vascular malformations

血管畸形
  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    背景技术肌疝是通常在四肢中发现的罕见病症;咬肌疝甚至更罕见。然而,临床医生和放射科医师意识到这种可能性是很重要的.肌内静脉畸形(IMVM)也不常见,主要见于头部,脖子,和四肢。这两种情况同时存在是非常罕见的,and,根据我们的知识,这在咬肌之前没有报道过。由于它们的稀有性,含糊的陈述,和不准确的临床诊断,需要进行放射学评估以避免不适当的手术计划.病例报告一个12岁的男孩有一个长期的局灶性左脸颊肿胀,牙齿咬紧加剧。侧位X线显示左下颌区域有圆形钙化。超声提示左侧咬肌粗大,局灶性结构不均匀,2个圆形钙化灶。在咬牙期间,超声检查通过左咬肌筋膜发现局灶性肌肉疝,随着休息而减少。患者被诊断为左侧咬肌疝与IMVM共存。进行了IMVM的手术切除,疝缺损被修复了.组织病理学证实了诊断,经短期随访,患者出院,无术后并发症。结论尽管它们很少,在任何咬肌病变的鉴别诊断中,应考虑咬肌疝和IMVM。尤其是儿童。我们报道了两种病理的非常罕见的共存。综合诊断可通过结合临床检查,X光片,和超声评估。
    BACKGROUND Muscle hernias are an uncommon condition typically found in the extremities; masseter muscle hernia is even rarer. However, it is important for clinicians and radiologists to be aware of this possibility. Intramuscular venous malformation (IMVM) is also uncommon and mostly found in the head, neck, and extremities. The simultaneous presence of both conditions is extraordinary uncommon, and, to our knowledge, this has not been reported before in the masseter muscle. Due to their rarity, vague presentation, and inaccurate clinical diagnosis, radiological evaluation is needed to avoid inappropriate surgical planning. CASE REPORT A 12-year-old boy had a long-standing focal left cheek swelling exacerbated by teeth clenching. Lateral X-ray revealed a round calcification over the left mandibular region. Ultrasonography indicated a bulky left masseter muscle with focal heterogeneous structure and 2 rounded calcified foci. During teeth clenching, ultrasonography detected focal muscular herniation through the left masseter muscle facia that reduced with rest. The patient was diagnosed with left masseteric muscle hernia coexistent with IMVM. Surgical excision of the IMVM was performed, and the hernia defect was repaired. Histopathology confirmed the diagnosis, and the patient was discharged without postoperative complications on short-term follow-up. CONCLUSIONS Despite their rarity, masseter hernias and IMVMs should be considered in the differential diagnosis of any masseter lesion, especially in children. We reported a very rare coexistence of both pathologies. Comprehensive diagnosis can be achieved through a combination of clinical examination, X-ray, and ultrasound assessments.
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  • 文章类型: Case Reports
    背景:血淋巴管瘤是一种罕见的,起源于血管和淋巴畸形的非侵袭性良性肿瘤。它通常是先天性的,并且可以根据其位置和大小而表现出不同的症状。很少有关于椎管内血淋巴管瘤的报道,巨大的病变非常罕见。
    方法:2023年7月,滨州市人民医院脊柱外科收治1例64岁男性胸11-腰2型巨大椎管内硬膜外血管瘤(T11-L2),中国。患者出现进行性腰椎和左下肢疼痛,麻木,双下肢无力,排尿和排便困难。影像学研究显示,T11-L2的椎管内有大量囊性肿块。进行了手术减压,病理证实为血淋巴管瘤。
    结论:完全切除血淋巴管瘤预后最好,最终诊断依赖于病理诊断。在这种情况下,用椎弓根钉棒系统完整地去除肿块,导致充分的脊柱减压和脊柱稳定性的恢复。
    BACKGROUND: Hemolymphangioma is a rare, noninvasive benign tumor that originates from vascular and lymphatic malformations. It is usually congenital and can present with varying symptoms depending on its location and size. There are very few reports of hemolymphangiomas within the spinal canal, and giant lesions are exceptionally rare.
    METHODS: In July 2023, a 64-year-old male with a giant intravertebral epidural hemolymphangioma from thoracic 11 to lumbar 2 (T11-L2) was admitted to the Department of Spine Surgery at the People\'s Hospital of Binzhou City, China. The patient experienced progressive lumbar and left lower limb pain, numbness, weakness in both lower limbs, and difficulty with urination and defecation. Imaging studies revealed a large cystic mass in the spinal canal at T11-L2. Surgical decompression was performed, and the pathology confirmed hemolymphangioma.
