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chiari malformation

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https://www.readbyqxmd.com/read/28196729/delayed-expansion-of-an-intracranial-cyst-induced-by-ventriculoperitoneal-shunt-in-a-patient-with-chiari-malformation-type-2
#1
Yoshinori Maki, Tsuyoshi Ohta, Hirokazu Onishi, Naoki Fukui, Masanori Morimoto
A male neonate was referred to our hospital for the treatment of myelomeningocele and hydrocephalus related to Chiari malformation type 2. After the placement of ventriculoperitoneal (VP) shunt for hydrocephalus, an intracranial cyst, which was asymptomatic at first, expanded and caused clinical symptoms due to increased intracranial pressure. The expansion of the cyst was confirmed dominantly in the right lateral ventricle where the VP shunt was inserted. Intraoperative findings through a neuroendoscope suggested that the expanded cyst was an isolated lesion...
February 11, 2017: Brain & Development
https://www.readbyqxmd.com/read/28192262/intra-operative-ultrasonography-for-definition-of-less-invasive-surgical-technique-in-patients-with-chiari-type-i-malformation
#2
Roger Schmidt Brock, Mario Augusto Taricco, Matheus Fernandes de Oliveira, Marcelo de Lima Oliveira, Manoel Jacobsen Teixeira, Edson Bor-Seng-Shu
INTRODUCTION: Chiari malformation Type I (CM) is the main congenital malformation disease of the craniovertebral junction. The ideal surgical treatment is still controversial. Invasive procedures inside CSF space and associated with dural repair are considered the gold standard, however a less invasive surgery with isolated bone decompression without dural opening may be possible in selected patients.. Our study evaluates the efficacy of intra-operative CSF flow measurement with ultrasonography (USG) as a determining parameter in the selection of these patients...
February 9, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28185017/intradiploic-pseudomeningocele-and-ossified-occipitocervical-pseudomeningocele-after-decompressive-surgery-for-chiari-i-malformation-report-of-two-cases-and-literature-review
#3
Arthur R Kurzbuch, Shailendra Magdum, Jayaratnam Jayamohan
Intradiploic cerebrospinal fluid (CSF) collections are rare findings. The authors describe two pediatric patients with iatrogenically induced occipital CSF collections after decompressive surgery for Chiari I malformation. The first patient presents a large occipital intradiploic pseudomeningocele and the second patient an intradiploic pseudomeningocele merging with an ossified occipitocervical pseudomeningocele. Though being rarities after decompression for Chiari I malformation, intradiploic fluid collection and ossified pseudomeningocele should be considered if patients represent with aggravating presurgical or new symptoms...
February 9, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28181025/surgical-management-of-chiari-i-malformation-based-on-different-cerebrospinal-fluid-flow-patterns-at-the-cranial-vertebral-junction
#4
Tao Fan, HaiJun Zhao, XinGang Zhao, Cong Liang, YinQian Wang, QiFei Gai
Chiari I malformation has been shown to present different cerebrospinal fluid (CSF) flow patterns at the cranial-vertebral junction (CVJ). Posterior fossa decompression is the first-line treatment for symptomatic Chiari I malformation. However, there is still controversy on the indication and selection of decompression procedures. This research aims to investigate the clinical indications, outcomes, and complications of the decompression procedures as alternative treatments for Chiari I malformation, based on the different CSF flow patterns at the cranial-vertebral junction...
February 9, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28157255/current-views-on-fetal-surgical-treatment-of-myelomeningocele-the-management-of-myelomeningocele-study-moms-trial-and-polish-clinical-experience
#5
Jacek Zamłyński, Ewa Horzelska, Mateusz Zamłyński, Katarzyna Olszak-Wąsik, Leszek Nowak, Piotr Bodzek, Tomasz Horzelski, Rafał Bablok, Anita Olejek
Myelomeningocele (MMC) is the most frequent congenital defect of the central nervous system for which there is no satisfactory alternative to postnatal treatment. On the contrary prenatal MMC surgery is conducting before birth and is aimed at protecting from Chiari II malformation. The main goal of fetal MMC repair is to improve development and life quality of children with Chiari II malformation. Management of Myelomeningocele Study (MOMS) which was published in 2011 clearly confirmed effectiveness of prenatal surgery...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28156254/population-based-description-of-familial-clustering-of-chiari-malformation-type-i
#6
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/28139319/paradoxical-vocal-fold-motion-pvfm-in-pediatric-otolaryngology
#7
Blake Smith, Claudio Milstein, Bryan Rolfes, Samantha Anne
INTRODUCTION: Paradoxical vocal fold motion (PVFM) is a condition in which the vocal cords exhibit inappropriate inspiratory adduction, and it has been poorly studied in the pediatric population. METHODS: Pediatric patients diagnosed with PVCM by a pediatric otolaryngologist and doctor of speech pathology from 2008 to 2012 were reviewed. Patients in whom another cause for their respiratory disturbance was eventually identified were excluded. Patient demographics, characteristics, treatment, and outcomes were reviewed...
