Nutcracker syndrome

胡桃夹综合征
  • 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: Case Reports
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  • 文章类型: Journal Article
    胡桃夹综合征(NCS)是精索静脉曲张的罕见原因,其治疗仍存在争议。
    总结微血管多普勒(MVD)辅助显微手术左精索-腹壁下静脉吻合术(MLSIEVA)与同一切口显微手术精索静脉曲张(MV)治疗NCS相关精索静脉曲张的手术策略及疗效。
    回顾性分析2018年7月至2022年1月间13例NCS相关精索静脉曲张。
    选择对应于腹股沟深环的身体突起中的小切口作为手术切口。所有患者在MVD的辅助下接受MLSIEVA和MV。
    患者在手术前后接受了实时多普勒超声(DUS);测试了尿液红细胞和蛋白质,随访时间为12-53个月。
    所有患者均无术中并发症,以及所有术后血尿或蛋白尿的症状,阴囊肿胀,腰痛消失了。比较术前和术后的DUS,两名患者的术后测量没有任何改善.然而,在剩下的病人中,肾门部和主动脉肠系膜角部分的肾静脉内径,以及它们的比率,与术前测量相比有显著改善。术后随访未见精索静脉曲张复发及并发症。
    我们的研究表明,MVD辅助的MLSIEVA伴MV是可行的,没有重大的短期并发症,对精索静脉曲张和NCS的治疗有效。
    我们研究了微超声介导的显微外科手术治疗与胡桃夹综合征相关的精索静脉曲张。我们发现此程序是安全有效的,长期效果良好。
    UNASSIGNED: Nutcracker syndrome (NCS) is a rare cause of varicocele and its treatment is still controversial.
    UNASSIGNED: To summarize the surgical strategy and outcomes of microvascular Doppler (MVD)-assisted microsurgical left spermatic-inferior epigastric vein anastomosis (MLSIEVA) with microsurgical varicocelectomy (MV) at the same incision for treating NCS-associated varicocele.
    UNASSIGNED: A retrospective analysis of 13 cases of NCS-associated varicocele between July 2018 and January 2022 was performed.
    UNASSIGNED: A small incision in the body projection corresponding to the deep inguinal ring was chosen as the surgical incision. All patients underwent MLSIEVA and MV with the assistance of MVD.
    UNASSIGNED: Patients received real-time Doppler ultrasound (DUS) before and after surgery; urine red blood cells and protein were tested, with a follow-up time of 12-53 mo.
    UNASSIGNED: All patients had no intraoperative complications, and all postoperative symptoms of hematuria or proteinuria, scrotal swelling, and low back pain disappeared. Comparing pre- and postoperative DUS, two patients did not show any improvement in their postoperative measurements. However, in the remaining patients, the internal diameter of the renal vein at the hilum portion and at the aortomesenteric angle portion, as well as their ratio, improved significantly compared with preoperative measurements. No complications or recurrence of varicocele was observed during postoperative follow-up.
    UNASSIGNED: Our study suggests that MVD-assisted MLSIEVA with MV is feasible with no major short-term complication and effective regarding the treatment of varicocele and NCS.
    UNASSIGNED: We investigated microsurgery mediated by microultrasound for the treatment of varicocele associated with nutcracker syndrome. We found this procedure to be safe and effective with good long-term results.
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  • 文章类型: Case Reports
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  • 文章类型: Review
    背景:高血糖是一种罕见的疾病,报告的病例很少,由甘氨酸代谢缺陷或肾脏甘氨酸重吸收障碍引起。高血糖症的遗传发现很少见,以前在中国年轻男性中没有报道。
    方法:一名24岁男子表现为符合双侧腰痛1个月。腹部计算机断层扫描显示双侧肾结石和右上输尿管扩张。24小时尿液分析显示,尿液草酸盐水平很高,为63mg/天。尿液中的氨基酸分析表明,他的尿甘氨酸水平异常高(2.38µmol/mg肌酐)。全外显子组测序检测到SLC6A19变体c.1278C>Tp.(Cys426)。输尿管软镜钬激光碎石术两次切除双侧肾结石。术后结石生化成分分析表明,结石由大约70%的草酸钙一水合物和30%的草酸钙二水合物组成。患者随后被诊断患有高血糖尿。结石手术三个月后,在健康检查中,超声检查显示右甲状腺叶下有一个结节。他的血清甲状旁腺激素(PTH)水平增加到392.3pg/mL。切除右侧甲状旁腺结节,组织病理学检查证实右侧甲状旁腺腺瘤。在2年的随访期间,肾结石没有复发,和血清PTH,钙,磷水平正常.