    CONCLUSIONS: Complete resection of hemolymphangiomas has the best prognosis, and final diagnosis relies on pathologic diagnosis. In this case, the mass was removed intact with a pedicle nail rod system, leading to adequate spinal decompression and restoration of spinal stability.
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  • 文章类型: Case Reports
    颈内动脉(ICA)的发育不全或发育不全很容易与夹层或闭塞相混淆。我们报告了一例24岁的女性,主诉急性左手感觉减退,并且偶尔有右手间歇性麻木并伴有肌阵挛性抽搐的病史。因为过去2年的影像学研究被解释为继发于颈动脉夹层的左侧ICA闭塞,主治医生开了抗凝治疗.经颅多普勒(TCD)检查期间,频谱波形出乎意料地正常,由于TCD结果,提示重复检查所有影像学检查。磁共振血管造影(MRA)显示ICA起源具有相同的“火焰状”外观。后期数字减影血管造影显示小口径颈部ICA(颅底闭塞)。计算机断层扫描显示没有颈动脉,确认ICA的颅内部分缺失,并正确诊断左侧颈内动脉发育不全。血管超声和TCD检查是非侵入性和廉价的工具,可以提高对其他“静态”影像学检查(MRA,数字减影血管造影)。准确的诊断对于避免风险至关重要,积极治疗,如“无”夹层的抗凝治疗。
    Agenesis or hypoplasia of the internal carotid artery (ICA) may easily be confused with dissection or occlusion. We report a case of a 24-year-old female with complaint of acute left-hand hypoesthesia and a history of occasional intermittent numbness of her right hand with myoclonic jerking. Because previous imaging studies over 2 years were interpreted as occlusion of the left ICA secondary to carotid dissection, the treating physician had prescribed anticoagulant therapy. During transcranial Doppler (TCD) examination, the spectral waveform was unexpectedly normal, prompting a repeat review of all imaging due to the TCD results. Magnetic resonance angiography (MRA) revealed the same \"flame-like\" appearance of the ICA origin. Late-phase digital subtraction angiography showed a small caliber cervical ICA (occluded at the skull base). Computed tomography demonstrated absence of the carotid canal, confirming an absent intracranial portion of the ICA and establishing a correct diagnosis of left internal carotid hypoplasia. Vascular ultrasound and TCD examinations are noninvasive and inexpensive tools that can improve the interpretation and understanding of the clinical significance of other \"static\" radiographic tests (MRA, digital subtraction angiography ). An accurate diagnosis is essential to avoid risky, aggressive treatment, such as anticoagulation for an \"absent\" dissection.
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  • 文章类型: Journal Article
    BACKGROUND: Vascular malformations (VaMs) are caused by errors in vascular morphogenesis. Diagnosis and treatment can be complex. Few specialized centers care for these patients, and limited literature exists regarding their characteristics and clinical course. The vascular anomalies clinic (VAC) at the Instituto Nacional de Pediatría (National Institute for Pediatrics) is a multidisciplinary team and has been a reference center for patients with VaMs since 2012. We sought to describe the characteristics of patients cared for at the VAC, types of VaMs, treatments used, and clinical course.
    METHODS: This was a descriptive, observational, retrospective, and cross-sectional study conducted from 2012 to 2022.
    RESULTS: We included 435 patients with VaMs; the median age of presentation was 1 month. The most frequent signs and symptoms were increased volume (97.2%), superficial color change (65.5%), and pain (43.3%). The most common VaMs were lymphatic (36.7%) and venolymphatic (18.3%). Sclerotherapy was the most frequent treatment (73.4%), followed by medical treatment with sirolimus (18.5%); response to both was excellent/good in > 85% of cases.
    CONCLUSIONS: In this retrospective study of children with VaMs, we found that low-flow malformations were the most common, and sclerotherapy and sirolimus were the most frequently used treatments. The therapeutic response was excellent/good in most cases.
    UNASSIGNED: Las malformaciones vasculares (MaV) son secundarias a errores en la morfogénesis vascular. El diagnóstico y tratamiento puede ser complejo. Existen pocos centros especializados en su atención y escasa literatura respecto a características y evolución clínica. La Clínica de Anomalías Vasculares (CAV) del Instituto Nacional de Pediatría es un equipo multidisciplinario y centro de referencia para estos pacientes desde 2012. Buscamos describir las características de los pacientes atendidos en la CAV, tipo de MaV, tratamiento y evolución clínica.
