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https://www.readbyqxmd.com/read/28922103/budd-chiari-syndrome
#1
Tomáš Grus, Lukáš Lambert, Gabriela Grusová, Rohan Banerjee, Andrea Burgetová
Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28885133/the-addition-of-duraplasty-to-posterior-fossa-decompression-in-the-surgical-treatment-of-pediatric-chiari-malformation-type-i-a-systematic-review-and-meta-analysis-of-surgical-and-performance-outcomes
#2
Victor M Lu, Kevin Phan, Sean P Crowley, David J Daniels
OBJECTIVE Surgery is the definitive treatment of Chiari malformation Type I (CM-I). It involves posterior fossa decompression, which can be performed along with C-1 laminectomy, reconstructive duraplasty, or tonsil shrinkage. The aim of this study was to provide an updated systematic review and meta-analysis of the latest available evidence regarding posterior fossa decompression only (PFDO) versus posterior fossa decompression with duraplasty (PFDD) in the treatment of CM-I in children. METHODS A literature search was performed in compliance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for article identification, screening, eligibility, and inclusion...
September 8, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28870052/-clinical-efficacy-of-enhanced-recovery-after-surgery-in-atrial-caval-shunting-for-type-%C3%A2-budd-chiari-syndrome
#3
G J Tian, D Y Li, H B Yu, Y D Dong, Y N Peng, P Liu, Y K Wei, H Z Xue
Objective: To investigate the clinical efficacy of enhanced recovery after surgery(ERAS) in atrial caval shunting (ACS) for type Ⅱ Budd-Chiari syndrome(BCS). Methods: The clinical data of patients underwent ACS for type Ⅱ BCS in the Henan Province People's Hospital from January 2014 to June 2016 were prospectively analyzed.Randomized and single-blind, controlled study was performed among the patients, and all of them underwent ACS and were divided into control group (patients underwent traditional perioperative management) and ERAS group (patients underwent ERAS perioperative management) based on a random number table...
September 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/28867320/complete-reversibility-of-the-chiari-type-ii-malformation-following-post-natal-repair-of-myelomeningocele
#4
P A Beuriat, A Szathmari, C Rousselle, I Sabatier, F di Rocco, C Mottolese
INTRODUCTION: It was believed that Chiari type II malformation (CM-II) was always present in a myelomeningocele (MMC). In fact, it is associated in about 80 % of cases. Improvement of the hindbrain herniation after prenatal closure of MMC has challenged the idea that this condition was irreversible. Only two studies report ascent of the cerebellar tonsil after post-natal closure. This work aimed to study a large group of MMC patients who benefitted from a post-natal repair to evaluate the rate of long-term total reversibility of CM-II...
August 31, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28863007/intraoperative-neurophysiologic-monitoring-for-prediction-of-postoperative-neurological-improvement-in-a-child-with-chiari-type-i-malformation
#5
Yukari Kawasaki, Susumu Uchida, Kouhei Onishi, Masako Toyokuni, Kazuo Okanari, Minoru Fujiki
INTRODUCTION: Although many surgical treatment strategies for Chiari malformation type I (CM-I) have been reported, the most appropriate surgical technique remains controversial. It is wholly ascribable to the complicacy of pathological condition in CM-I. Recently, intraoperative neurophysiologic monitoring (INM) is becoming prevalent in spinal surgery. Indeed, motor-evoked potentials (MEPs) monitoring and somatosensory-evoked potentials (SSEPs) monitoring are standard tools to minimize the risk of neurologic injury and postoperative deficits...
August 31, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28838875/comparison-of-porcine-and-bovine-collagen-dural-substitutes-in-posterior-fossa-decompression-for-chiari-i-malformation-in-adults
#6
Christine K Lee, Tara Mokhtari, Ian D Connolly, Gordon Li, Lawrence M Shuer, Steven D Chang, Gary K Steinberg, Melanie Hayden Gephart
BACKGROUND: Posterior fossa decompression surgeries for Chiari malformations are susceptible to post-operative complications such as pseudomeningocele, external cerebrospinal fluid (CSF) leak, and meningitis. Various dural substitutes have been employed to improve surgical outcomes. OBJECTIVE: This study examined whether the collagen matrix dural substitute type correlated with the incidence of post-operative complications following posterior fossa decompression in adult patients with Chiari I malformations...
