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chiari malformation

Thomas Beez, Jennifer Brown
BACKGROUND: Atlanto-occipital dislocation (AOD) is a rare but severe sequela of high energy trauma. Children are at increased risk, due to their immature spine and biomechanical characteristics. LITERATURE REVIEW: The prevailing mechanism of injury is motor vehicle collision as passenger. AOD commonly presents with cardiorespiratory arrest, spinal cord injury or cranial nerve deficits. Concomitant injuries, such as traumatic brain injury, are frequently encountered...
October 18, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Sivashanmugam Dhandapani, Madhivanan Karthigeyan, Sushanta K Sahoo, Vivek Gupta
BACKGROUND: Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. CASE DESCRIPTION: We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma...
October 18, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Omar N Pathmanaban, Kerry A Burke, Paul Leach, John Thorne, Ian D Kamaly-Asl
BACKGROUND: Positional plagiocephaly is the most common cause of cranial asymmetry. The underlying cause of Chiari-1 malformation has many possible theories, and anecdotally some pediatric neurosurgeons have had experience of severe cases of positional brachycephaly with Chiari-1. However, to date there have been no published cases linking non-synostotic plagiocephaly with Chiari-1 malformation. CASE DESCRIPTION: An 18-month-old boy presented with a head injury...
October 15, 2016: World Neurosurgery
Max O Krucoff, Steven Cook, Owoicho Adogwa, Jessica Moreno, Siyun Yang, Jichun Xie, Alexander O Firempong, Nandan Lad, Carlos A Bagley
OBJECT: To examine the role of race, gender, and socioeconomics on presentations and outcomes of adult Chiari 1 malformations. METHODS: The charts of 638 adult patients with Chiari 1 malformations were reviewed, and 287 patients were included. Race, gender, insurance status, symptoms, depth of cerebellar tonsillar herniation, and presence of syringomyelia were examined as covariates in multivariate logistic regression models to identify independent predictors of presentation and outcome...
October 14, 2016: World Neurosurgery
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
T Struffert
Many important structures are located in the confined space within the posterior cranial fossa. This article describes the main aspects of the anatomy. As a uniform classification of malformations of the posterior cranial fossa does not exist the main syndromes, such as Chiari malformations, zerebellar hypoplasia and dysplasia are discussed separately.
October 13, 2016: Der Radiologe
Daniele Starnoni, Roy Thomas Daniel, Mercy George, Mahmoud Messerer
BACKGROUND: Spontaneous meningoencephaloceles of the lateral sphenoid sinus are rare entities and their peculiar location represent a surgical challenge due to the importance of a wide exposure and skull base reconstruction. They are thought to arise from congenital base defect of the lateral sphenoid or in some cases have been postulated to represent a rare manifestation of altered CSF dynamics. We report the first case in literature of a Chiari malformation type I and a lateral sphenoid encephaloceles revising the theoretical etiology and surgical technique of endoscopic repair...
October 8, 2016: World Neurosurgery
Karen-Helene Støverud, Hans Petter Langtangen, Geir Andre Ringstad, Per Kristian Eide, Kent-Andre Mardal
PURPOSE: Previous computational fluid dynamics (CFD) studies have demonstrated that the Chiari malformation is associated with abnormal cerebrospinal fluid (CSF) flow in the cervical part of the subarachnoid space (SAS), but the flow in the SAS of the posterior cranial fossa has received little attention. This study extends previous modelling efforts by including the cerebellomedullary cistern, pontine cistern, and 4th ventricle in addition to the cervical subarachnoid space. METHODS: The study included one healthy control, Con1, and two patients with Chiari I malformation, P1 and P2...
2016: PloS One
Edith V Sullivan, Barton Lane, Dongjin Kwon, M J Meloy, Susan F Tapert, Sandra A Brown, Ian M Colrain, Fiona C Baker, Michael D De Bellis, Duncan B Clark, Bonnie J Nagel, Kilian M Pohl, Adolf Pfefferbaum
Structural MRI of volunteers deemed "normal" following clinical interview provides a window into normal brain developmental morphology but also reveals unexpected dysmorphology, commonly known as "incidental findings." Although unanticipated, these anatomical findings raise questions regarding possible treatment that could even ultimately require neurosurgical intervention, which itself carries significant risk but may not be indicated if the anomaly is nonprogressive or of no functional consequence. Neuroradiological readings of 833 structural MRI from the National Consortium on Alcohol and NeuroDevelopment in Adolescence (NCANDA) cohort found an 11...
