Renal Nutcracker Syndrome

  • 文章类型: Journal Article
    目的:评估右肾动脉(RRA)压迫左肾静脉(LRV)的发生率,一种主要报告为病例报告的现象。
    方法:横断面研究连续筛选了2018年11月至2023年3月在东北某教学医院的38例(肾性)肾病患者和305例(非肾性)外周动脉疾病患者的肾血管CT数据。相邻解剖结构对LRV的压缩,包括但不限于RRA和多个压缩相关参数,通过对CT数据的多平面分析进行了研究。
    结果:相邻结构的总体LRV截留率分别为41.93%(12/31)和24.00%(6/25),RRA来源的LRV压缩率为22.58%(7/31)和20.00%(5/25),肾组和非肾组肠系膜上动脉(SMA)压迫率分别为16.13%(5/31)和4.00%(1/25),分别,没有意义。两组中,RRA压缩部位远端的静脉段的横截面管腔面积均明显大于非压缩静脉(3.09±1.29vs.1.82±0.23,p<0.001和4.30±2.65vs.2.12±0.55,p=0.006;肾脏组和非肾脏组的最大最小面积比,分别)。发现近80%的RRA是向右向前产生的,而不是直接向右传递。
    结论:RRA来源的LRV压缩并不罕见,在两个患者队列中,其发生率均高于SMA压迫。关于LRV截留,RRA可能是比SMA更常见的压缩源。涉及不同人群的进一步调查,包括健康的个体,是需要的。
    OBJECTIVE: To estimate the incidences of left renal vein (LRV) entrapment by right renal artery (RRA), a phenomenon primarily reported as case reports.
    METHODS: The cross-sectional study consecutively screened renal vessel CT data of 38 (Renal) patients with nephropathy and 305 (Non-renal) patients with peripheral arterial diseases in a teaching hospital in northeast China between November 2018 and March 2023. The LRV compression by adjacent anatomical structures, including but not limited to RRA and multiple compression-related parameters, were investigated through multiplanar analysis of the CT data.
    RESULTS: The overall LRV entrapment rates by adjacent structures were 41.93% (12/31) and 24.00% (6/25), the rates of RRA-sourced LRV compression 22.58% (7/31) and 20.00% (5/25), and the rates of compression by superior mesenteric artery (SMA) 16.13% (5/31) and 4.00% (1/25) in the Renal and Non-renal groups, respectively, with no significance. The venous segments distal to the RRA-compressed site had a significantly larger transectional lumen area than those of the non-compressed veins in both groups (3.09 ± 1.29 vs. 1.82 ± 0.23, p < 0.001 and 4.30 ± 2.65 vs. 2.12 ± 0.55, p = 0.006; maximum-to-minimum area ratios in Renal and Non-renal groups, respectively). Nearly 80% of RRAs were found arising anteriorly rightwards instead of passing straight to the right.
    CONCLUSIONS: RRA-sourced LRV compression was not rare, and its incidence was higher than that of the compression by SMA in both patient cohorts. RRA could be a more common compression source than SMA concerning LRV entrapment. Further investigations involving different populations, including healthy individuals, are needed.
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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
    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: 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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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Journal Article
    背景:胡桃夹现象是肠系膜上动脉(SMA)和腹主动脉之间的左肾静脉受压。胡桃夹综合征是指存在胡桃夹现象并伴有症状。在2016年至2022年之间,我们进行了18次机器人辅助腹腔镜血管外肾静脉支架(RAVES)放置。我们试图评估患者报告的机器人辅助腹腔镜血管外肾静脉支架(RAVES)在单个机构放置的结果。
    方法:我们对18例患者进行了单中心回顾性分析,随访时间至少为3个月。在3个月时使用5分Likert量表进行的问卷调查评估症状。李克特量表上有五个很严重,而一个没有。主要研究结果比较了术前和术后患者报告的症状评分。
    结果:18名患者中有12名接受了调查。该队列的平均年龄为36岁,平均BMI为19.4。只有一个男性。平均手术时间为137分钟,平均估计失血量为12cc。术前平均SMA角度为19度,术后平均SMA角度为36度。患者报告说侧腹疼痛,腹痛,恶心,头痛,背痛,盆腔疼痛,早期饱腹感改善(P<0.05)。12位受访者中有10位建议使用RAVES治疗胡桃夹综合症。
    结论:RAVES证明了胡桃夹综合征的有效治疗方法。患者报告在多个领域的术后3个月症状改善。需要进行进一步的研究以评估血管外肾静脉移植物的长期耐久性。
