arteriovenous malformations

动静脉畸形
  • 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: 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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  • 文章类型: Case Reports
    获得性血友病A是一种罕见但严重的自身免疫性出血性疾病,由针对凝血因子VIII(FVIII)的自身抗体引起。将获得性血友病与其他更常见的出血原因区分开来,比如慢性肝病,弥散性血管内凝血,或脓毒症诱导的凝血功能障碍,对于指导危及生命的出血的管理至关重要。这项研究描述了一名原发性胆汁性胆管炎患者,他被发现患有A型血友病,这是危及生命的出血的独特原因,对于诊断和治疗潜在的肝脏疾病尤其具有挑战性。确定获得性血友病A允许了否则无法获得的治疗选择的途径。
    Acquired hemophilia A is a rare but severe autoimmune bleeding disorder that results from autoantibodies against clotting factor VIII (FVIII). Distinguishing acquired hemophilia from other more common causes of bleeding, such as chronic liver disease, disseminated intravascular coagulation, or sepsis-induced coagulopathies, is crucial in guiding the management of life-threatening hemorrhage. This study describes a patient with primary biliary cholangitis who was found to have acquired hemophilia A, a unique cause of life-threatening bleeding that was especially challenging to diagnose and manage with her underlying liver disease. Identifying acquired hemophilia A allowed an avenue of treatment options that would not have otherwise been available.
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  • 文章类型: Case Reports
    背景:肺动静脉畸形是一种相对罕见的医学疾病,影响大约每2500个人中的1人。在患有肺动静脉畸形的人中,80%有潜在的遗传病:遗传性出血性毛细血管扩张症。
    方法:我们介绍了一名20岁的巴基斯坦男性,他有持续性慢性发作性额叶头痛的病史,在一天的过程中严重程度增加。他的血红蛋白是18g/dl,表明红细胞增多症,为此,他在一个月内接受了七次手术。他的身体检查平淡无奇。他的计算机断层扫描扫描描绘了多个扩张的曲折血管,在肺的右下叶具有分支的线性混浊。多条供血动脉由右主肺动脉供血,大的引流静脉通向右下肺静脉。这被确定为弥漫性肺动静脉畸形。建议他做右肺动脉血管造影。它显示出多个曲折的血管,有一个鼻孔和大的引流静脉-在肺的右下叶具有弥漫性动静脉畸形的特征,与计算机断层扫描扫描一致。对供应动静脉畸形的这些血管中的两个进行了栓塞,使用Amplatzer血管塞2,而多个可推压线圈(五个线圈)用于栓塞第三个喂食血管。这实现了70-80%的成功栓塞右肺AVM;然而,考虑到病变的复杂性,在动静脉畸形中仍然可以看到一些残余血流。紧接着,他的氧饱和度从78%提高到96%.
    结论:弥漫性肺动静脉畸形,正如在这个病人身上看到的,是罕见的,占诊断的肺动静脉畸形总数的不到5%。病人主诉进行性额叶头痛,这可以归因于低氧饱和度或脑动静脉畸形的存在。患者家属无遗传性出血性毛细血管扩张症病史。此外,尽管大多数遗传性出血性毛细血管扩张症和肺动静脉畸形的患者都有缺铁性贫血的主诉,相反,我们的患者患有红细胞增多症。这可以解释为低氧血症的补偿机制。此外,患者没有咯血或鼻出血的主诉,与典型的肺动静脉畸形相比,表现多种多样。
    BACKGROUND: Pulmonary arteriovenous malformations are a relatively uncommon medical condition, affecting roughly 1 in every 2500 individuals. Of those suffering from pulmonary arteriovenous malformations, 80% have an underlying genetic condition: hereditary hemorrhagic telangiectasia.
    METHODS: We present the case of a 20-year-old Pakistani male with a history of persistent slower-onset frontal headaches that increased in severity within the course of the day. His hemoglobin was 18 g/dl, indicating polycythemia, for which he had undergone seven venesections in a month previously. His physical examination was unremarkable. His computed tomography scan depicted multiple dilated tortuous vessels with branching linear opacities in the right lower lobe of the lungs. The multiple feeding arteries were supplied by the right main pulmonary artery, and the large draining veins led to the right inferior pulmonary vein. This was identified as a diffuse pulmonary arteriovenous malformation. He was recommended for a right pulmonary artery angiogram. It showed multiple tortuous vessels with a nidus and large draining veins-features of a diffuse arteriovenous malformation in the right lower lobe of the lung consistent with the computed tomography scan. Embolization of two of these vessels feeding the arteriovenous malformation was conducted, using Amplatzer Vascular plug 2, whereas multiple pushable coils (five coils) were used for embolizing the third feeding vessel. This achieved 70-80% successful embolization of right pulmonary AVM; however, some residual flow was still seen in the arteriovenous malformation given the complexity of the lesion. Immediately after, his oxygen saturation improved from 78% to 96%.
