Arteriovenous Malformation of the Head and Neck in a Hemodialysis Patient.
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ABSTRACT:
SUBMITTER: Hattori Y
PROVIDER: S-EPMC7990015 | biostudies-literature | 2021 Mar
REPOSITORIES: biostudies-literature
Publications
Arteriovenous Malformation of the Head and Neck in a Hemodialysis Patient.
Hattori Yoshitsugu Y Kurita Masakazu M Yokoyama Takayuki T Yamashita Shuji S Okazaki Mutsumi M
Plastic and reconstructive surgery. Global open 20210324 3
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Project description:IntroductionThe "seagull cry" is an acoustic phenomenon heard during duplex ultrasound. It is caused by harmonic covibrations of a vessel wall in the presence of high-velocity blood flow. It has been reported in a few cases of cerebrovascular disease, such as severe intracranial stenosis, vasospasm or carotid-cavernous fistula.Material and methodsA 35-year-old man underwent transcranial color-coded sonography (TCCS) for work-up of a severe new-onset headache.ResultsDoppler spectral analysis of the right intracranial carotid bifurcation revealed multiple pairs of mirror-image parallel strings, and a high-frequency seagull cry was heard. Computed tomography-angiography and magnetic resonance imaging of the brain showed an arteriovenous malformation in the right temporal lobe.DiscussionThe seagull cry is a "musical murmur" with single or multiple frequency that sounds like a musical tone. This is the first report of this phenomenon in a cerebral arteriovenous malformation.
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Project description:ObjectiveTo identify risk factors for intracranial hemorrhage in the natural history course of brain arteriovenous malformations (AVMs) using individual patient data meta-analysis of 4 existing cohorts.MethodsWe harmonized data from Kaiser Permanente of Northern California (n = 856), University of California San Francisco (n = 787), Columbia University (n = 672), and the Scottish Intracranial Vascular Malformation Study (n = 210). We censored patients at first treatment, death, last visit, or 10-year follow-up, and performed stratified Cox regression analysis of time-to-hemorrhage after evaluating hemorrhagic presentation, sex, age at diagnosis, deep venous drainage, and AVM size as predictors. Multiple imputation was performed to assess impact of missing data.ResultsA total of 141 hemorrhage events occurred during 6,074 patient-years of follow-up (annual rate of 2.3%, 95% confidence interval [CI] 2.0%-2.7%), higher for ruptured (4.8%, 3.9%-5.9%) than unruptured (1.3%, 1.0%-1.7%) AVMs at presentation. Hemorrhagic presentation (hazard ratio 3.86, 95% CI 2.42-6.14) and increasing age (1.34 per decade, 1.17-1.53) independently predicted hemorrhage and remained significant predictors in the imputed dataset. Female sex (1.49, 95% CI 0.96-2.30) and exclusively deep venous drainage (1.60, 0.95-2.68, p = 0.02 in imputed dataset) may be additional predictors. AVM size was not associated with intracerebral hemorrhage in multivariable models (p > 0.5).ConclusionThis large, individual patient data meta-analysis identified hemorrhagic presentation and increasing age as independent predictors of hemorrhage during follow-up. Additional AVM cohort data may further improve precision of estimates, identify new risk factors, and allow validation of prediction models.
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Project description:BACKGROUND:Pulmonary arteriovenous malformations are abnormal direct connections between the pulmonary artery and pulmonary vein which result in a right-to-left shunt. They are associated with substantial morbidity and mortality mainly from the effects of paradoxical emboli. Potential complications include stroke, cerebral abscess, pulmonary haemorrhage and hypoxaemia. Embolisation is an endovascular intervention based on the occlusion of the feeding arteries the pulmonary arteriovenous malformations thus eliminating the abnormal right-to-left-shunting. This is an update of a previously published review. OBJECTIVES:To determine the efficacy and safety of embolisation in patients with pulmonary arteriovenous malformations including a comparison with surgical resection and different embolisation devices. SEARCH METHODS:We searched the Cystic Fibrosis and Genetic Disorders Group's Trials Register; date of last search: 10 April 2017.We also searched the following databases: the Australian New Zealand Clinical Trials Registry; ClinicalTrials.gov; International Standard Randomised Controlled Trial Number Register; International Clinical Trials Registry Platform Search Portal (last searched 27 August 2017). to be updatedWe checked cross-references and searched references from review articles. SELECTION CRITERIA:Trials in which individuals with pulmonary arteriovenous malformations were randomly allocated to embolisation compared to no treatment, surgical resection or embolisation using a different embolisation device. DATA COLLECTION AND ANALYSIS:Studies identified for potential inclusion were independently assessed for eligibility by two authors, with excluded studies further checked by a third author. No trials were identified for inclusion in the review and hence no analysis was performed. MAIN RESULTS:There were no randomised controlled trials included in the review; one ongoing trial has been identified which may be eligible for inclusion in the future. AUTHORS' CONCLUSIONS:There is no evidence from randomised controlled trials for embolisation of pulmonary arteriovenous malformations. However, randomised controlled trials are not always feasible on ethical grounds. Accumulated data from observational studies suggest that embolisation is a safe procedure which reduces morbidity and mortality. A standardised approach to reporting with long-term follow-up through registry studies can help to strengthen the evidence for embolisation in the absence of randomised controlled trials.