    UNASSIGNED: Estudio descriptivo, observacional, retrospectivo y transversal del periodo 2012 al 2022.
    RESULTS: Se incluyeron 435 pacientes con MaV, con edad mediana de presentación de 1 mes de vida. Los síntomas y signos más reportados fueron aumento de volumen (97.2%), cambio en coloración de la piel (65.5%) y dolor (43.3%). Las MaV más comunes fueron linfáticas (36.7%), siguiéndoles las venolinfáticas (18.3%). La escleroterapia fue el tratamiento más frecuente (73.4%) y el tratamiento médico más utilizado fue sirolimus (18.5%), ambos con excelente/buena respuesta en > 85% de los pacientes.
    CONCLUSIONS: En este estudio retrospectivo de niños con MaV encontramos que las más frecuentes son de bajo flujo y el tratamiento más usado escleroterapia y sirolimus. La respuesta terapéutica de la mayoría fue excelente/buena.
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  • 文章类型: Journal Article
    经常咨询小儿皮肤科医生以评估儿童的全身性疾病的皮肤体征。在皮肤病学文献中已经描述了各种皮肤发现的数值阈值,其中相关的皮肤外异常或已知的遗传综合征的可能性显着增加。了解这些数值阈值有助于诊断和管理,这改善了临床结果并避免了严重的并发症。这篇综述强调了临床表现,并发症,评估,和数值意义,适用时,对于以下皮肤发现:婴儿血管瘤,毛细血管畸形,Café-au-lait斑斑,低色素斑,幼年黄色肉芽肿,毛囊瘤,还有血管纤维瘤.
    Pediatric dermatologists are frequently consulted to evaluate children for cutaneous signs of systemic disorders. Numerical thresholds of significance have been described in the dermatologic literature for various skin findings where the likelihood of an associated extracutaneous abnormality or known genetic syndrome increases significantly. Knowledge of these numerical thresholds facilitates diagnosis and management, which improves clinical outcomes and avoids severe complications. This review highlights the clinical presentation, complications, evaluation, and numerical significance, when applicable, for the following skin findings: infantile hemangiomas, capillary malformations, café-au-lait macules, hypopigmented macules, juvenile xanthogranulomas, pilomatricomas, and angiofibromas.
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  • 文章类型: Journal Article
    动静脉畸形是复杂且具有挑战性的实体,他们的治疗通常是针对患者量身定制的。使用博来霉素进行间质性硬化治疗是有希望的,尤其是在根据SECg分类的S3AVM中,手术干预的美学和/或功能负担会产生影响的情况下。我们对15例出现小(<10cm3)S3动静脉畸形的患者进行了33次15.000IU间质性博来霉素治疗。9名患者对前3个疗程表现出中等至良好的反应,因此在两个周期之间进行了手术重塑。美国检查是在治疗前完成的,在每次治疗前和治疗方案结束后每3个月评估动静脉畸形。17名患者中有15名结束了协议。9例患者接受了手术重新塑形,而6例患者由于对前3次博来霉素注射的良好反应而跳过了手术。外观和症状方面的重大改善(搏动,溃疡,出血或疼痛)总是实现的。有20%的轻微并发症。所提供的数据令人鼓舞,似乎表明这可能是一个有希望的方案,可以扩大博来霉素在动静脉畸形中的使用并减少手术的影响。
    Arteriovenous Malformations are complex and challenging entities, and their treatment is often tailored on the patient. Interstitial sclerosing treatment with bleomycin is promising especially in cases where the aesthetical and/or functional burden of surgical intervention would be impactful as in the S3 AVM according to the SECg classification. We treated 15 patients presenting small (<10cm3) S3 arterio-venous malformations with 3 + 3 sessions of 15.000 IU of interstitial bleomycin. 9 patients presented moderate to good response to the first 3 sessions and thus surgical re-shaping was performed between the two cycles. US examination was done pre-treatment, before each session and every 3 months after the end of the protocol to assess the arterio-venous malformations. Fifteen of the seventeen patients concluded the protocol. 9 patients underwent surgical re-shaping while 6 skipped it because of their excellent response to the first 3 bleomycin injections. Major improvement in terms of appearance and symptoms (pulsations, ulcerations, bleeding or pain) were always achieved. There was a 20% of minor complications. The presented data are encouraging and seem to suggest that this may be a promising protocol to extend the use of bleomycin in arterio-venous malformations and reduce the impact of surgery.
