who underwent posterior cranial fossa decompression for Chiari type 1 malformation in 2012, 2013, and 2014 in the NSQIP-Pediatric database...
November 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27386388/the-indian-basket-trick-a-case-of-delayed-paraplegia-with-complete-recovery-caused-by-misplaced-thoracic-pedicle-screw
#19
Antonin Leroy, Reda Kabbaj, Arnaud Dubory, Manon Bachy, Anne-Isabelle Vermersch, Raphaël Vialle
INTRODUCTION: Pedicle screw fixation allows purchase of all three spinal columns without encroaching into the spinal canal improving fracture fixation, as well as deformity correction. Fortunately, neurologic injury associated with pedicle screw malposition is rare. CASE PRESENTATION: A 19-year-old boy was surgically treated for severe right thoracic scoliosis associated with a Chiari Type 1 malformation and a C6 to T7 syringomyelia. Six months after the initial surgery, the patient was referred to our institution after three weeks of gait disturbances and repeated falls...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27380569/management-of-craniosynostosis-at-an-advanced-age-controversies-clinical-findings-and-surgical-treatment
#20
Rajiv J Iyengar, Petra M Klinge, Wendy S Chen, Jerrold L Boxerman, Stephen R Sullivan, Helena O Taylor
BACKGROUND: The natural history of unrepaired craniosynostosis is not well defined. Delayed surgical intervention carries greater risk of postoperative complications and its functional benefits for older patients are poorly characterized. The authors reviewed patients in whom children presented beyond 1 year of age to better understand the natural history of craniosynostosis, and the risk-benefit relationship for delayed reconstruction. METHODS: After institutional IRB approval the authors conducted a retrospective review of patients who presented after 1 year of age with craniosynostosis...
July 2016: Journal of Craniofacial Surgery
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