Nutcracker syndrome

胡桃夹综合征
  • 文章类型: Case Reports
    胡桃夹综合征是一种罕见的疾病,由左肾静脉在主动脉和肠系膜上动脉之间受压引起。尽管具有临床意义,这种综合征的诊断通常具有挑战性,经常被忽视.其主要临床表现包括血尿和骨盆或背痛。这种情况涉及左肾静脉压力升高,导致各种体征和症状,血尿是常见的表现。在这里,我们报告1例28岁女性,无病史,出现2个月血尿.腹部CT显示肠系膜上动脉与腹主动脉间左肾静脉受压,左卵巢静脉扩张和盆腔静脉曲张,符合胡桃夹综合征.
    Nutcracker syndrome is a rare condition that arises from the left renal vein getting compressed between the aorta and the superior mesenteric artery. Despite its clinical significance, this syndrome is often challenging to diagnose and is frequently overlooked. Its key clinical presentations include hematuria and pelvic or back pain. This condition involves elevated pressure on the left renal vein, leading to various signs and symptoms, with hematuria being a common manifestation. Herein, we report a 28-year-old woman with no medical history who presented with hematuria for two months. Abdominal CT revealed compression of the left renal vein between the superior mesenteric artery and abdominal aorta, with dilated left ovarian vein and pelvic varices, consistent with nutcracker syndrome.
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  • 文章类型: Case Reports
    我们报告了罕见的成年女性患者并发左胡桃夹综合征和右输尿管肾盂连接部梗阻(UPJO)的病例,该患者经CT尿路图诊断为间歇性双侧腹部疼痛。为此,我们对右侧肾盂输尿管梗阻进行了Anderson-Hynes肾盂成形术,最初是腹腔镜手术,但由于支架置入困难,改为开腹手术。她的术后课程很顺利,然后在术后第三天出院。胡桃夹综合征的症状对病人来说并不那么麻烦,所以我们计划保守地跟踪她。
    We report rare case of concomitant left nutcracker syndrome and right ureteropelvic junction obstruction (UPJO) on adult female patient diagnosed by CT urogram after she presented with intermittent bilateral flank pain. For this we did Anderson-Hynes pyeloplasty for right ureteropelvic obstruction, it was laparoscopic initially but due to difficulty of stenting it is changed to open. She had smooth post-op course then discharged on 3rd post-op day. Symptoms of nutcracker syndrome are not that much bothersome for the patient so we planned to follow her conservatively.
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  • 文章类型: Case Reports
    胡桃夹综合征(NCS)是一种罕见的影响左肾的疾病。手术治疗是治疗的唯一选择。微创手术可以是有效的,并且可以预防大手术的并发症。我们介绍了一名33岁女性患有慢性左翼疼痛的病例,诊断为NCS并接受血管外支架治疗。机器人辅助血管外支架插入使用达芬奇单端口(SP)(直观的手术,Inc.,桑尼维尔,美国)系统。这种方法提供了最小侵入性的优点,精确的支架放置,减少手术时间。据我们所知,这是使用达芬奇SP进行该适应症的第一种情况。需要进一步的研究来评估长期结果和安全性。
    Nutcracker syndrome (NCS) is a rare disease affecting the left kidney. Surgical management is the only choice of treatment. Minimal invasive surgeries can be effective and may prevent complications of the major surgery. We present the case of a 33-year-old woman suffering from chronic left flank pain, diagnosed with NCS and treated with extravascular stents. Robotic-assisted extravascular stent insertion was performed using the Da Vinci single-port (SP) (Intuitive Surgical, Inc., Sunnyvale, USA) system. This approach offers the advantages of minimal invasiveness, precise stent placement, and reduced operative time. To our knowledge, this is the first case of using Da Vinci SP for this indication. Further studies are needed to evaluate the long-term outcomes and safety.
