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https://www.readbyqxmd.com/read/28626825/phace-s-syndrome-report-of-a-case-with-new-ocular-and-systemic-manifestations
#1
Raheleh Assari, Vahid Ziaee, Sasan Moghimi, Mohammad Reza Akbari, Arash Mirmohammadsadeghi
PURPOSE: To describe an infant with PHACE(S) syndrome [posterior fossa anomalies (P), hemangiomas (H), arterial anomalies (A), cardiac abnormalities and coarctation of aorta (C), eye abnormalities (E), and the sternal defects (S)] with unusual strabismus, congenital glaucoma, and new systemic manifestations. METHODS: A 6-month-old girl was referred with large hemangiomas on the left side of the face. RESULTS: In the ocular examination, right esotropia and hypotropia, and limitation of elevation in adduction in the right eye were seen...
June 2017: Journal of Current Ophthalmology
https://www.readbyqxmd.com/read/28625902/obesity-in-neurosurgery
#2
REVIEW
Mendel D Castle-Kirszbaum, Jin W Tee, Patrick Chan, Martin K Hunn
Obesity is an important consideration in neurosurgical practice. 28.3% of Australian adults are obese and it is estimated that over two-thirds of Australia's population will be overweight or obese by 2025. This review of the effects of obesity on neurosurgical procedures demonstrated that, in patients undergoing spinal surgery, an increased BMI is a significant risk factor for surgical site infection, venous thromboembolism, major medical complications, prolonged length of surgery and increased financial cost...
June 15, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28623522/h3-k27m-mutations-are-extremely-rare-in-posterior-fossa-group-a-ependymoma
#3
Scott Ryall, Miguel Guzman, Samer K Elbabaa, Betty Luu, Stephen C Mack, Michal Zapotocky, Michael D Taylor, Cynthia Hawkins, Vijay Ramaswamy
BACKGROUND: Mutations in the tail of histone H3 (K27M) are frequently found in pediatric midline high-grade glioma's but have rarely been reported in other malignancies. Recently, recurrent somatic nucleotide variants in histone H3 (H3 K27M) have been reported in group A posterior fossa ependymoma (EPN_PFA), an entity previously described to have no recurrent mutations. However, the true incidence of H3 K27M mutations in EPN_PFA is unknown. METHODS: In order to discern the frequency of K27M mutations in histone H3 in EPN_PFA, we analyzed 151 EPN_PFA previously profiled with genome-wide methylation arrays using a validated droplet digital PCR assay...
June 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28617804/integrating-tenascin-c-protein-expression-and-1q25-copy-number-status-in-pediatric-intracranial-ependymoma-prognostication-a-new-model-for-risk-stratification
#4
Felipe Andreiuolo, Gwénaël Le Teuff, Mohamed Amine Bayar, John-Paul Kilday, Torsten Pietsch, André O von Bueren, Hendrik Witt, Andrey Korshunov, Piergiorgio Modena, Stefan M Pfister, Mélanie Pagès, David Castel, Felice Giangaspero, Leila Chimelli, Pascale Varlet, Stefan Rutkowski, Didier Frappaz, Maura Massimino, Richard Grundy, Jacques Grill
PURPOSE: Despite multimodal therapy, prognosis of pediatric intracranial ependymomas remains poor with a 5-year survival rate below 70% and frequent late deaths. EXPERIMENTAL DESIGN: This multicentric European study evaluated putative prognostic biomarkers. Tenascin-C (TNC) immunohistochemical expression and copy number status of 1q25 were retained for a pooled analysis of 5 independent cohorts. The prognostic value of TNC and 1q25 on the overall survival (OS) was assessed using a Cox model adjusted to age at diagnosis, tumor location, WHO grade, extent of resection, radiotherapy and stratified by cohort...
2017: PloS One
https://www.readbyqxmd.com/read/28612632/comparison-of-first-trimester-transvaginal-anatomy-scan-to-the-second-trimester-structural-survey-in-normal-weight-and-obese-populations-a-pilot-study
#5
Anna McCormick, Heather Anaya, Ramkrishna Mehendale, Louis Fogg, Michael Roche, Jacques Abramowicz
PURPOSE: To compare visualization of first trimester fetal anatomic transvaginal ultrasound (TVUS) to the second trimester transabdominal anatomic ultrasound (TAU) in normal weight and obese patients. MATERIALS AND METHODS: In a prospective cross-sectional study design, 25 women underwent a TVUS between 12-14 weeks and a TAU at 18-22 weeks. For each anatomic structure, the percentage of patients achieving optimal visualization was recorded. Risk ratios for visualizing a structure in the second trimester versus the first were calculated...
