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https://www.readbyqxmd.com/read/28820281/isolated-cranio-orbitofacial-neurofibroma-mimicking-vascular-malformation
#1
Harinder S Chahal, Brandon Kuiper, Puneet S Braich, A Tyrone Glover
Isolated neurofibromas in the absence of systemic neurofibromatosis are known as solitary or localized neurofibromas. When present in the orbit, these lesions may appear clinically and radiographically similar to other tumors, complicating diagnosis and management. Key radiographic signs may guide clinicians to the correct diagnosis when the presentation and patient demographic data obfuscate the disease entity. We present a case of a large multi-lobulated neurofibroma misdiagnosed as a lymphangioma. A brief review of these tumors emphasizing key radiographic features is also included...
August 18, 2017: Orbit
https://www.readbyqxmd.com/read/28819935/plasma-biomarkers-of-inflammation-reflect-seizures-and-hemorrhagic-activity-of-cerebral-cavernous-malformations
#2
Romuald Girard, Hussein A Zeineddine, Maged D Fam, Anoop Mayampurath, Ying Cao, Changbin Shi, Robert Shenkar, Sean P Polster, Michael Jesselson, Ryan Duggan, Abdul-Ghani Mikati, Gregory Christoforidis, Jorge Andrade, Kevin J Whitehead, Dean Y Li, Issam A Awad
The clinical course of cerebral cavernous malformations (CCMs) is highly variable. Based on recent discoveries implicating angiogenic and inflammatory mechanisms, we hypothesized that serum biomarkers might reflect chronic or acute disease activity. This single-site prospective observational cohort study included 85 CCM patients, in whom 24 a priori chosen plasma biomarkers were quantified and analyzed in relation to established clinical and imaging parameters of disease categorization and severity. We subsequently validated the positive correlations in longitudinal follow-up of 49 subjects...
August 17, 2017: Translational Stroke Research
https://www.readbyqxmd.com/read/28818233/transcatheter-embolization-of-persistent-embryonic-veins-in%C3%A2-venous-malformation-syndromes
#3
Naiem Nassiri, Dustin Crystal, Lauren A Huntress, Susan Murphy
Persistent embryonic veins represent a major source of venous hypertension and morbidity in venous malformation syndromes, such as Klippel-Trénaunay syndrome and congenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal deformities syndrome. Surgical stripping and phlebectomy are the most commonly reported alternatives to compression therapy for refractory cases. These techniques, although effective in those patients who meet the necessary anatomic criteria, can be associated with bleeding, wound-related complications, and recurrence...
September 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28818232/review-of-the-endothelial-pathogenic-mechanism-of-tie2-related-venous-malformation
#4
REVIEW
Zhong Du, JiaWei Zheng, ZhiYuan Zhang, YanAn Wang
BACKGROUND: Venous malformation (VM) is a type of disease involving vascular morphogenesis in humans. Clinically, VM can be sporadic or inherited. TIE2, also known as TEK or HYK, is a member of the receptor tyrosine kinase subfamily and is highly conserved among species. In 1996, an arginine-to-tryptophan substitution at position 849 (R849W) in TIE2 was found to induce hereditary VM. Additional alterations in TIE2 involved in the pathogenesis of inherited or sporadic VM have since been reported...
September 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28818226/percutaneous-ultrasound-guided-sclerotherapy-with-polidocanol-microfoam-for-lymphatic-malformations
#5
Takashi Yamaki, Yumiko Sasaki, Yuki Hasegawa, Atsuyoshi Osada, Hisato Konoeda, Atsumori Hamahata, Motohiro Nozaki, Hiroyuki Sakurai
BACKGROUND: Lymphatic malformations (LMs) are low-flow congenital lesions that consist of cysts of varying size. Sclerotherapy with intralesional bleomycin and OK-432 has been reported to yield dramatically beneficial results for this disorder. However, inflammation-related symptoms are often seen after treatment with these sclerosing agents. On the other hand, polidocanol (POL) is reportedly associated with fewer allergic and inflammatory reactions. Up to now, however, very few reports have documented the use of POL microfoam for treatment of LMs...