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  • 文章类型: Journal Article
    血管压迫综合征是一组不同的病理,可以在其他健康的个体中无症状和偶然地表现出来,也可以表现出一系列症状。由于它们的相对稀有,这些综合症通常很少被理解和忽视。这些综合征的早期识别可以对随后的临床管理产生重大影响。这篇图片综述提供了七个腹部和骨盆内的血管压迫综合征的简要总结,包括正中弓状韧带(MAL)综合征。肠系膜上动脉(SMA)综合征,胡桃夹综合征(NCS),梅-瑟纳综合征(MTS),肾盂输尿管连接部梗阻(UPJO),输尿管的血管压迫,和门静脉胆道病。人口统计,病理生理学,诱发因素,并回顾了每种压迫综合征的预期治疗方法。通过使用包括超声在内的多种模态的成像示例来说明每个实体的显著成像特征。透视,CT,MRI。
    Vascular compression syndromes are a diverse group of pathologies that can manifest asymptomatically and incidentally in otherwise healthy individuals or symptomatically with a spectrum of presentations. Due to their relative rarity, these syndromes are often poorly understood and overlooked. Early identification of these syndromes can have a significant impact on subsequent clinical management. This pictorial review provides a concise summary of seven vascular compression syndromes within the abdomen and pelvis including median arcuate ligament (MAL) syndrome, superior mesenteric artery (SMA) syndrome, nutcracker syndrome (NCS), May-Thurner syndrome (MTS), ureteropelvic junction obstruction (UPJO), vascular compression of the ureter, and portal biliopathy. The demographics, pathophysiology, predisposing factors, and expected treatment for each compression syndrome are reviewed. Salient imaging features of each entity are illustrated through imaging examples using multiple modalities including ultrasound, fluoroscopy, CT, and MRI.
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  • 文章类型: Case Reports
    血管内手术后支架迁移是一种罕见但重要的并发症。管理移位支架的技术包括手术,血管内,保守的方法。该病例详述了一名患者,该患者在左肾静脉内放置了覆膜支架,该支架后来迁移到肺动脉,导致三尖瓣受损。使用透视检查和经食管超声心动图引导的经皮血管内方法成功地移除了迁移的支架。
    Stent migration is a rare but significant complication following endovascular procedures. Techniques for managing dislodged stents have included surgical, endovascular, and conservative approaches. This case details a patient who had a covered stent placed within the left renal vein which later migrated to the pulmonary artery causing damage to the tricuspid valve. The migrated stent was successfully removed using a percutaneous endovascular approach utilizing fluoroscopy and transesophageal echocardiogram guidance.
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  • 文章类型: Case Reports
    该病例报告强调了机器人辅助手术方法在治疗胡桃夹综合征中的成功应用。病人,一名36岁女性在保守治疗失败后出现严重症状并接受机器人左肾静脉转位.该过程是通过利用达芬奇机器人系统™的微创方法进行的,该系统提供了增强的可视化和精度。然而,在肾静脉吻合术期间,由于张力和通过转位的流量不足,出现了挑战,需要用牛心包补片进行两次修正。最终,从左肾静脉远端吻合8mm环状PTFE旁路至下腔静脉。尽管面临这些挑战,患者经历了一个成功的结果,这种复杂的病理症状完全消除。
    This case report highlights the successful application of a robotic-assisted surgical approach in managing Nutcracker syndrome. The patient, a 36-year-old female presented with severe symptoms and underwent robotic left renal vein transposition after failing conservative management. The procedure was performed through a minimally invasive approach utilizing the Da Vinci robotic system™ which offers enhanced visualization and precision. However, challenges arose during the renal vein anastomosis due to tension and poor flow through the transposition, requiring two revisions with a bovine pericardial patch. Ultimately, an 8 mm ringed PTFE bypass was anastomosed from the distal left renal vein to the Inferior Vena Cava. Despite these challenges, the patient experienced a successful outcome with complete symptom resolution of this complicated pathology.