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  • 文章类型: Journal Article
    Klippel-Trenaunay综合征(KTS)是一种罕见的先天性血管疾病,其特征是广泛的毛细血管和静脉畸形,在怀孕期间构成独特的挑战。本病例报告讨论了一名34岁的KTS孕妇两次剖腹产的成功治疗,导致两个健康的婴儿出生。尽管KTS缺乏基于证据的产科管理指南,一个多学科小组合作制定了高危血栓形成管理计划,涉及使用压缩袜和低分子量肝素预防。患者血栓形成的风险升高,在怀孕期间加剧,告知剖腹产的决定,与发现在大多数KTS怀孕中,根据产科适应症和动静脉畸形选择这种分娩方式.这个案例突出了系统和以病人为中心的护理的重要性,倡导全面的产科管理指南,以应对怀孕期间KTS带来的独特挑战。有必要进行进一步的研究,以增强我们对与KTS相关的血管异常个体的理解和完善指南。
    Klippel-Trenaunay Syndrome (KTS) is a rare congenital vascular disorder characterized by extensive capillary and venous malformations that pose unique challenges during pregnancy. This case report discusses the successful management of a 34-year-old pregnant woman with KTS who had two caesarean sections, resulting in the birth of two healthy babies. Despite the lack of evidence-based guidelines for obstetrical management in KTS, a multidisciplinary team collaborated to devise a high-risk thrombosis management plan, involving the use of compression stocking and low molecular weight heparin prophylaxis. The patient\'s elevated risk of thrombosis, exacerbated during pregnancy, informed the decision of caesarean sections, aligning with finding that in most KTS pregnancies, this method of delivery based on obstetric indications and arteriovenous malformations is chosen. This case highlights the importance of systematic and patient-centered care, advocating for comprehensive obstetric management guidelines to address the unique challenges posed by KTS during pregnancy. Further research is warranted to enhance our understanding and refine guidelines for individuals with vascular abnormalities linked to KTS.
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  • 文章类型: Case Reports
    肺静脉通常流入左心房,上肺静脉通常位于右肺动脉的前方和下方。然而,异常情况可能会发生。对于一位表现为非典型胸痛的患者,我们遇到了极为罕见的肺血管异常,右上肺静脉在右肺动脉(RPA)后方异常延伸,并在RPA和右主支气管之间被压缩。冠状动脉计算机断层扫描血管造影可识别出这种特定的肺静脉异常,但未发现冠状动脉异常。
    The pulmonary veins normally drain into the left atrium, with the superior pulmonary veins typically situated anterior and inferior to the right pulmonary arteries. However, anomalies can happen. We encountered an exceedingly rare pulmonary vascular anomaly for a patient presenting with atypical chest pain, where the right superior pulmonary vein aberrantly ran posterior to the right pulmonary artery (RPA) and became compressed between the RPA and the right main bronchus. Coronary computed tomography angiography identified this specific pulmonary vein anomaly but revealed unremarkable coronary arteries.
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  • 文章类型: Case Reports
    背景:纵隔静脉畸形(MVM)和梭形上腔静脉动脉瘤(F-SVCA)都是罕见的先天性血管畸形。
    方法:一名46岁男性,表现为病因不明的急性缺血性卒中。计算机断层扫描(CT)血管造影显示MVM和F-SVCA共存。诊断性静脉造影显示F-SVCA内血流速度显著降低,但未能确定与左心系统或肺静脉的直接连接。患者因中风引起的广泛脑损伤而死亡。
    结论:这种情况可能会增加对这些异常进行细致的放射学评估和预防性管理的必要性,纵隔血管异常可导致血栓栓塞并发症。
    BACKGROUND: Mediastinal venous malformation (MVM) and fusiform superior vena cava aneurysm (F-SVCA) are both rare congenital vascular anomalies.
    METHODS: A 46-year-old male presented with acute ischemic stroke of unknown etiology. Computed tomography (CT) angiography revealed the coexistence of MVM and F-SVCA. Diagnostic venography demonstrated a significant reduction in blood flow velocity within the F-SVCA, but failed to identify a direct connection to the left heart system or pulmonary vein. The patient expired due to extensive brain damage caused by a stroke.
    CONCLUSIONS: This case may increase the necessity of meticulous radiological evaluation and preventive management for these anomalies, as mediastinal vascular anomalies can result in thromboembolic complications.
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