Arteriovenous Malformations

动静脉畸形
  • 文章类型: Journal Article
    脑动静脉畸形(AVM)是具有挑战性的血管病变。需要广泛的后续研究来完善治疗算法,并提高这些患者的长期生存率。该研究的目的是评估手术结果,并评估接受脑AVM治疗的患者的总体长期死亡率。
    这项回顾性单中心研究包括191名患有脑AVM的患者,在2012年至2022年期间承认。已经分析了临床和血管造影的特殊性,确定可能影响早期结局和总体长期死亡率的因素.
    在79名接受手术的患者中,51例AVM破裂,全切除68例(86.1%)。深静脉引流与不完全切除有关。女性性别,入院改良的Rankin量表(mRS)>2,以及雄辩的位置是不良结局的独立预测因子.多静脉引流与早期预后恶化的风险较高相关。雄辩的大脑区域受累,保守治疗,年龄越来越大,入院时mRS>2,合并症显著降低了脑AVM患者的生存率。接受介入治疗的患者的生存率明显优于保守治疗的患者。当调整年龄和入院时。
    该研究确定了女性性别,入院时神经功能不良和口才是手术后阴性结局的独立预后因素。接受介入治疗的患者的生存率明显优于保守治疗的患者。我们建议采用量身定制的,积极的管理策略,因为它们显着提高脑AVM患者的长期生存率。
    UNASSIGNED: Brain arteriovenous malformations (AVMs) are challenging vascular lesions. Extensive follow-up studies are necessary to refine the therapeutic algorithm, and to improve long-term survival in these patients. The aim of the study was to assess surgical outcomes, and to evaluate overall long-term mortality in patients treated for brain AVMs.
    UNASSIGNED: This retrospective single-center study included 191 patients with brain AVMs, admitted between 2012 and 2022. Clinical and angiographical particularities have been analyzed, to identify factors that might influence early outcome and overall long-term mortality.
    UNASSIGNED: Out of 79 patients undergoing surgery, 51 had ruptured AVMs with total resection achieved in 68 cases (86.1%). Deep venous drainage was associated with incomplete resection. Female sex, admission modified Rankin Scale (mRS) > 2, and eloquent location were independent predictors of poor outcomes. Multiple venous drainage was associated with a higher risk of worsened early outcome. Eloquent brain region involvement, conservative treatment, increasing age, admission mRS > 2, and comorbidities significantly decrease survival in brain AVM patients. Patients treated with interventional treatments had significantly better survival than the conservatively managed ones, when adjusting for age and admission mRS.
    UNASSIGNED: The study identified female sex, poor neurologic status on admission and eloquence as independent prognostic factors for a negative outcome after surgery. Patients who received interventional treatment had significantly better survival than patients managed conservatively. We recommend employing tailored, proactive management strategies as they significantly enhance long-term survival in brain AVM patients.
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  • 文章类型: Case Reports
    动静脉畸形(AVM)是一种罕见的先天性血管异常,可影响脉管系统并涉及任何解剖结构的内皮和邻近细胞。AVM在组织学上的特征是异常的AV分流与非典型的互连毛细血管床。AVM会导致功能和美学问题,比如面部不对称,疼痛,溶骨变化,以及未预期的出血或周围组织的挤压和撕裂,而不会引起任何症状。文献检索在面部区域获得了有限的AVM病例报告。诊断不足,知识有限,缺乏文献会导致拔牙等牙科手术后严重出血和潜在致命的出血事件,手术,刺伤,或受灾地区的钝伤。在这份手稿中,我们报道一例房室畸形累及左脸颊和颊粘膜区的37岁男性患者,报告创伤后出血不受控制.本报告重点介绍了通过面部动脉结扎和手术切除在紧急情况下治疗房室畸形的方法。
    An arteriovenous malformation (AVM) is an infrequent congenital vascular anomaly that can affect the vasculature and involve the endothelium and neighboring cells of any anatomical structure. AVMs are characterized histologically by abnormal AV shunts with atypical interconnecting capillary beds. AVM can cause functional and esthetic issues like face asymmetry, pain, osteolytic changes, and unanticipated hemorrhage or squeeze and tear of the surrounding tissue without causing any symptoms. The literature search yielded limited case reports on AVMs in the facial region. Insufficient diagnosis, limited knowledge, and a lack of literature can lead to severe bleeding and potentially fatal hemorrhagic incidents following dental procedures like tooth extraction, surgery, puncture wounds, or blunt injuries in the affected area. In this manuscript, we report a case of AV malformation involving the left cheek and buccal mucosa region in a 37-year-old male patient who reported uncontrolled bleeding after trauma. This report highlights the management of AV malformation in an emergency by facial artery ligation and surgical excision.