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  • 文章类型: Journal Article
    背景:胡桃夹综合征是一种以复杂症状为特征的疾病,使其诊断具有挑战性并且经常延迟,通常会给患者带来痛苦的经历。
    目的:本研究通过模拟左肾静脉不同受压程度的血流,从血流动力学的角度探讨胡桃夹综合征的发病机制。
    方法:腹主动脉的3D患者特异性血管模型,根据疑似胡桃夹综合征患者的CT图像构建肠系膜上动脉和左肾静脉。然后使用计算流体动力学进行血液动力学模拟,以确定血液动力学参数的变化与不同程度的压缩之间的相关性。
    结果:研究表明,左肾静脉上存在明显的速度分布梯度,狭窄程度相对较高(α≤50°),在狭窄的中央区域具有最大速度。此外,当左肾静脉的压缩程度增加时,左肾静脉的压力分布呈现增加的梯度层数量。此外,壁面剪应力与血流速度的变化具有相关性,即,壁面剪应力的增加与血流速度的加速有关。
    结论:使用计算流体动力学作为一种非侵入性工具来获得胡桃夹综合征的血流动力学特征是可行的,可以提供对胡桃夹综合征的病理机制的见解,支持临床医生的诊断。
    BACKGROUND: Nutcracker syndrome is a disease characterized by complex symptoms, making its diagnosis challenging and often delayed, often resulting in a painful experience for the patients.
    OBJECTIVE: This study aimed to investigate the pathogenesis of nutcracker syndrome through the perspective of hemodynamics by simulating blood flow with varying compression degrees of the left renal vein.
    METHODS: 3D patient-specific vascular models of the abdominal aorta, superior mesenteric artery and left renal vein were constructed based on CT images of patients suspected of having nutcracker syndrome. A hemodynamic simulation was then conducted using computational fluid dynamics to identify the correlation between alterations in hemodynamic parameters and varying degrees of compression.
    RESULTS: The study indicated the presence of an evident gradient in velocity distribution over the left renal vein with relatively high degrees of stenosis (α ≤ 50°), with maximum velocity in the central region of the stenosis. Additionally, when the compression degree of the left renal vein increases, the pressure distribution of the left renal vein presents an increasing number of gradient layers. Furthermore, the wall shear stress shows a correlation with the variation of blood flow velocity, i.e., the increase of wall shear stress correlates with the acceleration of the blood flow velocity.
    CONCLUSIONS: Using computational fluid dynamics as a non-invasive instrument to obtain the hemodynamic characteristics of nutcracker syndrome is feasible and could provide insights into the pathological mechanisms of the nutcracker syndrome supporting clinicians in diagnosis.
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  • 文章类型: Journal Article
    目的:本研究旨在评估具有可耐受症状的胡桃夹综合征患者的预后,并在没有侵入性干预的情况下进行保守治疗。
    方法:这项前瞻性研究包括保守治疗的患者。促进体重增加,研究的终点是症状的自发缓解.
    结果:16例患者(75%为女性,平均年龄24.4±3.5岁)接受保守治疗。平均随访27.3个月[13-42,四分位距(IQR)],直径比(5.5[5-6.5,IQR]对4.3[4.1-6],p=NS),峰值速度比(6[5-7,IQR]对4.8[4.8-5.8],p=NS),喙角(27°[24-30,IQR]vs29°[24-32];p=NS),和主动脉肠系膜角度(26°[23-29,IQR]vs28°[24-30];p=NS)没有统计学上的显着变化。症状完全缓解和改善分别为28.5%和31.4%,分别,而68.5%保持不变。
    结论:这项研究表明,保守方法有助于症状轻微的年轻成年患者的自发改善或完全缓解。
    OBJECTIVE: This study aims to evaluate outcomes in nutcracker syndrome patients with tolerable symptoms and treated conservatively without invasive interventions.
    METHODS: This prospective study included patients treated conservatively. Promoting weight gain, the endpoint of the study was spontaneous resolution of symptoms.
