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chiari malformation

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https://www.readbyqxmd.com/read/28612426/embryology-and-pathophysiology-of-the-chiari-i-and-ii-malformations-a-comprehensive-review
#1
REVIEW
Mohammadali M Shoja, Jaspreet Johal, W Jerry Oakes, R Shane Tubbs
Although the Chiari malformations are well-studied and described developmental anomalies, there remains some incongruity in regards to their underlying etiologies. A number of theories have been proposed with the purpose of accounting for the embryology and pathogenesis of the Chiari I and II malformations and their associated complications and clinical syndromes. The present review aims to review the pertinent literature for all of the main theories that have been proposed, and outline their validity and relevance to our contemporary understanding of these anomalies...
June 13, 2017: Clinical Anatomy
https://www.readbyqxmd.com/read/28610817/vascular-diseases-of-the-liver-clinical-guidelines-from-the-catalan-society-of-digestology-and-the-spanish-association-for-the-study-of-the-liver
#2
Marta Martín-Llahí, Agustín Albillos, Rafael Bañares, Annalisa Berzigotti, M Ángeles García-Criado, Joan Genescà, Virginia Hernández-Gea, Elba Llop-Herrera, Helena Masnou-Ridaura, José Mateo, Carmen A Navascués, Ángela Puente, Marta Romero-Gutiérrez, Macarena Simón-Talero, Luis Téllez, Fanny Turon, Cándido Villanueva, Roberto Zarrabeitia, Juan Carlos García-Pagán
Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy. This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas...
June 11, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28607814/chiari-i-malformation-and-syringomyelia-in-mucopolysaccharidosis-type-i-hurler-syndrome-treated-with-posterior-fossa-decompression-case-report-and-review-of-the-literature
#3
Vyacheslav Makler, Christina L Goldstein, Daniel Hoernschemeyer, Tomoko Tanaka
BACKGROUND: Hurler Syndrome is the most severe phenotype of mucopolysaccharidosis type I. With bone marrow transplant and enzyme replacement therapy, the life expectancy of a child with Hurler syndrome has been extended, predisposing them to multiple musculoskeletal issues most commonly involving the spine. CASE DESCRIPTION: This is the case report of a 6-year-old male with Hurler syndrome who was diagnosed with Chiari I malformation and cervicothoracic syringomyelia on a preoperative magnetic resonance imaging (MRI) for his thoracolumbar kyphosis...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28598915/anesthetic-and-obstetric-management-of-syringomyelia-during-labor-and-delivery-a-case-series-and-systematic-review
#4
Gráinne Patricia Garvey, Vibhangini S Wasade, Kellie E Murphy, Mrinalini Balki
BACKGROUND: Syringomyelia is a rare, slowly progressive neurological condition characterized by the presence of a syrinx within the spinal cord. Consensus regarding the safest mode of delivery and anesthetic management in patients with syringomyelia remains controversial and presents management dilemmas. This study reviews the cases of syringomyelia at our institution and provides a systematic review of the literature to guide decisions regarding labor and delivery management. METHODS: A retrospective review of cases at our hospital from 2002 to 2014 and a systematic review of the literature from 1946 to 2014 were undertaken...
June 8, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28598265/robot-assisted-endoscopic-third-ventriculostomy-institutional-experience-in-9-patients
#5
Reid Hoshide, Mark Calayag, Hal Meltzer, Michael L Levy, David Gonda
OBJECTIVE The endoscopic third ventriculostomy (ETV) is an established and effective treatment for obstructive hydrocephalus. In its most common application, surgeons plan their entry point and the endoscope trajectory for the procedure based on anatomical landmarks, then control the endoscope freehand. Recent studies report an incidence of neural injuries as high as 16.6% of all ETVs performed in North America. The authors have introduced the ROSA system to their ETV procedure to stereotactically optimize endoscope trajectories, to reduce risk of traction on neural structures by the endoscope, and to provide a stable mechanical holder of the endoscope...
June 9, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28593553/prenatal-diagnosis-of-spina-bifida-from-intracranial-translucency-to-intrauterine-surgery
#6
Waldo Sepulveda, Amy E Wong, Francisco Sepulveda, Juan L Alcalde, Juan C Devoto, Felipe Otayza
Accurate and timely prenatal diagnosis of spina bifida (SB) is a major goal of modern antenatal care. Prenatal screening for open SB should be first performed at the time of routine first-trimester ultrasound by examining the posterior fossa for obliteration or non-visualization of the fourth ventricle ("intracranial translucency") and cisterna magna. The second step of screening is the second-trimester anatomy scan, at which time the features of the Chiari type II malformation should be looked for, including ventriculomegaly, scalloping of the frontal bones ("lemon" sign), and backward and caudal displacement of the cerebellar vermis with obliteration of the cisterna magna ("banana" sign)...