    结论:SLC6A19基因可能在中国年轻人高血糖症的发展中具有重要意义。遇到肾结石时,可以考虑进一步评估甘氨酸排泄障碍的可能性。
    BACKGROUND: Hyperglycinuria is a rare disorder, with few reported cases, caused by either a defect in glycine metabolism or a disturbance in renal glycine reabsorption. Genetic findings of hyperglycinuria are rare and have not previously been reported in Chinese young men.
    METHODS: A 24-year-old man presented with a compliant of bilateral lumbago for 1 month. Abdominal computed tomography revealed bilateral kidney stones and right upper ureteral dilatation. The 24-h urine analysis showed high urine oxalate levels of 63 mg/day. Analysis of amino acids in urine revealed that his urinary glycine levels were abnormally high (2.38 µmol/mg creatinine). Whole-exome sequencing detected the SLC6A19 variant c.1278 C > T p. (Cys426). Flexible ureteroscopy with holmium laser lithotripsy was conducted twice to remove his bilateral nephrolithiasis. Postoperative stone biochemical composition analysis revealed that the stones were composed of approximately 70% calcium oxalate monohydrate and 30% calcium oxalate dihydrate. The patient was subsequently diagnosed with hyperglycinuria. Three months after the stone surgery, ultrasonography revealed one nodule under the right thyroid lobe during a health checkup. His serum parathyroid hormone (PTH) levels increased to 392.3 pg/mL. Resection of the right parathyroid nodule was performed, and the histopathological examination confirmed right parathyroid adenoma. During the 2-year follow-up period, nephrolithiasis did not relapse, and serum PTH, calcium, and phosphorus levels were normal.
    CONCLUSIONS: The SLC6A19 gene may have been significant in the development of hyperglycinuria in a Chinese young man. Further evaluation for the possibility of a glycine excretion disorder could be considered when encountering nephrolithiasis.
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  • 文章类型: Case Reports
    应用三维打印聚醚醚酮(PEEK)血管外支架治疗一名14岁男孩胡桃夹综合征。数字减影血管造影显示,左肾静脉(LRV)的一段造影剂充盈减少,然后立即流入下腔静脉。和高压力梯度。三维重建模型显示,LRV和十二指肠在主动脉肠系膜角收缩,导致异常高位十二指肠室的LRV压缩。当十二指肠在腹主动脉和肠系膜上动脉之间行进时(十二指肠间置术),LRV截留甚至发生在<90主动脉肠系膜度。选择三维打印PEEK血管外支架抬高肠系膜上动脉,降低十二指肠位置,从而减轻LRV压缩。这种血管外应用比开放手术具有显著的优势,用膨胀聚四氟乙烯进行血管内支架和人工血管手术。它通过确保软组织增殖提供更好的细胞活力。通过减少外部加速度和离心力,三维打印的PEEK血管外支架可减少不良副作用。这样的支架具有独特的个性化设计,良好的刚度,以及允许血管生长的耐久性,防止支架迁移和血栓形成。因此,它适用于成人和儿科患者。根据腹部超声和多层螺旋CT扫描,术后1年随访结果满意。病人感觉很好,LRV的血流没有阻塞,血流速度为平均值。外部支架就位。
    Three-dimensional printed polyetheretherketone (PEEK) extravascular stent was applied to treat a 14-year-old boy with nutcracker syndrome. Digital subtraction angiography revealed a segment of the left renal vein (LRV) with reduced contrast filling immediately before its inflow into the inferior vena cava, and high-pressure gradient. The three-dimensional reconstruction model demonstrated that the LRV and the duodenum were contracted at the aortomesenteric angle, resulting in LRV compression from the abnormal high-level duodenal compartment. When duodenum courses between the abdominal aorta and superior mesenteric artery (duodenal interposition), the LRV entrapment occurs even at <90 aortomesenteric degrees. Three-dimensional printed PEEK extravascular stent was chosen to elevate the superior mesenteric artery and lower the duodenum position, thus relieving LRV compression. This extravascular application has significant advantages over open surgery, endovascular stenting and artificial vessel procedures with expanded polytetrafluoroethylene. It provides better cellular vitality by ensuring soft tissue proliferation. By reducing external acceleration and centrifugal force, a three-dimensional printed PEEK extravascular stent reduces adverse side effects. Such a stent has a distinctive personalized design, good stiffness, and durability that allows blood vessel growth, preventing stent migration and thrombosis. Therefore, it is suitable for both adult and pediatric patients. According to the abdominal ultrasound and multi-slice computed tomography scan, the postoperative follow-up results were satisfactory one year after surgery. The patient felt well, the blood flow in the LRV was not obstructed, and the blood flow velocity was average. The external stent was in place.