    RESULTS: Sixteen patients (75% female and mean age 24.4 ± 3.5 years) underwent conservative management. Over a mean follow-up of 27.3 months [13-42, interquartile range (IQR)], the diameter ratio (5.5 [5-6.5, IQR] vs 4.3 [4.1-6], p = NS), the peak velocity ratio (6 [5-7, IQR] vs 4.8 [4.8-5.8], p = NS), beak angle (27° [24-30, IQR] vs 29° [24-32]; p = NS), and aortomesenteric angle (26° [23-29, IQR] vs 28° [24-30]; p = NS) exhibited no statistically significant changes. Complete resolution and improvement of symptoms were 28.5% and 31.4%, respectively, while 68.5% remained unchanged.
    CONCLUSIONS: This study shows that a conservative approach contributes to the spontaneous improvement or complete resolution in young adult patients with mild symptoms.
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  • 文章类型: Case Reports
    伤寒,由伤寒沙门氏菌引起的,是发展中国家普遍存在的严重细菌感染,如果不治疗,可能会导致危及生命的并发症。胡桃夹综合征是一种罕见的血管疾病,涉及主动脉和肠系膜上动脉之间的左肾静脉受压。它可能导致各种症状,并提出诊断和管理挑战。我们提供了一个在重症急诊病房诊断为伤寒的患者的案例研究。巧合的是,通过CT扫描进行的评估显示存在胡桃夹综合征。该报告强调了在紧急护理环境中对伤寒患者进行评估期间偶然发现的胡桃夹综合症。
    Typhoid fever, caused by Salmonella Typhi, is a severe bacterial infection prevalent in developing countries, and can result in life-threatening complications if untreated. Nutcracker Syndrome is a rare vascular disorder involving compression of the left renal vein between the aorta and the superior mesenteric artery. It can lead to various symptoms and poses diagnostic and management challenges. We present a case study of a patient diagnosed with typhoid fever in a Unit of Critical Emergency Care. Coincidentally, the evaluation through CT-scan revealed the presence of Nutcracker Syndrome. This report underscores the incidental discovery of Nutcracker Syndrome during the assessment of a patient with typhoid fever in a critical emergency care setting.
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  • 文章类型: Journal Article
    目的:胡桃夹综合征是一种罕见的疾病,由于主动脉和肠系膜上动脉之间的左肾静脉阻塞而发生。它通常与诸如左侧腹疼痛等症状有关,血尿,蛋白尿,盆腔充血.目前的治疗方法可能是保守的,在存在可耐受的症状,以及在存在无法忍受症状的情况下,按优先顺序进行手术或混合和支架置入手术。这项研究的目的是回顾我们的经验,以评估本系列中两种方法的结果,在这些方法中,我们更倾向于手术而不是支架置入。
    方法:回顾性分析2019年7月至2030年10月连续行左肾静脉转位和左肾静脉支架置入术的胡桃夹综合征患者的临床资料。根据治疗方法将患者分为两组:手术和支架置入。对于程序选择,主要推荐LRV转座,向那些拒绝的人提供支架。主要终点是发病率和死亡率。次要终点包括晚期并发症,通畅,免于再干预,和症状的解决。采用标准的基本统计和生存分析方法。
    结果:19例胡桃夹综合征患者(女性-100%)接受了LRV支架(n=5)和左肾静脉转位(n=14)治疗。平均年龄为24(20-27,IQR)岁。平均随访23个月(9-32,IQR)。两种手术后均无重大并发症和死亡率。与左肾静脉卡压相关的最常见的体征和症状是左侧腹疼痛100%(n=19),蛋白尿88%(n=15),血尿占47%(n=9)。多普勒超声检查的平均峰值速度比为6.13(6-6.44,IQR)。主肠系膜角,喙角(喙标志),计算机断层扫描的平均直径比为26°(22.6-28.5,IQR),25°(23.9-28,IQR),和5.3(5-6,IQR),分别。静脉压测量仅用于确定支架置入组5例患者的诊断。测得的肾腔梯度为4(3.9-4.4,IQR)mmHg。在这两个程序之后,经典的症状,包括左翼疼痛,蛋白尿,血尿,解决了89.5%(n=17),57.8%(n=11),82.3%(n=15)的病例,分别。共有4名患者需要再次干预,3例LRV转位后患者(闭塞,n=2;狭窄,n=1),支架置入后1名患者(闭塞,n=1)。19例患者的1年和3年原发性通畅率分别为87%和80%,分别。三年初级辅助通畅率为100%。同样,一年和三年的再干预自由率为83%和72%,分别。此外,手术组的1年和3年主要通畅率分别为91%和81%,分别,支架组的1年和3年主要通畅率为75%.