September 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28816965/blue-rubber-bleb-nevus-syndrome-our-experience-and-new-endoscopic-management
#6
Wenguo Chen, Hongtan Chen, Guodong Shan, Ming Yang, Fengling Hu, Qi Li, Lihua Chen, Guoqiang Xu
The aim of our study is to enhance the awareness of blue rubber bleb nevus syndrome (BRBNS) through the patients in our hospital and introduced a new measure of endoscopic intervention.A retrospective review of 5 patients, who were diagnosed as BRBNS in our hospital from January 2013 to January 2017, was conducted. Data were collected with regard to demographics, clinical presentation, endoscopic and imaging findings, management, and follow-up data.In total of 5 patients, the mean age was 28.8 years, range 16 to 44 years (male/female, 1/4) with the average initial age of onset 15...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28816935/perimesencephalic-nonaneurysmal-subarachnoid-hemorrhage-caused-by-transverse-sinus-thrombosis-a-case-report-and-review-of-literature
#7
Fang-Wang Fu, Jie Rao, Yuan-Yuan Zheng, Liang Song, Wei Chen, Qi-Hui Zhou, Jian-Guang Yang, Jiang-Qiong Ke, Guo-Qing Zheng
RATIONALE: Perimesencephalic nonaneurysmal subarachnoid hemorrhage (PNSAH) is characterized by a pattern of extravasated blood restricted to the perimesencephalic cisterns, normal angiographic findings, and an excellent prognosis with an uneventful course and low risks of complication. The precise etiology of bleeding in patients with PNSAH has not yet been established. The most common hypothesis is that PNSAH is venous in origin. Intracranial venous hypertension has been considered as the pivotal factor in the pathogenesis of PNSAH...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28814672/vipar-a-quantitative-approach-to-3d-histopathology-applied-to-lymphatic-malformations
#8
René Hägerling, Dominik Drees, Aaron Scherzinger, Cathrin Dierkes, Silvia Martin-Almedina, Stefan Butz, Kristiana Gordon, Michael Schäfers, Klaus Hinrichs, Pia Ostergaard, Dietmar Vestweber, Tobias Goerge, Sahar Mansour, Xiaoyi Jiang, Peter S Mortimer, Friedemann Kiefer
BACKGROUND: Lack of investigatory and diagnostic tools has been a major contributing factor to the failure to mechanistically understand lymphedema and other lymphatic disorders in order to develop effective drug and surgical therapies. One difficulty has been understanding the true changes in lymph vessel pathology from standard 2D tissue sections. METHODS: VIPAR (volume information-based histopathological analysis by 3D reconstruction and data extraction), a light-sheet microscopy-based approach for the analysis of tissue biopsies, is based on digital reconstruction and visualization of microscopic image stacks...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28811547/up-regulation-of-nadph-oxidase-mediated-redox-signaling-contributes-to-the-loss-of-barrier-function-in-krit1-deficient-endothelium
#9
Luca Goitre, Peter V DiStefano, Andrea Moglia, Nicholas Nobiletti, Eva Baldini, Lorenza Trabalzini, Julie Keubel, Eliana Trapani, Vladimir V Shuvaev, Vladimir R Muzykantov, Ingrid H Sarelius, Saverio Francesco Retta, Angela J Glading
The intracellular scaffold KRIT1/CCM1 is an established regulator of vascular barrier function. Loss of KRIT1 leads to decreased microvessel barrier function and to the development of the vascular disorder Cerebral Cavernous Malformation (CCM). However, how loss of KRIT1 causes the subsequent deficit in barrier function remains undefined. Previous studies have shown that loss of KRIT1 increases the production of reactive oxygen species (ROS) and exacerbates vascular permeability triggered by several inflammatory stimuli, but not TNF-α...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28808648/traumatic-arteriovenous-malformation-of-the-superficial-temporal-artery
#10
Mahmoud Omrani Fard, Omid Yousofnejad, Mohammadbagher Heydari
Most of the vascular lesions on head and neck soft tissue are congenital, but a rare cause can be trauma. A 23-year-old man came to our clinic with a wide pulsatile tortuous mass in the left temporofrontal area. That mass was appeared since 10 years ago. Ten years before his coming to our clinic, he had a blunt trauma in that area. After that, a small wound appeared there and healed gradually. In clinical examination, mass was large and pulsatile, and a fine murmur was detected from it. In paraclinical examination, computed tomography scan with intravenous contrast and sonography revealed a vascular mass with arteriovenous (AV) fistula in soft tissue only in that area...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28806758/developmental-vascular-remodeling-defects-and-postnatal-kidney-failure-in-mice-lacking-gpr116-adgrf5-and-eltd1-adgrl4
#11
Shun Lu, Shuya Liu, Astrid Wietelmann, Baktybek Kojonazarov, Ann Atzberger, Cong Tang, Ralph Theo Schermuly, Hermann-Josef Gröne, Stefan Offermanns
GPR116 (ADGRF5) and ELTD1 (ADGRL4) belong to different subfamilies of the adhesion G-protein-coupled receptor group but are both expressed in endothelial cells. We therefore analyzed their functions in mice lacking these receptors. While loss of GPR116 or ELTD1 alone had no obvious effect on cardiovascular or kidney function, mice lacking both, GPR116 and ELTD1, showed malformations of the aortic arch arteries and the cardiac outflow tract leading to perinatal lethality in about 50% of the mutants. In addition to cardiovascular malformations, surviving mice developed renal thrombotic microangiopathy as well as hemolysis and splenomegaly, and their lifespan was significantly reduced...