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Project description:Cerebral arteriovenous malformations (AVMs) are the most common vascular malformations worldwide and the leading cause of hemorrhagic strokes that result in crippling neurological deficits. Here, using newly generated mouse model, we discovered that genome-wide ocuppancy of H4K8ac and H3K27me3 decreased in mouse cerebral AVMs.
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Project description:Somatic activating MAP2K1 mutations in endothelial cells (ECs) cause extracranial arteriovenous malformation (AVM). We previously reported the generation of a mouse line allowing inducible expression of constitutively active MAP2K1 (p.K57N) from the Rosa locus (R26GT-Map2k1-GFP/+) and showed, using Tg-Cdh5CreER, that EC expression of mutant MAP2K1 is sufficient for the development of vascular malformations in the brain, ear, and intestines. To gain further insight into the mechanism by which mutant MAP2K1 drives AVM development, we induced MAP2K1 (p.K57N) expression in ECs of postnatal-day-1 pups (P1) and investigated the changes in gene expression in P9 brain ECs by RNA-seq. We found that over-expression of MAP2K1 altered the transcript abundance of > 1600 genes. Several genes had > 20-fold changes between MAP2K1 expressing and wild-type ECs; the highest were Col15a1 (39-fold) and Itgb3 (24-fold). Increased expression of COL15A1 in R26GT-Map2k1-GFP/+; Tg-Cdh5CreER+/- brain ECs was validated by immunostaining. Ontology showed that differentially expressed genes were involved in processes important for vasculogenesis (e.g., cell migration, adhesion, extracellular matrix organization, tube formation, angiogenesis). Understanding how these genes and pathways contribute to AVM formation will help identify targets for therapeutic intervention.
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Project description:Hemoptysis is a common presenting symptom of hereditary hemorrhagic telangiectasia (HHT), an often overlooked diagnosis in the emergency setting. Patient history often includes telangiectasias, epistaxis, visceral lesions, and a family history of similar findings. Here, we review a case of HHT in a young woman, presenting initially with hemoptysis. Imaging was significant for large left upper lobe arteriovenous malformation (AVM), requiring patient admission and interventional radiology embolization of the AVM. The purpose of this report is to highlight a diagnosis that is important not to miss in the emergency department.TopicsPulmonary arteriovenous malformation, hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome.
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Project description:Background and purposeDo children have an increased risk for brain arteriovenous malformation (AVM) recurrence compared with adults and does this risk vary depending on initial presentation with AVM rupture?MethodsWe retrospectively studied 115 patients initially presenting with brain AVM under age 25 years who underwent complete surgical resection of the AVM as documented by digital subtraction angiography (DSA) and had delayed follow-up DSA to evaluate for AVM recurrence after apparent initial cure.ResultsThe mean time from baseline DSA to follow-up DSA was 2.3 years, ranging from 0 to 15 years. Twelve patients (10.4% of the 115 patient cohort and 16.7% of 72 patients with hemorrhage at initial presentation) demonstrated AVM recurrence on follow-up DSA. All patients with recurrence initially presented with intracranial hemorrhage, and intracranial hemorrhage was a significant predictor of recurrence (log rank P=0.037). Among patients with initial hemorrhage, the 5-year recurrence rate was 17.8% (95% CI, 8.3%-35.7%). All recurrences occurred in patients who were children at the time of their initial presentation; the oldest was 15 years of age at the time of initial AVM surgery. The 5-year recurrence rate for children (0-18 years of age) with an initial presentation of hemorrhage was 21.4% (95% CI, 10.1%-41.9%). Using Cox regression, we found the risk of AVM recurrence decreased by 14% per each year increase in age at the time of initial surgical resection (hazard ratio=0.86 [95% CI, 0.75-0.99]; P=0.031).ConclusionsThere is a high rate of recurrence of apparently cured brain AVMs in children who initially present with AVM rupture. Imaging follow-up is warranted to prevent re-rupture.
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Project description:Torrential bleeding is a rare and life-threatening complication of arteriovenous malformations (AVMs). We report a case of head and neck AVMs present with uncontrollable torrential bleeding, which was treated with embolization and sclerotherapy. Then we explored the potential multidisciplinary handling of the procedure for this kind of case. A 25-year-old female patient was born with right face and head AVMs. The AVMs had grown gradually and ruptured spontaneously with uncontrollable torrential bleeding before admission. Emergent direct hemostasis, nasotracheal intubation, and staged embolization and sclerotherapy were carried out on this patient. Finally, the bleeding stopped and the wound healed successfully. Embolization and sclerotherapy are effective for head and neck AVMs with uncontrollable torrential bleeding. Multidisciplinary collaboration is needed to achieve a good outcome.
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