    CONCLUSIONS: Diffuse pulmonary arteriovenous malformations, as seen in this patient, are rare, accounting for less than 5% of total pulmonary arteriovenous malformations diagnosed. The patient presented with a complaint of progressive frontal headaches, which can be attributed to low oxygen saturation or the presence of a cerebral arteriovenous malformation. There was no history of hereditary hemorrhagic telangiectasia in the patient\'s family. Furthermore, although most patients with hereditary hemorrhagic telangiectasia and hence pulmonary arteriovenous malformation have complaints of iron-deficiency anemia, our patient in contrast was suffering from polycythemia. This can be explained as a compensatory mechanism in hypoxemic conditions. Moreover, the patient had no complaint of hemoptysis or epistaxis, giving a varied presentation in comparison with a typical pulmonary arteriovenous malformation.
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  • 文章类型: Case Reports
    冠状动脉动静脉瘘(CAVFs)是冠状动脉和冠状静脉系统之间的先天性或获得性通信,它们还可以包括其他心脏结构或脉管系统。我们讨论了一例有胃肠道动静脉畸形(AVM)病史的老年患者的左冠状动脉主干和右心房之间的大瘘。CAVFs的出现,罕见的心脏不规则,在老年人中尤其罕见。通常,它是在调查呼吸急促或胸痛等症状时偶然发现的,其中冠状动脉造影是必要的,以确定最有效的治疗策略。该病例强调了在有胃肠道AVM病史的患者中评估CAVF的可能实用性,这些患者类似地表现出高输出心力衰竭的临床症状。一旦确定,这可以简化治疗方法并改善医疗保健提供者之间的沟通,以最大程度地减少对患者造成伤害的风险。
    Coronary arteriovenous fistulas (CAVFs) are congenital or acquired communications between the coronary arteries and coronary venous system, and they can also include other cardiac structures or vasculature. We discuss a case of a large fistula between the left main coronary artery and the right atrium in a geriatric patient with a history of gastrointestinal arteriovenous malformations (AVM). The occurrence of CAVFs, an uncommon cardiac irregularity, is particularly infrequent among older adults. Typically, it is discovered by chance when investigating symptoms such as shortness of breath or chest pain, where coronary angiography is necessary to determine the most effective treatment strategy. This case highlights the possible utility of evaluating CAVFs in patients with a history of gastrointestinal AVM who similarly present with clinical symptoms of high-output heart failure. Once identified, this could simplify the treatment approach and improve communication between healthcare providers to minimize the risk of harm to the patient.
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  • 文章类型: Case Reports
    动静脉畸形(AVM)是脉管系统的异常连接,导致毛细血管床旁路并导致神经系统恶化和出血的高风险。颈脊髓中的髓内AVM很少见,需要精确的诊断和治疗。我们介绍了一名28岁的白人女性复发性AVM的临床病例,患有突然和严重的颈部疼痛和各种神经系统症状,以及目前的诊断和治疗方式。保守治疗部分有效。MRI和DSA证实了C4水平的AVM,随后在15岁和24岁时进行了几次血管内治疗,神经系统得到了轻度改善。之后,患者接受了康复治疗,神经系统略有改善。该病例突出了AVM的临床进展和治疗,以及当前的病理生理学,分类,和成像。
    Arteriovenous malformation (AVM) is an abnormal connection of vasculature resulting in capillary bed bypassing and leading to neurological deterioration and high risk of bleeding. Intramedullary AVMs in the cervical spinal cord are rare and require precise diagnostics and treatment. We present a clinical case of recurrent AVMs in a 28-year-old Caucasian female with sudden and severe neck pain and variable neurological symptoms along with current diagnostic and treatment modalities. Conservative treatment was partially effective. MRI and DSA confirmed AVMs at C4 level with subsequent several endovascular treatment sessions at the age of 15 and 24 with mild neurological improvement. Afterwards the patient underwent rehabilitation with minor neurological improvement. This case highlights the clinical progression and treatment of AVMs along with showcasing current pathophysiology, classification, and imaging.
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  • 文章类型: Case Reports
    背景:肺动静脉畸形(PAVM),也被称为肺动静脉瘘,是一种罕见的血管发育异常。大多数PAVM病例与遗传性出血性毛细血管扩张症(HHT)有关。与PAVM相关的血胸甚至更罕见,有关这一并发症的管理仍然面临挑战。
    方法:我院收治一名突然出现呼吸困难和胸痛的55岁男性患者。他有鼻出血病史,腹膜内生殖细胞肿瘤和PAVM。胸部未增强CT显示左侧胸腔积液伴部分被动肺不张,间隔6天逐渐增加。诊断性胸腔穿刺术进一步显示出血性积液。CT血管造影(CTA)显示左下肺动脉和PAVM的管腔弯曲扩张,并形成动脉瘤。由于他的家人拒绝手术,患者接受了经导管栓塞治疗.然而,左胸腔积液没有明显减少,即使在介入治疗后血红蛋白值也缓慢下降,表明持续活动性出血的可能性。最终,患者接受了左下叶肺叶切除术,结果令人满意。
    结论:PAVM破裂进入胸膜腔引起的大量血胸可导致致命的结果。CTA可以准确诊断这种病理状况。经导管栓塞术常用于治疗PAVM,但在血胸患者中达到理想的效果可能是具有挑战性的。结合我们的案例和文献回顾,当PAVM并发血胸和大直径的引流静脉时,直接根治性手术可以导致成功的结果。
    BACKGROUND: Pulmonary arteriovenous malformation (PAVM), also known as pulmonary arteriovenous fistula, is a rare vascular developmental anomaly. Most cases of PAVM are associated with hereditary hemorrhagic telangiectasia (HHT). Hemothorax associated with PAVM is even rarer, and management concerning this complication still challenges.