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  • 文章类型: Journal Article
    目的:确定脑动静脉畸形(rBAVM)破裂患者是否会从早期栓塞中获益。
    方法:rBAVM首先在2002年3月至2022年5月之间通过栓塞治疗。当在出血后10天内进行时,早期定义为栓塞(第1组)。如果以后,栓塞被认为是晚期(第2组)。比较两组人口统计学和rBAVM数据。检索了高危出血成分和推迟栓塞的原因。主要终点为再出血。次要终点是良好的功能结果(FO,改良Rankin量表mRS≤2)和血管造影闭塞。通过多变量分析确定再出血和FO的预测因子。
    结果:招募了105名患者(第1组N=34;第2组N=71)。之前没有发现再出血,在早期栓塞组的第一次栓塞期间或之后。晚期栓塞取决于漏诊和转诊方式。11名患者(10.5%)出现了再出血,其中在栓塞前N=3(仅在第2组中),N=5周栓塞(第1组第二次栓塞时N=2),栓塞后30天以上自发N=3。第1组栓塞了更多的高危成分(19/34;55.9vs17/71;23.9%;p=.011)。再出血率,末次FU的FO(90.9%vs74.3%)和闭塞率(80.8%vs88.5%)在组间没有差异。格拉斯哥昏迷评分≤8预测再出血,再出血与FO不良相关。
    结论:早期栓塞确实可以预防再出血。总体再出血风险与晚期栓塞前出血和第二次栓塞时出血有关。再出血预测了最终的FO。
    OBJECTIVE: To determine whether patients with a ruptured brain arteriovenous malformation (rBAVM) would benefit from an early embolization.
    METHODS: rBAVM treated first by embolization between March 2002 and May 2022 were included. Embolization was defined early (Group 1) when performed within 10 days postbleeding. If later, embolization was considered late (Group 2). Demographic and rBAVM data were compared between the groups. High-risk bleeding components and reasons for deferring embolization were retrieved. Primary endpoint was rebleeding. Secondary endpoints were good functional outcome (FO, modified Rankin Scale mRS ≤ 2) and angiographic occlusion. Predictors of rebleeding and FO were determined by multivariate analysis.
    RESULTS: 105 patients were recruited (N = 34 in Group 1; N = 71 in Group 2). No rebleeding was noted before, during or after the first embolization session in the early embolization group. Late embolization depended on missed diagnosis and referral pattern. Eleven patients (10.5%) suffered a rebleeding, of whom N = 3 before embolization (only in Group 2), N = 5 periembolization (N = 2 at the second embolization session in Group 1) and N = 3 spontaneous more than 30 days postembolization. More high-risk components were embolized in Group 1 (19/34; 55.9 vs 17/71; 23.9%; p = .011). Rebleeding rates, FO at last FU (90.9% vs 74.3%) and occlusion rates (80.8% vs 88.5%) did not differ between the groups. Glasgow coma scale ≤ 8 predicted rebleeding, rebleeding correlated with poor FO.
    CONCLUSIONS: Early embolization did prevent rebleeding. The overall rebleeding risk was linked to bleeding before late embolization and bleeding at the second embolization. Rebleeding predicted the final FO.
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  • 文章类型: Journal Article
    目的:手动静脉畸形(AVM)因其功能重要性和美容功能缺陷而极难处理。进行了一项单中心回顾性研究,以确定手AVM多学科团队管理的长期结果。
    方法:机构审查委员会批准了这项回顾性研究。回顾了1995年至2023年的多学科血管异常中心数据。病人的人口统计学,Schobinger的AVM阶段,硬化治疗细节,手术史,并对硬化治疗后的不良事件进行了回顾。
    结果:从1995年至2023年,共有150例手部AVM患者来我院就诊,平均年龄为33岁(范围,1-75岁)和91名女性。44例患者为SchobingerII期,106例为III期。对101例患者(67%)进行了硬化治疗,共320次。硬化治疗后血管造影断流率为:16,100%,30,超过90%,34与50-90%,15,0-50%,六个人表现出恶化。硬化治疗相关的不良事件发生在320个疗程中的123个(39%),有112个小事件和11个大事件。15例患者(15%)最终在硬化治疗后平均1618天接受了截肢手术(n=3)和延迟并发症(n=12)。13例患者(9%)因溃疡或出血而接受了初次手术截肢(均为SchobingerIII期)。36名患者(24%)在没有任何手术的情况下进行了随访。
    结论:手部AVM的多学科管理显示出不同的长期结果。虽然硬化疗法对许多患者有效,它具有严重的不良事件风险。在某些情况下,截肢的必要性凸显了晚期AVM的严重程度以及对个性化治疗方法的需求。
    OBJECTIVE: Hand arteriovenous malformations (AVMs) are extremely difficult to manage for their functional importance and cosmetic disfiguration. A single center retrospective study was conducted to identify long-term outcomes of multidisciplinary team management of hand AVM.
