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chiari malformation

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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
#1
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/27911244/neurosurgical-management-in-lateral-meningocele-syndrome-case-report
#2
Erik C Brown, Kunal Gupta, Christina Sayama
Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder. It is associated with morphological changes similar to those of other connective tissue disorders, with the unique distinction of multiple, often bilateral and large, lateral meningoceles herniating through the spinal foramina. In some cases, these lateral meningoceles can cause pain and discomfort due to their presence within retroperitoneal tissues or cause direct compression of the spinal nerve root exiting the foramen; in some cases compression may also involve motor weakness...
December 2, 2016: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27894943/acquired-chiari-malformation-and-syringomyelia-secondary-to-space-occupying-lesions-a-systematic-review
#3
REVIEW
Justin Wang, Naif M Alotaibi, Nardin Samuel, George M Ibrahim, Aria Fallah, Michael D Cusimano
BACKGROUND: Acquired Chiari malformations (ACM) and associated syringomyelia secondary to space-occupying lesions can cause neurological deficits independent of or in combination with the offending mass. Although type I CM are traditionally treated with posterior fossa decompression, optimal surgical management of ACM and associated syringomyelia remains unclear. The purpose of this study is to review the current literature surrounding the management of ACM. METHODS: A systematic review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines...
November 25, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27890751/atlantoaxial-fixation-for-basilar-invagination-without-obvious-atlantoaxial-instability-group-b-basilar-invagination-outcome-analysis-of-63-surgically-treated-cases
#4
Atul Goel, Prashant Sathe, Abhidha Shah
BACKGROUND: The authors discuss the rationale of surgical treatment of Group B basilar invagination by atlantoaxial facet joint stabilization and segmental arthrodesis. MATERIAL: During the period January 2010 to April 2016, 63 patients having Group B basilar invagination were surgically treated. All patients had varying degree of myelopathy related functional disability. Fifty-two patients had both Chiari malformation and syringomyelia. All patients were treated by atlantoaxial plate and screw fixation with the techniques described by us in 1994 and 2004...
November 24, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27863152/direct-numerical-simulation-of-transitional-hydrodynamics-of-the-cerebrospinal-fluid-in-chiari-i-malformation-the-role-of-cranio-vertebral-junction
#5
Kartik Jain, Geir Ringstad, Per-Kristian Eide, Kent-André Mardal
Obstruction to the cerebrospinal fluid (CSF) outflow caused by the herniation of cerebellar tonsils as a result of Chiari malformation type I leads to altered CSF hydrodynamics. This contribution explores the minutest characteristics of the CSF hydrodynamics in cervical subarachnoid spaces (SAS) of a healthy subject and two Chiari patients by performing highly resolved direct numerical simulation. The Lattice Boltzmann method is used for the simulations due to its scalability on modern supercomputers that allow us to simulate up to  ∼ 10(9) cells while resolving the Kolmogorov microscales...
November 10, 2016: International Journal for Numerical Methods in Biomedical Engineering
https://www.readbyqxmd.com/read/27858679/entire-syringomyelia-associated-with-chiari-ii-malformation-and-severe-scoliosis-and-hydrocephalus
#6
Jie Liang, Weifei Wu, Neng Ru, Jianfeng Chen
BACKGROUND: The syrinx can occur in any region of the spinal cord and is common in cervical and/or thoracic region, and distributing along spinal cord is unusual, especially association with Chiari II malformation and scoliosis. OBJECTIVE: To report a first case of entire syringomyelia associated with Chiari II malformation and severe scoliosis and hydrocephalus. CASE PRESENTATION: The patient began to experience symptoms of bilateral hand weakness in adulthood...