    结论:如果腰痛和血尿不能与肾脏疾病有关,则应牢记胡桃夹综合征。放射学证据必须伴有严重的症状,以便通过左肾静脉转位和血管内支架置入手术开始治疗胡桃夹综合征。这两个程序,以及它们各自的优点和缺点,可优选作为胡桃夹综合征的主要治疗方法。我们的研究表明,这两个程序都可以安全有效地执行,产生良好的结果。
    BACKGROUND: Nutcracker syndrome is a rare condition that occurs as a result of the entrapment of the left renal vein (LRV) between the aorta and the superior mesenteric artery. It is typically associated with symptoms such as left flank pain, hematuria, proteinuria, and pelvic congestion. The current treatment approach may be conservative in the presence of tolerable symptoms, and surgical or hybrid and stenting procedures in the order of priority in the presence of intolerable symptoms. The aim of this study is to review our experiences to evaluate the results of both methods in this series in which we have a greater tendency toward surgery instead of stenting.
    METHODS: The clinical data of consecutive patients with nutcracker syndrome who underwent LRV transposition and LRV stenting between July 2019 and October 2023 were retrospectively reviewed. The patients were divided into 2 groups based on the methods of treatment: surgical and stenting. For procedure selection, LRV transposition was primarily recommended, with stenting offered to those who declined. Primary end points were morbidity and mortality. Secondary end points included late complications, patency, freedom from reintervention, and resolution of symptoms. Standard basic statistics and survival analysis methods were employed.
    RESULTS: Nineteen patients with nutcracker syndrome (female: 100%) were treated with LRV stentings (n = 5) and LRV transposition (n = 14). The mean age was 24 (20-27, interquartile range [IQR]) years. The mean follow-up was 23 (9-32, IQR) months. There were no major complications and mortality after both procedures. The most frequent sign and symptom associated with LRV entrapment were left flank pain 100% (n = 19), proteinuria 88% (n = 15), and hematuria 47% (n = 9). The mean peak velocity ratio on Doppler ultrasound was 6.13 (6-6.44, IQR). Aortomesenteric angle, beak angle (beak sign), and mean diameter ratio on computed tomography were 26° (22.6-28.5, IQR), 25° (23.9-28, IQR), and 5.3 (5-6, IQR), respectively. Venous pressure measurements were only used to confirm the diagnosis in 5 patients in the stenting group. The measured renocaval gradient was 4 (3.9-4.4, IQR) mm Hg. After both procedures, the classical symptoms, including left flank pain, proteinuria, and hematuria, resolved in 89.5% (n = 17), 57.8% (n = 11), and 82.3% (n = 15) of the cases, respectively. A total of 4 patients required reintervention, 3 patients after LRV transposition (occlusion, n = 2; stenosis, n = 1), and 1 patient after stenting (occlusion, n = 1). The 1-year and 3-year primary patency for the 19 patients was 87% and 80%, respectively. Three-year primary-assisted patency was 100%. Similarly, the 1-year and 3-year freedom from reintervention rate was 83% and 72%, respectively. Additionally, the 1-year and 3-year primary patency for the surgical group was 91% and 81%, respectively, and the 1-year and 3-year primary patency for the stenting group was 75%.
    CONCLUSIONS: Nutcracker syndrome should be kept in mind in cases where flank pain and hematuria cannot be associated with kidney diseases. Radiographic evidence must be accompanied by serious symptoms to initiate the treatment of nutcracker syndrome with LRV transposition and endovascular stenting procedures. Both procedures, along with their respective advantages and disadvantages, can be preferred as primary treatments for nutcracker syndrome. Our study demonstrates that both procedures can be safely and effectively performed, yielding good outcomes.
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