October 8, 2016: Brain Imaging and Behavior
Paria Kashani, Madan Roy, Linda Gillis, Olufemi Ajani, M Constantine Samaan
A 19-month-old boy was referred for progressive weight gain. His past medical history included congenital hypothyroidism and developmental delay. Physical examination revealed characteristics of Albright Hereditary Osteodystrophy, macrocephaly, and calcinosis cutis. He had hypocalcemia, hyperphosphatemia, and elevated Parathyroid Hormone levels. Genetic testing revealed a known mutation of GNAS gene, confirming the diagnosis of Pseudohypoparathyroidism Type Ia (PHP-Ia) (c.34C>T (p.G1n12X)). He had a normal brain MRI at three months, but developmental delay prompted a repeat MRI that revealed Chiari Malformation Type I (CM-I) with hydrocephalus requiring neurosurgical intervention...
2016: Case Reports in Medicine
Christian Fisahn, Mohammadali M Shoja, Mehmet Turgut, Rod J Oskouian, W Jerry Oakes, R Shane Tubbs
INTRODUCTION: In 1894, Giuseppe Muscatello described what we believe to be the only case of an occipitocervical encephalocele with a communication to the stomach. This case and its history and context compared to the Chiari 3 malformation as described 3 years earlier by Hans Chiari are presented. CONCLUSIONS: Based on the uniqueness of this case, we propose the term Chiari 3.5 malformation be used to describe its anatomical derailment.
September 27, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Zachary C Fausel, Dale C Strasser
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
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
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
Venkata R C Vemula, Chandramouliswara P Bodapati, Jayachandar Vuttarkar, Bala B Vosuri
No abstract text is available yet for this article.
September 2016: Neurology India
Jennifer Strahle, Cormac O Maher
No abstract text is available yet for this article.
September 9, 2016: Journal of Neurosurgery
Ori Barzilai, Jonathan Roth, Akiva Korn, Shlomi Constantini
No abstract text is available yet for this article.
September 9, 2016: Journal of Neurosurgery
Mehmet Selcuki, Mesut Mete, Deniz Selcuki
AIM: The Chiari Malformation I (CM I) and tethered cord syndrome (TCS) are both congenital abnormalities whose mechanism are still not fully understood. Associaton of CM I and TCS have been reported only for a few times previously Material and Methods: This retrospective study includes 7 patients who were diagnosed with CM I and TCS managed by cutting of the filum terminale. RESULTS: The mean follow-up period was 21 months and 28 days. Although all patients were undergone untethering surgical procedure by cutting the filum terminale only, all patients reported significant early postsurgical resolution of CM I symptoms and symptoms related to TCS as well...
July 1, 2016: Turkish Neurosurgery
Charles E Mackel, Patrick J Cahill, Marie Roguski, Amer F Samdani, Patrick A Sugrue, Noriaki Kawakami, Peter F Sturm, Joshua M Pahys, Randal R Betz, Ron El-Hawary, Steven W Hwang
OBJECTIVE The authors performed a study to identify clinical characteristics of pediatric patients diagnosed with Chiari I malformation and scoliosis associated with a need for spinal fusion after posterior fossa decompression when managing the scoliotic curve. METHODS The authors conducted a multicenter retrospective review of 44 patients, aged 18 years or younger, diagnosed with Chiari I malformation and scoliosis who underwent posterior fossa decompression from 2000 to 2010. The outcome of interest was the need for spinal fusion after decompression...
September 2, 2016: Journal of Neurosurgery. Pediatrics
Davis G Taylor, Panagiotis Mastorakos, John A Jane, Edward H Oldfield
OBJECTIVE A subset of patients with Chiari I malformation demonstrate patent subarachnoid spaces around the cerebellum, indicating that reduced posterior fossa volume alone does not account for tonsillar descent. The authors distinguish two subsets of Chiari I malformation patients based on the degree of "posterior fossa crowdedness" on MRI. METHODS Two of the coauthors independently reviewed the preoperative MR images of 49 patients with Chiari I malformation and categorized the posterior fossa as "spacious" or "crowded...
September 2, 2016: Journal of Neurosurgery
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