November 18, 2016: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/27858370/intracranial-hypertension-in-cystinosis-is-a-challenge-experience-in-a-children-s-hospital
#7
Nieves Martín-Begué, Silvia Alarcón, Charlotte Wolley-Dod, Luis Enrique Lara, Álvaro Madrid, Paola Cano, Mireia Del Toro, Gema Ariceta
BACKGROUND: Cystinosis is a rare systemic lysosomal disease affecting mainly the kidney and eye. Ocular involvement in cystinosis is universal being the presence of cystine crystals in the cornea a diagnostic criterion and one of the earliest manifestations of the disease. Neuro-ophthalmologic manifestations are considered a rare and late complication in these patients. The aim of this article is to report the unexpectedly high incidence of intracranial hypertension in children with cystinosis at our centre...
November 18, 2016: JIMD Reports
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
#8
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/27850449/811-effectiveness-of-bupivacaine-to-manage-postoperative-pain-in-chiari-malformation-correction-surgery
#9
Swetha Madhavarapu, Zachary Goldstein, Stephene Morena, Shaun Rodgers, Steven Schneider, Mark Mittler, Sandeep Gangadharan
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27834622/trends-in-surgical-treatment-of-chiari-malformation-type-i-in-the-united-states
#10
D Andrew Wilkinson, Kyle Johnson, Hugh J L Garton, Karin M Muraszko, Cormac O Maher
OBJECTIVE The goal of this analysis was to define temporal and geographic trends in the surgical treatment of Chiari malformation Type I (CM-I) in a large, privately insured health care network. METHODS The authors examined de-identified insurance claims data from a large, privately insured health care network of over 58 million beneficiaries throughout the United States for the period between 2001 and 2014 for all patients undergoing surgical treatment of CM-I. Using a combination of International Classification of Diseases (ICD) diagnosis codes and Current Procedural Terminology (CPT) codes, the authors identified CM-I and associated diagnoses and procedures over a 14-year period, highlighting temporal and geographic trends in the performance of CM-I decompression (CMD) surgery as well as commonly associated procedures...
November 11, 2016: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27826646/diagnostic-value-of-neuro-ophthalmological-signs-in-cases-of-chiari-i-malformation
#11
Inessa Bekerman, Tal Sigal, Itzhak Kimiagar, Zina Evy Almer, Michael Vaiman
INTRODUCTION: Our purpose was to evaluate the diagnostic value of measuring diameters of optic nerve sheath (ONSD), presence/absence of papilledema, tortuosity of the optic nerve, flattening of the posterior sclera, and intraocular protrusion of the prelaminar optic nerve for intracranial pressure assessment in cases of Chiari I malformation. METHODS: In a retrospective study, MRI data of 37 consecutive pediatric patients with Chiari malformation and data of 400 patients without intracranial pathology were compared and analyzed...
December 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27823711/central-sleep-apnea-in-children-experience-at-a-single-center
#12
Orlane Felix, Alessandro Amaddeo, Jorge Olmo Arroyo, Michel Zerah, Stephanie Puget, Valerie Cormier-Daire, Genevieve Baujat, Graziella Pinto, Marta Fernandez-Bolanos, Brigitte Fauroux
OBJECTIVE: Central sleep apnea (CSA) syndromes are rare in children and data in children over one year of age are scarce. The aim of the study was to describe the sleep characteristics, underlying disorders, management, and outcome of children with CSA. PATIENTS/METHODS: A retrospective chart review of all children >1 year of age, diagnosed with CSA on a laboratory sleep study during a 20-month period, was performed. CSA was defined by a central apnea index (CAI) >5 events/h...
September 2016: Sleep Medicine
https://www.readbyqxmd.com/read/27819403/-treatment-of-syringomyelia-in-patients-with-chiari-malformation-and-craniosynostosis-a-case-report-and-review-of-the-literature
#13
A Aransay-Garcia, F J Villarejo-Ortega
INTRODUCTION: Patients with multisutural or single craniosynostosis, often suffer from Chiari malformation and syringomyelia. The surgical management of syringomyelia in these patients is controversial. CASE REPORT: A 3-year-old girl was referred with complex craniosynostosis that had not been corrected surgically. She was asymptomatic despite the cranial MRI showed a Chiari malformation and one year later she developed a cervico-dorso-lumbar syringomyelia. She underwent a decompressive suboccipital craniectomy but subsequently suffered a worsening of syringomyelia...