    METHODS: Institutional review board approved this retrospective study. Multidisciplinary vascular anomalies center data was reviewed from 1995 to 2023. Patient\'s demographics, Schobinger\'s AVM stage, sclerotherapy details, surgical history, and adverse events after sclerotherapy were reviewed.
    RESULTS: A total of 150 hand AVM patients visited our hospital from 1995 to 2023, with a mean age of 33 years (range, 1-75 years) and 91 females. Forty-four patients were Schobinger stage II and 106 were stage III. Sclerotherapy was performed on 101 patients (67%) with 320 sessions. Angiographic devascularization rates after sclerotherapy were: 16 with 100%, 30 with over 90%, 34 with 50-90%, 15 with 0-50%, and six showed aggravation. Sclerotherapy-related adverse events occurred in 123 of 320 sessions (39%), with 112 minor and 11 major events. Fifteen patients (15%) eventually underwent amputation surgery a mean of 1618 days after sclerotherapy for necrosis (n=3) and delayed complications (n=12). Thirteen patients (9%) underwent primary surgical amputation for ulcers or bleeding (all Schobinger stage III). Thirty-six patients (24%) were followed without any procedure.
    CONCLUSIONS: Multidisciplinary management of hand AVMs shows varied long-term outcomes. While sclerotherapy is effective for many patients, it carries a significant risk of adverse events. The necessity for amputation in some cases highlights the severity of advanced AVMs and the need for individualized treatment approaches.
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  • 文章类型: Journal Article
    Endoglin(ENG)突变导致1型遗传性出血性毛细血管扩张症(HHT1)。HHT1患者有多个器官的动静脉畸形(AVM),包括大脑。在老鼠身上,由R26RCreER或SM22αCre诱导的Eng缺失导致脑和其他器官中的AVM发育。我们假设负电ECs的增加将增强AVM的严重性。要增加ECEng删除,我们使用了密码子改进的cre(icre),与野生型(WT)cre相比,在Floxed等位基因的重组中更有效。R26RCreER;Engf/f小鼠具有Rosa启动子驱动和他莫昔芬(TM)诱导的WTcre全球表达,和PdgfbiCreER;用三次腹膜内注射TM(2.5mg/25g体重)治疗在EC中具有Pdgfb启动子驱动和TM诱导型icre表达的Engf/f小鼠,以在全局或EC中删除Eng。将AAV-VEGF立体定向注射到脑中以诱导脑局灶性血管生成和脑AVM。我们发现icre在大脑中导致更多的Eng缺失,与具有WTcre的小鼠相比,Eng蛋白水平较低(p<0.001)和Eng阳性EC较少(p=0.01)表明。icre介导的Eng缺失的小鼠有更多的异常血管(p=0.02),CD68+巨噬细胞(p=0.002),与WTcre小鼠相比,出血(p=0.04)和血管周细胞和平滑肌覆盖率较低。此外,在icre小鼠的肠道中检测到动静脉分流,之前在WTcre小鼠中未检测到的表型。RNA-seq分析显示,通过基因本体论(GO)分析鉴定的10个最高上调途径中的8个与炎症相关。因此,ECs中Eng删除的增加加剧了AVM的严重程度,这与炎症增强有关。可以减少Eng阴性ECs的策略可用于开发新的疗法以降低HHT1患者的AVM严重程度。
    Endoglin (ENG) mutation causes type 1 hereditary hemorrhagic telangiectasia (HHT1). HHT1 patients have arteriovenous malformations (AVMs) in multiple organs, including the brain. In mice, Eng deletion induced by R26RCreER or SM22αCre leads to AVM development in the brain and other organs. We hypothesized that an increase in Eng- negative ECs will enhance AVM severity. To increase EC Eng deletion, we used a codon-improved cre (icre), which is more potent in recombination of the floxed alleles than the wild-type (WT) cre. R26RCreER;Engf/f mice that have a Rosa promoter driving and tamoxifen (TM)-inducible WT cre expression globally, and PdgfbiCreER;Engf/f mice that have a Pdgfb promoter driving and TM-inducible icre expression in ECs were treated with three intra-peritoneal injections of TM (2.5 mg/25 g of body weight) to delete Eng globally or in the