June 7, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28592078/-treatment-of-single-one-stage-posterior-atlantoaxial-fixation-in-chiari-malformation
#7
L N Lu, R Zong, X B Xu, X G Yu, G Y Qiao
Objective: To explore the effects of surgical technique of single one-stage posterior C(1-2) screw rod fixation of Chiari malformation (CM) associated with occipitalization and without atlantoaxial dislocation. Methods: A total of 23 patients with CM treated between January 2014 and October 2015 in Department of Neurosurgery of Chinese People's Liberation Army General Hospital were retrospective reviewed. All of them were diagnosis with CM associated with occipitalization and without atlantoaxial dislocation, including 8 males and 15 females, aging from 11 to 57 years (mean (35...
June 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/28589373/3d-structural-complexity-analysis-of-cerebellum-in-chiari-malformation-type-i
#8
Engin Akar, Sadık Kara, Hidayet Akdemir, Adem Kırış
Chiari malformation type I (CM-I), described by a descent of the cerebellar tonsils, is assumed to be a neurological developmental disorder. The aim of the present study was to investigate morphological variance in cerebellar sub-structures, including gray matter (GM), white matter (WM), and cerebrospinal fluid (CSF), using magnetic resonance (MR) images with three-dimensional (3D) fractal dimension (FD) analysis in patients with CM-I. MRI data of 16 patients and 15 control subjects were obtained, and structural complexity analyses were performed using a box-counting FD algorithm...
June 7, 2017: Medical & Biological Engineering & Computing
https://www.readbyqxmd.com/read/28587601/cavalier-king-charles-spaniels-with-chiari-like-malformation-and-syringomyelia-have-increased-variability-of-spatio-temporal-gait-characteristics
#9
Emil Olsen, Emma Jane Suiter, Thilo Pfau, Imelda M McGonnell, Kaspar Matiasek, Anna Giejda, Holger Andreas Volk
BACKGROUND: Chiari-like malformation in the Cavalier King Charles Spaniel is a herniation of the cerebellum and brainstem into or through the foramen magnum. This condition predisposes to Syringomyelia; fluid filled syrinxes within the spinal cord. The resulting pathology in spinal cord and cerebellum create neuropathic pain and changes in gait. This study aims to quantify the changes in gait for Cavalier King Charles Spaniel with Chiari-like malformation and Syringomyelia. METHODS: We compared Cavalier King Charles Spaniel with Chiari-like malformation with (n = 9) and without (n = 8) Syringomyelia to Border Terriers (n = 8)...
June 6, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28586935/endoscopic-management-of-arnold-chiari-malformation-type-i-with-or-without-syringomyelia
#10
Shailendra Ratre, Nishtha Yadav, Yad Ram Yadav, Vijay Singh Parihar, Jitin Bajaj, Yatin Kher
Introduction Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. Endoscope-assisted decompression of the posterior fossa has been found to be safe and effective. We report our initial experience of endoscopic management of ACM-I. Material and Methods This was a prospective study of 15 symptomatic patients. Pre- and postoperative clinical status and computed tomography and magnetic resonance imaging findings were recorded...
June 6, 2017: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/28585676/evaluation-of-apparent-diffusion-coefficients-in-the-cerebellar-tonsils-and-bulbus-in-chiari-type-i-malformations-comparison-before-and-after-surgery
#11
Bekir Akgun, Sait Ozturk, Ismail Taskent, Mehmet Besir Surme, Fatih Serhat Erol, Hanefi Yildirim
AIM: To evaluate the preoperative and postoperative 6th month mean apparent diffusion coefficient (ADC) values of the cerebellar tonsils and bulbus in patients with Chiari Malformation Type I (CMI), and to compare the results with healthy controls. MATERIAL AND METHODS: We included 15 patients with CMI who underwent suboccipital decompression, upper cervical laminectomy, and duraplasty surgery, and compared them with 10 healthy individuals. Three regions of interest were placed, one each in the cerebellar tonsils and one in the bulbus...
May 7, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28583457/vertebrobasilar-insufficiency-due-to-distal-posterior-inferior-cerebellar-artery-compression-in-chiari-1-5
#12
M Burhan Janjua, Iryna Ivasyk, Jeffrey P Greenfield
Chiari malformation is characterized by radiographic evidence of herniation of cerebellar tonsils below the foramen magnum, and symptoms complex of headaches, breathing, swallowing or sleep difficulties, ataxia, restless, motor and/or sensory deficits. We report a case of a 34-year old female whose imaging indicated a Chiari 1.5 with brainstem (caudal medulla) herniation, and an expansive cervical syrinx. Her symptom complex showed signs both of cervical syringomyelia, as well as ones localizable to the medulla...
June 2, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28582306/delayed-presentation-of-isolated-sagittal-synostosis-with-raised-intracranial-pressure-and-secondary-chiari-malformation-with-cervical-syringomyelia
#13
Stratos S Sofos, Ben Robertson, Christian Duncan, Ajay Sinha
Sagittal craniosynostosis (SC) is the most common type of premature suture fusion presenting in approximately 1 in every 5000 births with a 3:1 male:female ratio. The most common indication for surgery is the improvement of the cosmetic appearance of the skull, since a cranial deformation may have a significant psychosocial impact on affected patients. Relief from raised intracranial pressure is a further indication for surgery, although an increased intracranial pressure (ICP) can be demonstrated only in a minority of affected children at diagnosis...