August 21, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28838449/successful-blood-transfusion-management-of-a-living-donor-liver-transplant-recipient-in-the-presence-of-anti-jr-a-a-case-report
#7
N Kurata, Y Onishi, H Kamei, T Hori, M Komagome, C Kato, T Matsushita, Y Ogura
A 48-year-old Japanese woman was diagnosed with Budd-Chiari syndrome and transferred for possible living donor liver transplantation (LDLT). Examinations before LDLT revealed that the recipient had anti-Jr(a) and preformed donor-specific anti-human leukocyte antigen (HLA) antibodies (DSA). Rituximab was administrated at 16 days prior to the patient's scheduled LDLT for the prophylaxis of antibody-mediated rejection by DSA. The clinical significance of anti-Jr(a) has not been clearly established because of the rarity of this antibody, so we discussed blood transfusion strategy with the Department of Blood Transfusion Service and prepared for Jr(a)-negative packed red blood cells (RBCs)...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28838117/technique-for-mini-open-decompression-of-chiari-type-i-malformation-in-adults
#8
Peyman Pakzaban
BACKGROUND: The technique for decompression of Chiari type I malformation relies on open exposure of craniocervical junction for suboccipital craniectomy and upper cervical laminectomy with or without duraplasty. There is no detailed technical report of a minimally invasive approach for Chiari decompression in adults. OBJECTIVE: To describe a mini-open technique for decompression of Chiari type I malformation (including duraplasty) in adults. METHODS: Six consecutive adult patients with symptomatic Chiari type I malformation underwent decompression through a 3 to 4 cm midline incision via a speculum retractor...
August 1, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/28738751/a-single-center-retrospective-study-clinical-features-of-different-types-of-budd-chiari-syndrome-in-chinese-patients-in-the-hubei-area
#9
Gangping Li, Ying Huang, Shunyu Tang, Yuhu Song, Huimin Liang, Dehan Liu, Ling Yang, Xiaohua Hou
Background The characteristics and prevalence of Budd-Chiari syndrome in China remain unclear. This study aimed to analyze the clinical features of Budd-Chiari syndrome in Chinese patients in the Hubei area. Methods One-hundred and thirty patients with Budd-Chiari syndrome, admitted to Union Hospital from January 2002 to January 2011, were included in this retrospective study. Clinical features, laboratory data, imaging characteristics, and cumulative patency rates were analyzed. Results Of the 130 patients with Budd-Chiari syndrome, 77 were men (59...
January 1, 2017: Vascular
https://www.readbyqxmd.com/read/28734813/incidence-and-types-of-pediatric-nystagmus
#10
David L Nash, Nancy N Diehl, Brian G Mohney
PURPOSE: To report the incidence, prevalent subtypes and clinical characteristics of pediatric nystagmus diagnosed over a 30-year period. DESIGN: Retrospective, population-based study METHODS: Using the Rochester Epidemiology Project, the medical records of all children (<19 years) diagnosed as residents in Olmsted County, Minnesota with any form of nystagmus from January 1, 1976, through December 31, 2005, were reviewed. RESULTS: Seventy-one children were diagnosed during the 30-year period, yielding an annual incidence of 6...
July 19, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28732419/evolution-of-different-surgical-treatment-techniques-for-management-and-improving-outcome-of-chiari-malformation-type-1
#11
LETTER
Guru Dutta Satyarthee
No abstract text is available yet for this article.