ECs. AAV-VEGF was stereotactically injected into the brain to induce brain focal angiogenesis and brain AVM. We found that icre caused more Eng deletion in the brain, indicated by a lower level of Eng proteins (p < 0.001) and fewer Eng-positive ECs (p = 0.01) than mice with WT cre. Mice with icre-mediated Eng deletion have more abnormal vessels (p = 0.02), CD68+ macrophages (p = 0.002), and hemorrhage (p = 0.04) and less vascular pericyte and smooth muscle coverage than mice with WT cre. In addition, arteriovenous shunts were detected in the intestines of icre mice, a phenotype that has not been detected in WT cre mice before. RNA-seq analysis showed that 8 out of the 10 top upregulated pathways identified by gene ontology (GO) analysis are related to inflammation. Therefore, the increase in Eng deletion in ECs exacerbates AVM severity, which is associated with enhanced inflammation. Strategies that can reduce Eng-negative ECs could be used to develop new therapies to reduce AVM severity for HHT1 patients.
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  • 文章类型: Case Reports
    肠系膜血管中的动静脉畸形(AVM)极为罕见。这些先天性血管异常导致高压动脉系统和低压静脉系统之间的直接血管流动。我们描述了一例先前进行左结肠切除术的脾弯曲结肠腺癌患者,在发生多发性肠系膜血栓形成后表现出持续性腹痛。CT和结肠镜显示左半结肠充血,吻合口狭窄和粘膜水肿。肠系膜血管造影显示右绞痛和左绞痛动脉中的AVM。左绞痛AVM的栓塞导致症状缓解,而在间隔随访中没有复发。
    Arteriovenous malformations (AVMs) in mesenteric vessels are exceptionally rare. These congenital vascular anomalies lead to direct vascular flow between the highly pressured arterial system and the low-pressure venous system. We describe the case of a patient with prior left colectomy for splenic flexure colonic adenocarcinoma presenting with persistent abdominal pain after developing multiple mesenteric thromboses. CT and colonoscopy showed left hemicolon congestion, anastomotic stenosis and mucosal oedema. Mesenteric angiogram revealed AVMs in the right colic and left colic arteries. Embolisation of the left colic AVM led to symptom resolution without recurrence at interval follow-up.
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  • 文章类型: Journal Article
    目的:术前血管内栓塞术是脑动静脉畸形(AVM)外科治疗中广泛使用的辅助手段。然而,这是否提高了AVM切除的完整性是未知的,因为以前的分析没有针对潜在的混杂因素进行调整。我们旨在确定术前血管内栓塞是否与首次手术时完全AVM切除率的增加有关。以下是Spetzler-Martin等级项目的调整。
    方法:我们确定了2004年6月至2022年6月期间在苏格兰NHS洛锡安卫生委员会地区的一个专业神经科学部门接受首次AVM切除术的所有患者的队列。前瞻性地从医疗记录中提取数据。我们的主要结果是AVM切除的完整性。我们使用二项逻辑回归对Spetzler-Martin分级系统项目进行调整,确定了完整AVM切除的几率:最大病灶直径,邻近大脑的口才和深静脉引流的存在。
    结果:88例患者(中位年龄40岁[IQR19-53],55%男性)行AVM切除。34/88(39%)的患者进行了术前栓塞,并在74/88(84%)的首次手术中实现了完全切除。术前栓塞与AVM完全切除的校正几率增加相关(校正比值比[aOR]8.6[95%置信区间(95%CI)1.7-67.7];p=0.017)。深静脉引流的存在与AVM完全切除的机会减少相关(aOR0.18[95%CI0.04-0.63];p=0.009)。
    结论:术前栓塞与Spetzler-Martin分级调整后AVM完全切除的机会增加相关,因此,在计划手术切除AVM时应予以考虑。
    OBJECTIVE: Preoperative endovascular embolisation is a widely used adjunct for the surgical treatment of brain arteriovenous malformations (AVMs). However, whether this improves completeness of AVM resection is unknown, as previous analyses have not adjusted for potential confounding factors. We aimed to determine if preoperative endovascular embolisation was associated with increased rate of complete AVM resection at first surgery, following adjustment for Spetzler-Martin grade items.