January 24, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28122099/severe-neck-stiffness-in-a-child-with-an-undiagnosed-chiari-malformation
#8
Cameron E Kliner, Eric E Berg, Ron B Mitchell
No abstract text is available yet for this article.
January 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28122014/use-of-morphometric-mapping-to-characterise-symptomatic-chiari-like-malformation-secondary-syringomyelia-and-associated-brachycephaly-in-the-cavalier-king-charles-spaniel
#9
Susan P Knowler, Chloe Cross, Sandra Griffiths, Angus K McFadyen, Jelena Jovanovik, Anna Tauro, Zoha Kibar, Colin J Driver, Roberto M La Ragione, Clare Rusbridge
OBJECTIVES: To characterise the symptomatic phenotype of Chiari-like malformation (CM), secondary syringomyelia (SM) and brachycephaly in the Cavalier King Charles Spaniel using morphometric measurements on mid-sagittal Magnetic Resonance images (MRI) of the brain and craniocervical junction. METHODS: This retrospective study, based on a previous quantitative analysis in the Griffon Bruxellois (GB), used 24 measurements taken on 130 T1-weighted MRI of hindbrain and cervical region...
2017: PloS One
https://www.readbyqxmd.com/read/28121988/craniometric-analysis-of-the-hindbrain-and-craniocervical-junction-of-chihuahua-affenpinscher-and-cavalier-king-charles-spaniel-dogs-with-and-without-syringomyelia-secondary-to-chiari-like-malformation
#10
Susan P Knowler, Anna-Mariam Kiviranta, Angus K McFadyen, Tarja S Jokinen, Roberto M La Ragione, Clare Rusbridge
OBJECTIVES: To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM) in the Chihuahua, Affenpinscher and Cavalier King Charles Spaniel (CKCS). METHOD: Analysis of 273 T1-weighted mid-sagittal DICOM sequences of the hindbrain and craniocervical junction from 99 Chihuahuas, 42 Affenpinschers and 132 CKCSs. The study compared 22 morphometric features (11 lines, eight angles and three ratios) of dogs with and without SM using refined techniques based on previous studies of the Griffon Bruxellois (GB) using Discriminant Function Analysis and ANOVA with post-hoc corrections...
2017: PloS One
https://www.readbyqxmd.com/read/28121970/resorbable-mesh-cranioplasty-repair-of-bilateral-cerebrospinal-fluid-leaks-following-pediatric-simultaneous-bilateral-auditory-brainstem-implant-surgery
#11
Giacomo Colletti, Marco Mandalà, Vittorio Colletti, Alberto Deganello, Fabiana Allevi, Liliana Colletti
OBJECTIVE: To present a child with cochlear nerve deficiency (CND) who received simultaneous bilateral simultaneous auditory brainstem implants (BS-ABI) and subsequently presented with bilateral cerebrospinal fluid (CSF) leaks unresponsive to standard treatments. To propose a novel rigid retrosigmoid cranioplasty for treating and preventing CSF leaks in children at high risk for this complication. PATIENT: A 3.5-year-old child with CND, vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities, coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality, and ear abnormality, Arnold Chiari malformation, previous treated tracheo-esophageal fistula underwent BS-ABI...
January 24, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28121938/comparison-of-posterior-fossa-decompression-with-and-without-duraplasty-for-the-surgical-treatment-of-chiari-malformation-type-i-in-adult-patients-a-retrospective-analysis-of-103-patients
#12
Junchen Chen, Yongning Li, Tianyu Wang, Jun Gao, Jincheng Xu, Runlong Lai, Dianhui Tan
Chiari malformation type I (CM-I) is a congenital neurosurgical disease about the herniation of cerebellar tonsil through the foramen magnum. A variety of surgical techniques for CM-I have been used, and there is a controversy whether to use posterior fossa decompression with duraplasty (PFDD) or posterior fossa decompression without duraplasty (PFD) in CM-I patients. Here, we compared the clinical results and effectiveness of PFDD and PFD in adult patients with CM-I. The cases of 103 adult CM-I patients who underwent posterior fossa decompression with or without duraplasty from 2008 to 2014 were reviewed retrospectively...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28108854/elective-icp-monitoring-how-long-is-long-enough
#13
S D Thompson, A Coutts, C L Craven, A K Toma, L W Thorne, L D Watkins
BACKGROUND: Intracranial pressure monitoring is commonly undertaken to assess and manage acute patients following head injury. However, ICP monitoring can also be a useful diagnostic tool in the management of CSF dynamics in elective patients. To date, there is little published research to suggest how long these elective patients require ICP monitoring in order to gain an accurate picture of a patient's ICP dynamics. At the author's institution, a minimum of 48-h data collection is currently undertaken in patients with a suspected ICP abnormality...