2017: PloS One
https://www.readbyqxmd.com/read/28805772/-diagnosis-and-treatment-of-extracranial-internal-carotid-artery-lesion-combined-with-cerebrovascular-malformation
#12
A I Khripun, A D Pryamikov, A B Mironkov, S A Asratyan, I N Tyurin, V S Suryakhin, M V Abashin, V P Mikhaylenko
AIM: To analyze own experience of diagnosis and treatment of patients with extracranial internal carotid artery lesion and cerebrovascular malformations. MATERIAL AND METHODS: There were 16 patients with combined lesion of extracranial and intracranial internal carotid artery for the period January 2013 - December 2014. Occlusive-stenotic lesion and tortuosity of ICA were observed in 11 and 5 cases respectively. RESULTS: Incidence of combined ICA lesion was 5...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28804779/language-mapping-using-t2-prepared-bold-functional-mri-in-the-presence-of-large-susceptibility-artifacts-initial-results-in-patients-with-brain-tumor-and-epilepsy
#13
Jun Hua, Xinyuan Miao, Shruti Agarwal, Chetan Bettegowda, Alfredo Quiñones-Hinojosa, John Laterra, Peter C M Van Zijl, James J Pekar, Jay J Pillai
At present, presurgical functional mapping is the most prevalent clinical application of functional magnetic resonance imaging (fMRI). Signal dropouts and distortions caused by susceptibility effects in the current standard echo planar imaging (EPI)-based fMRI images are well-known problems and pose a major hurdle for the application of fMRI in several brain regions, many of which are related to language mapping in presurgical planning. Such artifacts are particularly problematic in patients with previous surgical resection cavities, craniotomy hardware, hemorrhage, and vascular malformation...
June 2017: Tomography: a Journal for Imaging Research
https://www.readbyqxmd.com/read/28802071/microrna-137-and-195-inhibit-vasculogenesis-in-brain-arteriovenous-malformations
#14
Jun Huang, Jianping Song, Meijie Qu, Yang Wang, Qinzhu An, Yaying Song, Wei Yan, Bingshun Wang, Xiaojin Wang, Song Zhang, Xi Chen, Bing Zhao, Peixi Liu, Tongyi Xu, Zhijun Zhang, David A Greenberg, Yongting Wang, Pingjin Gao, Wei Zhu, Guo-Yuan Yang
OBJECTIVES: Brain arteriovenous malformations (AVMs) are the most common cause of non-traumatic intracerebral hemorrhage in young adults. The genesis of brain AVM remains enigmatic. We investigated microRNA (miRNA) expression and its contribution to the pathogenesis of brain AVMs. METHODS: We used a large-scale miRNA analysis on 16 samples including AVMs, hemangioblastoma, and controls to identify a distinct AVM miRNA signature. AVM smooth muscle cells (AVMSMCs) were isolated and identified by flow cytometry and immunohistochemistry and candidate miRNAs were then tested in these cells...