    METHODS: We identified a cohort of all patients undergoing first ever AVM resection in a specialist neurosciences unit in the NHS Lothian Health Board region of Scotland between June 2004 and June 2022. Data was prospectively extracted from medical records. Our primary outcome was completeness of AVM resection. We determined the odds of complete AVM resection using binomial logistic regression with adjustment for Spetzler-Martin grading system items: maximum nidus diameter, eloquence of adjacent brain and the presence of deep venous drainage.
    RESULTS: 88 patients (median age 40y [IQR 19-53], 55% male) underwent AVM resection. 34/88 (39%) patients underwent preoperative embolisation and complete resection was achieved at first surgery in 74/88 (84%). Preoperative embolisation was associated with increased adjusted odds of complete AVM resection (adjusted odds ratio [aOR] 8.6 [95% confidence interval (95% CI) 1.7-67.7]; p = 0.017). The presence of deep venous drainage was associated with reduced chance of complete AVM resection (aOR 0.18 [95% CI 0.04-0.63]; p = 0.009).
    CONCLUSIONS: Preoperative embolisation is associated with improved chances of complete AVM resection following adjustment for Spetzler-Martin grade, and should therefore be considered when planning surgical resection of AVMs.
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  • 文章类型: Case Reports
    背景:目前,复杂的先天性动静脉畸形(AVM)的治疗结果仍不能令人满意.本文报道了腹主动脉支架移植物的应用,结合栓塞技术,用于治疗由下肢复杂的先天性动静脉畸形引发的急性心力衰竭。
    方法:我们介绍一例下肢先天性AVM患者,他的左下肢长期肿胀,最近出现了心力衰竭的症状。在67岁时,患者被明确诊断为下肢复杂的先天性AVM。本文深入研究了使用腹主动脉支架移植物的实践经验和局限性。再加上栓塞,解决下肢复杂先天性AVM引起的急性心力衰竭。
    结论:我们的文章介绍了在治疗由下肢复杂先天性AVM引发的急性心力衰竭时遇到的挑战和局限性的初步报告,利用腹主动脉支架植入术和栓塞技术的组合。
    BACKGROUND: Currently, the treatment outcomes for complex congenital arteriovenous malformations (AVMs) remain unsatisfactory. This article reports on the utilization of an abdominal aortic stent graft, in conjunction with embolization techniques, for managing acute heart failure triggered by complex congenital arteriovenous malformations in the lower limb.
    METHODS: We present a case involving a patient with congenital AVMs in the lower limb, who had suffered from prolonged swelling in the left lower limb and recently developed symptoms of heart failure. At the age of 67, the patient was definitively diagnosed with a complex congenital AVMs in the lower limb. This article delves into the practical experiences and limitations encountered in employing an abdominal aortic stent graft, coupled with embolization, to address acute heart failure caused by complex congenital AVMs in the lower limb.
    CONCLUSIONS: Our article presents the initial report on the challenges and limitations encountered in treating acute heart failure triggered by complex congenital AVMs in the lower limb, utilizing a combination of abdominal aortic stent graft placement and embolization techniques.
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  • 文章类型: Case Reports
    一个十几岁的男孩在6周内间歇性地表现出右眼和鼻孔的出血逐渐增加。完整的眼科检查没有发现任何意义。他的耳湿性检查和血液学检查在正常范围内。一个月后,病人来了,右眼流血。眼科检查显示右下睑结膜充血和浸渍。对该部位结膜刮片的组织病理学检查显示血管异常扩张,提示血管畸形。数字减影血管造影证实了由颈外动脉和眼动脉分支提供的结膜微动静脉畸形的存在。他经历了成功的经动脉Onyx栓塞术,从而完全消除了血液阻塞。
    A boy in his mid-teens presented with progressively increasing bleeding from the right eye and nostril intermittently over a period of 6 weeks. A complete ophthalmic examination revealed nothing significant. His otorhinological examination and haematological investigations were within normal limits. The patient came a month later with frank bleeding from the right eye. Ophthalmic examination revealed hyperaemia and maceration of the right lower palpebral conjunctiva. A histopathological examination of conjunctival scrapings from the site showed abnormal dilated blood vessels suggestive of a vascular malformation. Digital subtraction angiography confirmed the presence of a conjunctival micro arteriovenous malformation supplied by the external carotid and ophthalmic artery branches. He underwent successful transarterial Onyx embolisation resulting in complete resolution of the haemolacria.
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