    METHODS: A 55-year-old man with sudden onset of dyspnea and chest pain was admitted to our hospital. He had a medical history of epistaxis, intraperitoneal germ cell tumor and PAVM. Chest unenhanced CT revealed the left-sided pleural effusion together with partial passive atelectasis and gradual increase at the interval of six days. Diagnostic thoracocentesis further revealed hemorrhagic effusion. CT angiography (CTA) showed tortuously dilated lumen of the left lower pulmonary artery and PAVM with the formation of aneurysm. Due to his family\'s refusal of surgery, the patient underwent transcatheter embolization therapy. However, the left pleural effusion did not significantly reduce and there was a slow drop in hemoglobin value even after interventional treatment, indicating the possibility of ongoing active bleeding. Eventually, the patient received lobectomy of the left lower lobe with a satisfactory outcome.
    CONCLUSIONS: Massive hemothorax resulting from PAVM rupture into the pleural space can lead to fatal outcomes. CTA can accurately diagnose this pathologic condition. Transcatheter embolization is frequently used in the treatment of PAVM, but it may be challenging to achieve the desirable effect in patients with hemothorax. Combined with our case and literature review, direct radical surgery can lead to a successful outcome when PAVM complicated with hemothorax and a large diameter of the draining vein.
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  • 文章类型: Case Reports
    肺动静脉畸形(PAVM)是导致肺动脉和静脉之间异常连接的血管异常。在80%的案例中,PAVM从出生就存在,但临床表现在儿童时期很少见。这些先天性畸形通常与遗传性出血性毛细血管扩张症(HHT)有关,一种罕见的疾病,影响5000/8000人中的1人。HHT疾病通常由参与TGF-β途径的基因突变引起。然而,大约15%的患者没有基因诊断,在基因诊断中,超过33%的人不符合库拉索岛的标准。这使得儿科年龄组的临床诊断更具挑战性。这里,我们介绍了1例8岁患者,其携带由一种未知突变引起的多重弥漫性PAVM的严重表型,该突变在肺移植结束.表型,正在研究的病例遵循类似HHT的分子模式。因此,已在从外植肺分离的原代内皮细胞(EC)中进行了分子生物学和细胞功能分析。该发现揭示了肺内皮组织的功能丧失和内皮-间质转化的刺激。了解这种转变的分子基础可能为延迟严重病例的肺移植提供新的治疗策略。
    Pulmonary arteriovenous malformations (PAVMs) are vascular anomalies resulting in abnormal connections between pulmonary arteries and veins. In 80% of cases, PAVMs are present from birth, but clinical manifestations are rarely seen in childhood. These congenital malformations are typically associated with Hereditary Hemorrhagic Telangiectasia (HHT), a rare disease that affects 1 in 5000/8000 individuals. HHT disease is frequently caused by mutations in genes involved in the TGF-β pathway. However, approximately 15% of patients do not have a genetic diagnosis and, among the genetically diagnosed, more than 33% do not meet the Curaçao criteria. This makes clinical diagnosis even more challenging in the pediatric age group. Here, we introduce an 8-year-old patient bearing a severe phenotype of multiple diffuse PAVMs caused by an unknown mutation which ended in lung transplantation. Phenotypically, the case under study follows a molecular pattern which is HHT-like. Therefore, molecular- biological and cellular-functional analyses have been performed in primary endothelial cells (ECs) isolated from the explanted lung. The findings revealed a loss of functionality in lung endothelial tissue and a stimulation of endothelial-to-mesenchymal transition. Understanding the molecular basis of this transition could potentially offer new therapeutic strategies to delay lung transplantation in severe cases.
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  • 文章类型: Case Reports
    源自颈外静脉壁的血管畸形极为罕见。我们提出了一个由颈外静脉引起的静脉畸形的独特病例,通过手术切除成功治疗,无复发。此病例强调了早期诊断和及时干预在管理此类罕见临床实体而没有任何发病率的重要性。
    Vascular malformations originating from the wall of the external jugular vein are exceedingly uncommon. We present a unique case of a venous malformation arising from the external jugular vein, successfully treated through surgical excision with no subsequent recurrence. This case highlights the importance of early diagnosis and timely intervention in managing such rare clinical entities without any resulting morbidity.
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