    Introduction: Nutcracker phenomenon is the compression of the left renal vein between the superior mesenteric artery (SMA) and the abdominal aorta. Nutcracker syndrome refers to the presence of nutcracker phenomenon with symptoms. Between 2016 and 2022, we performed 18 robot-assisted laparoscopic extravascular renal vein stent (RALERVS) placements. We sought to assess patient-reported outcomes of RALERVS placement by a single institution. Methods: We performed a single-center retrospective review of 18 patients with a minimum of 3 months follow-up. Symptoms were assessed utilizing a questionnaire conducted with a 5-point Likert scale at a minimum of 3 months postoperatively. Five on the Likert scale was severe while 1 was none. Primary study outcomes compared pre- and postoperative patient-reported symptom scores. Results: Twelve out of 18 patients responded to the survey. The average length of time from date of operation to completion of survey was 2.6 years. Average age of the cohort was 36 years with a mean BMI of 19.4 kg/m2. There was only one man. Mean operative time was 137 minutes and mean estimated blood loss was 12 mL. Mean preoperative SMA angle was 19° and mean postoperative SMA angle was 36°. Patients reported that flank pain, abdominal pain, nausea, headaches, back pain, pelvic pain, and early satiety improved (p < 0.05). Ten of 12 respondents would recommend RALERVS for nutcracker syndrome. Conclusion: RALERVS demonstrates an effective treatment for nutcracker syndrome. Patients reported improved symptoms at 3 months postoperatively across multiple domains. Further studies need to be conducted to assess long-term durability of the extravascular renal vein graft.
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  • 文章类型: Journal Article
    胡桃夹子现象,也被称为左肾静脉压迫,发生在左肾静脉外在压迫时,通常在腹主动脉和肠系膜上动脉之间。胡桃夹综合征是指胡桃夹现象可能引起的临床症状的星座,通常包括血尿,侧腹/骨盆疼痛,体位性蛋白尿和(男性患者)精索静脉曲张。我们提供了胡桃夹综合征的简短回顾,包括各种诊断和治疗方式。我们利用自己与患者的经验作为案例研究,并强调血管内支架置入术的现代管理选择。
    The nutcracker phenomenon, also known as left renal vein entrapment, occurs when there is extrinsic compression of the left renal vein, most often between the abdominal aorta and the superior mesenteric artery. Nutcracker syndrome refers to the constellation of clinical symptoms that may arise from the nutcracker phenomenon, typically inclusive of haematuria, flank/pelvic pain, orthostatic proteinuria and (in male patients) varicocele. We provide a short review of the nutcracker syndrome including various diagnostic and therapeutic modalities. We utilise our own experience with a patient as a case study and highlight the modern management option of endovascular stenting.
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  • 文章类型: Journal Article
    背景:胡桃夹综合征(NCS)描述了一组由左肾静脉(LRV)受压引起的症状和体征。对它的自然过程缺乏了解,诊断,和管理,尤其是儿童。在这里,我们向大量有长期随访结果的患者介绍了单中心的经验.
    方法:纳入2011年1月至2021年3月诊断的所有NCS患者,并对其数据进行回顾性分析。
    结果:共纳入123例NCS患者(85例女性)。诊断时的中位年龄为12(IQR10-14)岁,在38%的病例中,BMI百分位数低于5%。在诊断的时候,三分之二的患者无症状。最常见的实验室发现是肾病性蛋白尿(98%),其次是显微镜下血尿(16%)。在直立位置多普勒超声(DUS)检查中,LRV受压的迹象明显更明显。对所有患者进行了保守随访;在随访的35.8±25.8个月内,108例患者中有43例(40%)出现血尿和/或蛋白尿。在平均39.1±21.3个月后,对52例患者进行了对照DUS。发现与初始评估相比,直立位置的LRV的中值峰值速度和直径比率显着降低(p<0.05)。在最终评估时,13例患者的DUS表现正常。
    结论:在无法解释的蛋白尿和/或血尿中,NCS应该考虑,尤其是在虚弱的青少年中。我们的结果支持儿童保守治疗作为一线治疗方法。更高分辨率版本的图形摘要可作为补充信息。
    BACKGROUND: Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein, we present our single-center experience with a large number of patients who have long-term follow-up results.
    METHODS: All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were obtained retrospectively.
    RESULTS: A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10-14) years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria (16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination. All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%) within 35.8 ± 25.8 months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3 months. The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation.
    CONCLUSIONS: In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents. Our results support conservative management in children as the first-line treatment approach.
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