March 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28099558/the-mortality-rate-after-hospital-discharge-in-patients-with-myelomeningocele-decreased-after-implementation-of-mandatory-flour-fortification-with-folic-acid
#14
Renato Manganelli Salomão, Tatiana Protzenko Cervante, José Francisco Manganelli Salomão, Soniza Vieira Alves Leon
Objective: To evaluate the mandatory folic acid fortification of flour on mortality rates after the hospital discharge of children born with myelomeningocele, the most affected age group and the most frequent cause of death. Methods: A retrospective study of 383 children born with myelomeningocele from January 1990 to December 2013 in a high-fetal-risk reference hospital. Results: A total of 39 patients died (10.1%),of which 23 (6%) died after discharge...
January 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28097251/management-of-progressive-late-onset-scoliosis-with-magnetic-growth-rod-insertion-leading-to-improvement-of-neural-anomalies-a-case-report
#15
Amit Zaveri, Valerio Pace, Dimpu Bhagawati, Vijay Rajamani, Thillainayagam Muthukumar, Hilali Noordeen
BACKGROUND: To present the first known reported case of late onset idiopathic scoliosis with concomitant neural anomalies, treated with sequential distraction using magnetic growth rod, had significant improvement in both cranio-cervical and intraspinal anomaly. METHODS: A caucasian, growing female child (at the age of ten) presented with moderately progressive late onset right thoracic scoliosis. She was found to have Chiari type I malformation and a cervicothoracic syrinx on routine pre-operative MRI scanning...
December 2016: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/28094426/the-role-of-cine-flow-magnetic-resonance-imaging-in-patients-with-chiari-0-malformation
#16
Kerem Mazhar Ozsoy, Kadir Oktay, Nuri Eralp Cetinalp, Yurdal Gezercan, Tahsin Erman
AIM: The aim of this study was to define the role of phase-contrast cine magnetic resonance imaging in deciding the therapeutic strategy and underlying pathophysiology resulting in syrinx formation in patients with Chiari type 0 malformation. MATERIAL AND METHODS: Seven patients admitted to our clinic with diagnosis of Chiari 0 malformations during the period January 2005 to July 2016 were enrolled in the study. All patients underwent a detailed preoperative neurological examination...
December 14, 2016: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28052881/headache-outcomes-in-children-undergoing-foramen-magnum-decompression-for-chiari-i-malformation
#17
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/28049537/imperforated-cor-triatriatum-dexter-in-a-dog-with-concurrent-caudal-vena-cava-wall-mineralization
#18
Tetyda Paulina Dobak, Gregory Starrak, Kathleen Linn, Elisabeth Christine Roberston Snead
BACKGROUND: Cor triatriatum dexter (CTD) is a rare congenital cardiac malformation with various manifestations and has been sporadically described in dogs. Clinically the dogs present with nonspecific signs of right heart failure or Budd-Chiari-like syndrome. Other associated concurrent cardiovascular anomalies are commonly reported. Diagnosis and full characterization of this complex malformation requires careful investigation and often a multimodal imaging approach. CASE PRESENTATION: A 10-week-old, male intact, Golden Retriever was presented with clinical signs of stunted growth, anorexia, and progressive ascites...
January 3, 2017: Acta Veterinaria Scandinavica
https://www.readbyqxmd.com/read/28036062/an-unusual-case-of-post-traumatic-headache-complicated-by-intracranial-hypotension
#19
Sara Siavoshi, Carrie Dougherty, Jessica Ailani, Kaustubh Yadwadkar, Frank Berkowitz
We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF) leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension...
December 29, 2016: Brain Sciences
https://www.readbyqxmd.com/read/28018608/digenic-mutations-of-human-ocrl-paralogs-in-dent-s-disease-type-2-associated-with-chiari-i-malformation
#20
Daniel Duran, Sheng Chih Jin, Tyrone DeSpenza, Carol Nelson-Williams, Andrea G Cogal, Elizabeth W Abrash, Peter C Harris, John C Lieske, Serena Je Shimshak, Shrikant Mane, Kaya Bilguvar, Michael L DiLuna, Murat Günel, Richard P Lifton, Kristopher T Kahle
OCRL1 and its paralog INPP5B encode phosphatidylinositol 5-phosphatases that localize to the primary cilium and have roles in ciliogenesis. Mutations in OCRL1 cause the X-linked Dent disease type 2 (DD2; OMIM# 300555), characterized by low-molecular weight proteinuria, hypercalciuria, and the variable presence of cataracts, glaucoma and intellectual disability without structural brain anomalies. Disease-causing mutations in INPP5B have not been described in humans. Here, we report the case of an 11-year-old boy with short stature and an above-average IQ; severe proteinuria, hypercalciuria and osteopenia resulting in a vertebral compression fracture; and Chiari I malformation with cervico-thoracic syringohydromyelia requiring suboccipital decompression...
2016: Human Genome Variation
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