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  • 文章类型: Journal Article
    目的:我们旨在评估经腹膜或腹膜后入路腹腔镜血管外支架治疗胡桃夹综合征的可行性和有效性。
    方法:从三级转诊中心回顾性纳入76例胡桃夹综合征患者,2011年3月至2020年12月接受了经腹膜(63例)或腹膜后(13例)腹腔镜血管外支架。手术参数,并发症,收集并分析影像学和临床结果。
    结果:所有程序均成功进行,没有开放转化。中位手术时间,估计失血量,术后住院天数为120(四分位距[IQR]:90-144)min,20(IQR:10-30)ml,和7(IQR:6-9)天。在中位随访52个月(范围:9-127个月),60例(79%)患者症状完全缓解,14例(18%)患者症状明显改善,2例(3%)患者报告没有症状改善。百分之九十四(50/53)的血尿,91%(30/33)的蛋白尿,血管外LRV支架置入后,89%(25/28)的侧腹/腹痛缓解。两种方法在手术参数和临床症状恢复率方面没有显着差异(均p>0.05)。然而,与腹膜后入路相比,经腹膜入路的患者需要更长的时间才能完全恢复(8.7vs.1.5个月,p=0.016)。
    结论:腹腔镜经腹膜或腹膜后血管外支架治疗胡桃夹综合征是可行和有效的选择。后腹腔镜血管外支架需要较短的时间才能达到完全恢复,在手术决策中应尽可能考虑这一点。
    We aimed to assess the feasibility and efficacy of laparoscopic extravascular stent in treatment of nutcracker syndrome by transperitoneal or retroperitoneal approach.
    Seventy-six patients with nutcracker syndrome were retrospectively enrolled from a tertiary referral center, and underwent transperitoneal (63 patients) or retroperitoneal (13 patients) laparoscopic extravascular stent from March 2011 to December 2020. Surgical parameters, complications, imaging and clinical outcomes were collected and analyzed.
    All procedures were successfully carried out without open conversion. The median operation time, estimated blood loss, and postoperative hospital day were 120 (interquartile range [IQR]: 90-144) min, 20 (IQR: 10-30) ml, and 7 (IQR: 6-9) days. At a median follow-up of 52 (range: 9-127) months, 60 (79%) patients had complete symptom resolution, 14 (18%) patients had significant symptom improvement, and 2 (3%) patients reported no symptom improvement. Ninety-four percent (50/53) of hematuria, 91% (30/33) of proteinuria, and 89% (25/28) of flank/abdominal pain resolved after extravascular LRV stenting. No significant differences were detected in surgery parameters and recovery rates of clinical symptoms between two approaches (each p > 0.05). However, patients with transperitoneal approach need longer to achieve complete recovery compared with retroperitoneal approach (8.7 vs. 1.5 months, p = 0.016).
    Laparoscopic extravascular stent performed either transperitoneally or retroperitoneally is a feasible and effective option in treatment of nutcracker syndrome. Retroperitoneal laparoscopic extravascular stent required shorter time to achieve complete recovery, which should be considered whenever possible in surgical decision-making.
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