August 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28693072/-comparison-of-posterior-fossa-decompression-with-and-without-duraplasty-for-surgical-management-for-adult-chiari-malformation-type-%C3%A2
#12
H Y Li, Y M Li, H Chen, Y Li, X W Shi
Objective: To evaluate and compare the efficacy between posterior fossa decompression without duraplasty (PFD) and posterior fossa decompression with duraplasty (PFDD) in the surgical management for adult Chiari Ⅰ malformation. Methods: Fifty-seven patients suffered from Chiari malformation type Ⅰ were treated in Department of Neurosurgery, Henan Provincial People's Hospital from August 2008 to October 2013. Twenty-three patients received posterior fossa decompression without duraplasty and the other 34 patients received posterior fossa decompression with duraplasty...
July 4, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28690584/decompression-in-chiari-malformation-clinical-ocular-motor-cerebellar-and-vestibular-outcome
#13
Nicolina Goldschagg, Katharina Feil, Franziska Ihl, Siegbert Krafczyk, Mathias Kunz, Jörg Christian Tonn, Michael Strupp, Aurelia Peraud
BACKGROUND: Treatment of Chiari malformation can include suboccipital decompression with resection of one cerebellar tonsil. Its effects on ocular motor and cerebellar function have not yet been systematically examined. OBJECTIVE: To investigate whether decompression, including resection of one cerebellar tonsil, leads to ocular motor, vestibular, or cerebellar deficits. PATIENTS AND METHODS: Ten patients with Chiari malformation type 1 were systematically examined before and after (1 week and 3 months) suboccipital decompression with unilateral tonsillectomy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28686331/neural-tube-defects-in-waardenburg-syndrome-a-case-report-and-review-of-the-literature
#14
Joseph Hart, Kalpana Miriyala
Waardenburg syndrome type 1 (WS1) is an autosomal dominant genetic condition characterized by sensorineural deafness and pigment abnormalities, and is caused by variants in the PAX3 homeodomain. PAX3 variants have been associated with severe neural tube defects in mice and humans, but the frequency and clinical manifestations of this symptom remain largely unexplored in humans. Consequently, the role of PAX3 in human neural tube formation remains a study of interest, for clinical as well as research purposes...
July 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28665389/-syringosubarachnoid-shunting-in-treatment-of-syringomyelia-a-literature-review-and-a-clinical-case-report
#15
V S Klimov, Yu S Gulay, A V Evsyukov, G I Moysak
In the article, we describe a clinical case of syringomyelia associated with an Arnold-Chiari type 1 malformation, evaluate the efficacy of syringosubarachnoid shunting, and analyze the literature data of domestic and international researchers involved in investigation and treatment of the pathology. Application of syringosubarachnoid shunting in the described case resulted in a clinical improvement in the form of regression of paresis and hypoesthesia, which demonstrated the efficacy of the shunting technique for correction of the syringomyelia symptoms...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28647656/delayed-retroclival-and-cervical-spinal-subdural-hematoma-complicated-by-preexisting-chiari-malformation-in-adult-trauma-patient
#16
Ha Son Nguyen, Hoon Choi, Shekar Kurpad, Hesham Soliman
INTRODUCTION: Traumatic spinal subdural hematoma involving the retroclival region and upper cervical spine is a rare pathology. To our knowledge, there have only been 2 prior cases in an adult trauma patient. We describe a patient with preexisting Chiari 1 malformation, who recently sustained a unilateral type 1 occipital condyle fracture with associated disruption of the tectorial membrane and transverse ligament, which returned with a retroclival subdural hematoma extending down to C7, causing spinal cord compression and symptomatic obstructive hydrocephalus...
September 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28592078/-treatment-of-single-one-stage-posterior-atlantoaxial-fixation-in-chiari-malformation
#17
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/28586935/endoscopic-management-of-arnold-chiari-malformation-type-i-with-or-without-syringomyelia
#18
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/28582306/delayed-presentation-of-isolated-sagittal-synostosis-with-raised-intracranial-pressure-and-secondary-chiari-malformation-with-cervical-syringomyelia
#19
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
#20
REVIEW
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
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