June 14, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28609892/reappraisal-of-i-synagrops-i-g%C3%A3-nther-1887-with-rehabilitation-and-revision-of-i-parascombrops-i-alcock-1889-including-description-of-seven-new-species-and-two-new-genera-perciformes-acropomatidae
#6
Werner W Schwarzhans, Artem M Prokofiev
An ongoing review of the fishes of the basal percoid family Acropomatidae has revealed that the genus Synagrops Günther, 1887 as it is currently understood is not a natural group. Species with a serrated pelvic-fin spine are here placed in the resurrected genus Parascombrops Alcock, 1889 (type-species: Parascombrops pellucidus Alcock, 1889), and the new, monospecific genus Caraibops n. gen. (type-species: Synagrops trispinosus Mochizuki & Sano, 1984). Parascombrops is unique amongst Acropomatidae in the combination of the presence of vacant 8th interneural space, a predorsal formula /0+0/0+2/ and an epaxialis attachment type 1...
May 4, 2017: Zootaxa
https://www.readbyqxmd.com/read/28608353/-causes-of-hospital-mortality-due-to-brain-haemorrhage-in-patients-with-arteriovenous-malformation
#7
J Vilalta, F Arikan, R Torne, R Najarro, D Lopez, A Rodriguez-Hernandez, D Gandara, P Banos
AIM: To determine the causes of mortality in cases of brain haemorrhage among patients with arteriovenous malformations (AVM) treated in a tertiary hospital. PATIENTS AND METHODS: The patients with AVM who died over the period 1990-2014 were selected from a prospective register of vascular malformations. Demographic aspects, localisation of the AVM, associated aneurysms and previous treatments were reviewed. Three main causes of death were established: initial bleeding/rebleeding, those related with the treatment of the AVM and other causes not related with AVM...
June 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28608052/craniofacial-and-dental-characteristics-of-patients-with-vitamin-d-dependent-rickets-type-1a-compared-to-controls-and-patients-with-x-linked-hypophosphatemia
#8
Hans Gjørup, Signe Sparre Beck-Nielsen, Dorte Haubek
ᅟOBJECTIVES: Vitamin-D-dependent rickets type 1A (VDDR1A) is a rare inherited disease caused by defective activation of vitamin D. The aim of the study was to describe the craniofacial characteristics and the dental phenotype of patients with genetically confirmed VDDR1A. The VDDR1A findings were compared to findings in patients with X-linked hypophosphatemia (XLH) and healthy controls. MATERIAL AND METHODS: Ten patients with VDDR1A were identified. The reference group for the comparison of cephalometric findings was 49 adults without chronic disease...
June 12, 2017: Clinical Oral Investigations
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
#9
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/28602922/the-spheno-pterional-point-a-strategic-point-for-burr-role-placement-in-frontotemporal-craniotomies
#10
Baltazar Leão Reis, Roberto Leal da Silveira, Sebastião Nataniel Silva Gusmão
BACKGROUND: Frontotemporal craniotomies are the most commonly performed neurosurgical approaches. We studied the external bony landmarks on the lateral surface of the skull to identify a "strategic" point where both the anterior and middle cranial fossae are exposed simultaneously during frontotemporal craniotomies through a single burr hole placed over the greater wing of sphenoid bone (spheno-pterional point). OBJECTIVE: This study aimed to anatomically define the spheno-pterional point via craniometric measurements taken on the lateral surface of the human skull...
June 8, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28602259/assessment-of-condyle-position-fossa-morphology-and-disk-displacement-in-symptomatic-patients
#11
Katharina Alves Rabelo, Saulo Leonardo Sousa Melo, Marianna Guanaes Gomes Torres, Larissa Rangel Peixoto, Paulo Sérgio Flores Campos, Iêda Margarida Crusoé Rocha Rebello, Daniela Pita de Melo
OBJECTIVE: The aim of the study was to determine whether there are any associations between the morphology of the glenoid fossa, articular spaces, and disk displacement by evaluating the magnetic resonance imaging (MRI) scans of symptomatic patients. Gender and age were also evaluated for any effect on the parameters. STUDY DESIGN: MRI scans of 199 temporomandibular joints were assessed for the morphology of the glenoid fossa and articular spaces in both sagittal and coronal views...
April 19, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28601978/recurrent-or-symptomatic-residual-posterior-fossa-hemangioblastomas-how-are-they-different-from-their-primary-counterparts
#12
Shruti Gupta, Lily Pal, Jayesh C Sardhara, Awadhesh K Jaiswal, Arun Srivastava, Anant Mehrotra, Kuntal Kanti Das, Sanjay Behari
BACKGROUND: Posterior fossa hemangioblastomas are WHO grade I benign lesions with a surprisingly high recurrence rate. This study determines the factors responsible for recurrence and the clinico-radiological and histopathological differences between primary (group A; n = 60) and recurrent/symptomatic residual (group B; n = 24) tumors. METHODS: Radiologically, tumors were differentiated into cystic, cystic with a mural nodule, solid-cystic/microcystic and solid...