November 16, 2016: Revista de Neurologia
https://www.readbyqxmd.com/read/27816775/adding-expansile-duraplasty-to-posterior-fossa-decompression-may-restore-cervical-range-of-motion-in-grade-3-chiari-i-malformation-patients
#14
Adem Yilmaz, Kamran Urgun, Salah G Aoun, Ibrahim Colak, Ilhan Yilmaz, Kadir Altas, Murat Musluman
No abstract text is available yet for this article.
November 2, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27815143/chiari-i-malformation-as-part-of-the-floating-harbor-syndrome
#15
Arthur R Kurzbuch, Shailendra Magdum
We report the first case of a patient diagnosed with Floating-Harbor syndrome (FHS) and Chiari I malformation. The 3-year-old girl was of proportional short stature, had delay of language development, conductive hearing loss and a high threshold of pain. Diagnosis of Chiari I malformation may be difficult in FHS patients who present with communication problems. Clinicians following patients with FHS should be aware of a possible relation between FHS and Chiari I malformation.
December 2016: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/27806370/historical-perspective-of-split-cord-malformations-a-tale-of-two-cords
#16
Erfanul Saker, Marios Loukas, Christian Fisahn, Rod J Oskouian, R Shane Tubbs
INTRODUCTION: Our appreciation and understanding of what is now known as the split cord malformation (SCM) have a long history. The oldest known example of SCM is from roughly AD 100. Other isolated examples can be found in the large body of work of the pathologists of the 1800s, where the SCMs were found incidentally during autopsies. CONCLUSIONS: SCM has a rich history and has intrigued physicians for over 200 years. Many well-known figures from the past such as Chiari and von Recklinghausen, both pathologists, made early postmortem descriptions of SCM...
2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27798986/chiari-related-hydrocephalus-assessment-of-clinical-risk-factors-in-a-cohort-of-297-consecutive-patients
#17
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/27790480/bilateral-vocal-cord-palsy-with-arnold-chiari-malformation-a-rare-case-series
#18
Nikhil Arora, Ruchika Juneja, Ravi Meher, Eishaan K Bhargava
Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27777155/shunting-of-syringomyelic-cavities-by-using-a-myringotomy-tube-technical-note-and-long-term-results
#19
Dorota Tassigny, Basel Abu-Serieh, Daniella Tsague Fofe, Jacques Born, Germain Milbouw
BACKGROUND AND IMPORTANCE: Syringomyelia is a progressive cerebrospinal fluid disorder which can lead to irreversible spinal cord injury. To date, the optimal management of syringomyelic cavities remains controversial. Multiple studies have emphasized the importance of the cranio-cervical decompression or shunting procedures. However, excepted for syrinx related to Arnold Chiari malformation, nearly half of patients need to be re-operated. The purpose of the present study was to describe a simple and efficient surgical technique and to report the long-term radio-clinical outcomes...
October 21, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27766742/intracranial-translucency-assessment-at-first-trimester-nuchal-translucency-ultrasound
#20
Annah Lane, Ling Lee, Donna Traves, Andreas Lee
INTRODUCTION: The antenatal diagnosis of open spina bifida (OSB), a neural tube defect, is predominantly made at the second trimester morphology scan by ultrasound detection of structural abnormalities resulting from the associated Chiari II malformation. Evidence has emerged suggesting that these structural abnormalities can be detected earlier, by examination of the posterior fossa as part of the first trimester nuchal translucency scan. In particular, absence of the intra-cranial translucency (IT) of the fourth ventricle has shown promise as a diagnostic marker of OSB, although the sensitivity and specificity of this finding varies widely in the literature...
October 21, 2016: Journal of Medical Imaging and Radiation Oncology
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