August 12, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28796572/endoglin-and-alk1-as-therapeutic-targets-for-hereditary-hemorrhagic-telangiectasia
#15
Lidia Ruiz-Llorente, Eunate Gallardo-Vara, Elisa Rossi, David M Smadja, Luisa M Botella, Carmelo Bernabeu
Hereditary Haemorrhagic Telangiectasia (HHT) is as an autosomal dominant trait characterized by frequent nose bleeds, mucocutaneous telangiectases, arteriovenous malformations (AVMs) of the lung, liver and brain, and gastrointestinal bleedings due to telangiectases. HHT is originated by mutations in genes whose encoded proteins are involved in the transforming growth factor β (TGF-β) family signalling of vascular endothelial cells. In spite of the great advances in the diagnosis as well as in the molecular, cellular and animal models of HHT, the current treatments remain just at the palliative level...
August 10, 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28791278/venous-thromboembolism-in-pediatric-vascular-anomalies
#16
REVIEW
Taizo A Nakano, Chadi Zeinati
The presence of a vascular anomaly suggests that capillaries, veins, arteries, and/or lymphatic vessels have demonstrated abnormal development and growth. Often dilated and misshaped, these vessels augment normal flow of blood and lymphatic fluids that increases the overall risk to develop intralesional thrombosis. Abnormal endothelial and lymphoendothelial cells activate hemostasis and hyperfibrinolytic pathways through poorly understood mechanisms, which contribute to the development of localized intravascular coagulopathy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28780588/spontaneous-resolution-of-low-flow-spinal-arteriovenous-fistulas
#17
Joe Kang, Lydia Gregg, Philippe Gailloud
PURPOSE: Spinal vascular anomalies with arteriovenous blood shunting include spinal arteriovenous malformations (SAVMs) and spinal arteriovenous fistulas (SAVFs), which are distinguished by the presence or absence of an interposed nidus. SAVFs can be further characterized based on their location (perimedullary, dural, or extradural) and flow pattern (high-flow versus low-flow shunts). The spontaneous resolution of a spinal vascular malformation, i.e., the complete disappearance-in the absence of therapeutic measures-of a lesion previously identified by angiography, seems to represent an exceptional phenomenon...
August 5, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28777021/masson-s-tumor-of-the-pineal-region-case-report
#18
Lefko T Charalambous, Anirudh Penumaka, Jordan M Komisarow, Amanda C Hemmerich, Thomas J Cummings, Patrick J Codd, Allan H Friedman
Intracranial intravascular papillary endothelial hyperplasia (IPEH), also referred to as Masson's tumor, is a condition that rarely occurs in the nervous system. IPEH most frequently occurs extracranially in the skin of the face, skull, neck, and trunk and can easily be mistaken clinically, radiologically, and histologically for angiosarcoma, organizing hematoma, or other vascular malformations. IPEH accounts for roughly 2% of all vascular tumors and is extremely rare intracranially, with only 23 reported cases compared with more than 300 cases of IPEH occurring in the skin and subcutaneous tissue...
August 4, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28775059/basal-ganglia-t1-hyperintensity-in-hereditary-hemorrhagic-telangiectasia
#19
A Parvinian, V N Iyer, B S Pannu, D R Apala, C P Wood, W Brinjikji
BACKGROUND AND PURPOSE: The implications of basal ganglia T1 hyperintensity remain unclear in patients with hereditary hemorrhagic telangiectasia. This study was performed to assess the prevalence of this imaging finding in a large cohort of patients with hereditary hemorrhagic telangiectasia and to identify any association between this phenomenon and other disease manifestations. MATERIALS AND METHODS: In this retrospective study, we identified all patients at our institution diagnosed with definite hereditary hemorrhagic telangiectasia from 2001 to 2017...
August 3, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28764614/quantitative-evaluation-of-arteriovenous-malformation-hemodynamic-changes-after-endovascular-treatment-using-parametric-color-coding-a-case-series-study
#20
Rodrigo Rivera, Juan G Sordo, Daniel Echeverria, Lautaro Badilla, Camila Pinto, Catalina Merino-Osorio
Background Brain arteriovenous malformations (AVMs) are complex vascular lesions. Endovascular treatment results are usually measured by calculating the volume reduction of the lesions. Nevertheless, vascular flow quantification seems a more physiologically accurate way of measuring endovascular results. We evaluated the use of parametric color coding (PCC) with digital subtraction angiography (DSA), in order to determine the feasibility of PCC to detect and measure the impact of AVM endovascular treatment-induced changes using real-time hemodynamic parameters...
January 1, 2017: Interventional Neuroradiology
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