June 11, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28600010/hemicraniectomy-for-ischemic-and-hemorrhagic-stroke-facts-and-controversies
#13
REVIEW
Aman Gupta, Mithun G Sattur, Rami James N Aoun, Chandan Krishna, Patrick B Bolton, Brian W Chong, Bart M Demaerschalk, Mark K Lyons, Jamal McClendon, Naresh Patel, Ayan Sen, Kristin Swanson, Richard S Zimmerman, Bernard R Bendok
Malignant large artery stroke is associated with high mortality of 70% to 80% with best medical management. Decompressive craniectomy (DC) is a highly effective tool in reducing mortality. Convincing evidence has accumulated from several randomized trials, in addition to multiple retrospective studies, that demonstrate not only survival benefit but also improved functional outcome with DC in appropriately selected patients. This article explores in detail the evidence for DC, nuances regarding patient selection, and applicability of DC for supratentorial intracerebral hemorrhage and posterior fossa ischemic and hemorrhagic stroke...
July 2017: Neurosurgery Clinics of North America
https://www.readbyqxmd.com/read/28598276/risk-factors-associated-with-postoperative-cerebrospinal-fluid-leak-after-endoscopic-endonasal-skull-base-surgery
#14
Shannon Fraser, Paul A Gardner, Maria Koutourousiou, Mark Kubik, Juan C Fernandez-Miranda, Carl H Snyderman, Eric W Wang
OBJECTIVE The aim in this paper was to determine risk factors for the development of a postoperative CSF leak after an endoscopic endonasal approach (EEA) for resection of skull base tumors. METHODS A retrospective review of patients who underwent EEA for the resection of intradural pathology between January 1997 and June 2012 was performed. Basic demographic data were collected, along with patient body mass index (BMI), tumor pathology, reconstruction technique, lumbar drainage, and outcomes. RESULTS Of the 615 patients studied, 103 developed a postoperative CSF leak (16...
June 9, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28597834/-magnetic-resonance-imaging-artefacts-and-fixed-orthodontic-attachments
#15
Aurélien Beau, Denis Bossard, Sarah Gebeile-Chauty
OBJECTIVES: Orthodontic appliances are often removed before magnetic resonance imaging (MRI) scans because they are known to produce artefacts. The purpose of this study was to find the exact indications for removal of various fixed attachments when imaging four specific areas of the head and neck. MATERIALS AND METHODS: Sixty patients requiring an MRI scan of the head for medical reasons volunteered for this investigation. One of four different types of fixed attachments (stainless steel brackets, titanium brackets, ceramic brackets with metal slots, and stainless steel retainers) were assigned to a patient...
June 2017: L' Orthodontie Française
https://www.readbyqxmd.com/read/28595841/an-atypical-variant-of-superolateral-dislocation-of-the-mandibular-condyle-a-case-report
#16
Kapil Malik, Subhas C Debnath, Apurba K Adhyapok, Kriti Hazarika
Dislocation of the mandibular condyle from the glenoid fossa can occur in anterior, posterior, lateral, and superior directions. Posterior, lateral, and superior dislocations are rare. Superolateral dislocation is seldom encountered in clinical practice. It is generally associated with fracture of the anterior or contralateral side of the mandible. The occurrence of superolateral dislocation of the condyle hooked above the zygomatic arch with an associated fracture of the medial pole of the condyle is rare and has been reported only once in the literature...
May 15, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28593553/prenatal-diagnosis-of-spina-bifida-from-intracranial-translucency-to-intrauterine-surgery
#17
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/28590989/-it-s-not-a-tumor-a-rare-case-of-symptomatic-cerebellar-developmental-venous-anomaly
#18
Julie A Augenstein, Teresa Chapman, Michael J McNeil, Mark D Lo
Cerebral developmental venous anomalies (DVAs) are benign anatomical variants of the venous system and are commonly described as an incidental finding without clinical significance. Neurologic symptoms or abnormal examination findings are rare and usually attributed to hemorrhagic complications related to coexisting cavernous malformations. There have been limited case reports of symptomatic, uncomplicated DVAs described in the literature. The following case describes a previously healthy child who presented to the emergency department with an acute onset of altered mental status, headache, and focal neurologic examination abnormalities...
June 6, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28590398/bilateral-coronoid-hyperplasia-in-a-43-year-old-patient-treated-with-intraoral-coronoidectomy
#19
Jonathan Ribeiro da Silva, Rodrigo Dos Santos Pereira, Caroline Águeda, Paulo Marcos Nunes, Charles Marin, Eduardo Hochuli-Vieira
The mandibular coronoid hyperplasia can be classified as a congenital or development change of the temporomandibular joint. This condition occurs due to a no-neoplasic growth of the coronoid process to the infratemporal fossa. Thus, this part of the bone impacts in the posterior face of the zygomatic bone causing limitation of the mouth opening. The aim of this paper is to report a clinical case of a 43-year-old male with mouth-opening limitation 28 years ago. After clinical and computed tomographic examinations, he was diagnosed with bilateral mandibular coronoid hyperplasia...
June 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28586935/endoscopic-management-of-arnold-chiari-malformation-type-i-with-or-without-syringomyelia
#20
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
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