June 2, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28566405/intraspinal-anomalies-in-early-onset-idiopathic-scoliosis
#14
E A C Pereira, M Oxenham, K S Lam
AIMS: In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. PATIENTS AND METHODS: This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities...
June 2017: Bone & Joint Journal
https://www.readbyqxmd.com/read/28564670/current-concepts-in-the-pathogenesis-diagnosis-and-management-of-type-i-chiari-malformations
#15
Cody A Doberstein, Radmehr Torabi, Petra M Klinge
Type 1 Chiari malformations (CMs) are a group of congenital or acquired disorders which include the abnormal presence of the cerebellar tonsils in the upper spinal canal, rather than the posterior fossa. The resulting anatomic abnormality causes crowding of the structures at the craniocervical junction and can impair the normal flow of cerebral spinal fluid (CSF) in this region. This impairment in CSF flow dynamics can led to the development of syringomyelia or hydrocephalus. Type 1 CMs have been associated with a wide array of symptoms resulting from either cerebellar and brainstem compression and distortion or disturbances in CSF dynamics, and can affect both children and adults...
June 1, 2017: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/28559064/endoscopic-third-ventriculostomy-for-the-treatment-of-hydrocephalus-in-a-paediatric-population-with-myelomeningocele
#16
Joana Rei, Josué Pereira, Carina Reis, Sérgio Salvador, Rui Vaz
BACKGROUND: Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although Endoscopic Third Ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiological outcomes of ETV in children with hydrocephalus related to myelomeningocele. METHODS: Medical records of 18 paediatric patients with myelomeningocele submitted to ETV from 1998 to 2015, at the Centro Hospitalar S...
May 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28553378/dermoid-of-the-posterior-fossa-in-chiari-ii-malformation-the-first-reported-case
#17
R Shane Tubbs, Ketan Verma, Sarah N Mirahsani, Martin M Mortazavi, Randle A Umeh, Brandon Rocque, Jeffrey B Blount
Dermoid cysts are rare lesions, particularly in children. Chiari II malformations are seen in patients with myelomeningocele. Here, we present a child with Chiari II malformation who, during a Chiari II decompression, was found to have a dermoid cyst. To the best of our knowledge, this is the first such case ever reported.
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28540253/craniocervical-junction-abnormalities-with-atlantoaxial-subluxation-caused-by-ventral-subluxation-of-c2-in-a-dog
#18
Harumichi Itoh, Kazuhito Itamoto, Shotaro Eto, Tomoya Haraguchi, Shimpei Nishikawa, Kenji Tani, Yoshiki Itoh, Masato Hiyama, Toshie Iseri, Munekazu Nakaichi, Yasuho Taura
Craniocervical junction abnormalities with atlantoaxial subluxation caused by ventral subluxation of C2 were diagnosed in a 6-month-old female Pomeranian with tetraplegia as a clinical sign. Lateral survey radiography of the neck with flexion revealed atlantoaxial subluxation with ventral subluxation of C2. Computed tomography revealed absence of dens and atlanto-occipital overlapping. Magnetic resonance imaging showed compression of the spinal cord and indentation of caudal cerebellum. The diagnosis was Chiari-like malformation, atlantoaxial subluxation with ventral displacement of C2, atlanto-occipital overlapping, and syringomyelia...
2017: Open veterinary journal
https://www.readbyqxmd.com/read/28537458/chiari-i-malformation-in-children-with-transverse-myelitis
#19
Sathya Vadivelu, Sudhakar Vadivelu, Maureen Mealy, Smurti Patel, Libby Kosnik-Infinger, Daniel Becker
PURPOSE: Transverse myelitis (TM) is an acute inflammatory spinal cord injury. Asymptomatic Chiari I malformation (CMI) management is highly controversial, particularly when associated with a spinal syrinx. Here, we assess the occurrence of CMI in the pediatric TM population and management outcomes. METHODS: We performed a retrospective cohort study based on 61 consecutively identified pediatric TM cases over an -8-year period. We reviewed demographic characteristics, radiographic findings, presenting symptoms, and long-term outcomes...
May 24, 2017: Developmental Neurorehabilitation
https://www.readbyqxmd.com/read/28537352/is-pseudotumor-cerebri-an-unusual-expression-of-chiari-syndrome-a-case-report-and-review-of-the-literature
#20
Paolo Pacca, Roberto Altieri, Francesco Zenga, Diego Garbossa, Alessandro Ducati, Michele Lanotte
The Chiari I malformation (CM-I) is a developmental alteration of the posterior cranial fossa (PCF), radiographically defined as the descent of the cerebellar tonsils = 5 mm below the foramen magnum (FM) inside the cervical canal. Headache is the most frequent symptom associated with CM-I. The association of CM-I and neurological symptoms configures with Chiari syndrome. A rare symptom associated with Chiari syndrome is intracranial hypertension syndrome with cephalea and papilloedema-the typical findings of pseudotumor cerebri (PTC)...
May 24, 2017: Surgical Technology International
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