A Novel Approach for the Treatment of Nutcracker Syndrome: A Case Report.

We describe a case of nutcracker syndrome in a 35 year-old male that was treated with a left renal vein transposition via an open retroperitoneal approach. Our case highlights some of the advantages of the retroperitoneal approach, which may decrease the risk of postoperative complications when compared to the traditional midline abdominal transperitoneal approach. The patient agreed to publish the case details and images included below.

An Uncommon Variant of Nutcracker Syndrome Secondary to Left Renal Vein Compression Between the Right Renal Artery and The Proper Hepatic Artery.

Nutcracker syndrome refers to the compression of the left renal vein between the abdominal aorta and the superior mesenteric artery. The subsequent venous congestion of the left kidney, when symptomatic, could be associated with left flank pain, hematuria, varicocele, dyspareunia, dysmenorrhea, and proteinuria. Here we describe a 42-year-old female patient with simultaneous Dunbar syndrome and a rare variant of nutcracker syndrome in which the left renal vein (LRV) compression is secondary to the unusual path of the vein between the right renal artery and the proper hepatic artery. For both the nutcracker syndrome and the Dunbar syndrome, open approach by median mini-laparotomic access for transposition of LRV, and resection of the diaphragmatic pillars and arcuate ligament was attempted. During the intervention, due to anatomical issues, the LRV transposition was converted to endovascular stenting of the LRV, moreover the implanted stent was transfixed with an external non-absorbable suture to avoid migration. At the 12 months follow-up the patient was asymptomatic, and the duplex scan confirmed the patency of the celiac trunk without re-stenosis and a correct position of the LRV stent with no proximal or distal migration.

Nutcracker Syndrome: How to Diagnose It and When/How Should It Be Treated in the Pelvic Venous Disease Population.

With wider recognition of left renal vein compression / obstruction, especially as an incidental finding, the significance as it relates to the patient's symptoms needs to be evaluated in light of variable practices and results of treatment. This communication deals with problems of diagnosis, clinical significance, options and indications for treatment.

Nutcracker syndrome.

This study represents a concise review of Nutcracker syndrome with its history, incidence, clinical presentation and currently available diagnostic criteria and treatment options. This should help any clinician identify and better serve patients with rare venous disorders such as this. Although the literature is scarce, clinicians should be keen to diagnose and treat patients with this potentially debilitating syndrome.

The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: A report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders.

As the importance of pelvic venous disorders (PeVD) has been increasingly recognized, progress in the field has been limited by the lack of a valid and reliable classification instrument. Misleading historical nomenclature, such as the May-Thurner, pelvic congestion, and nutcracker syndromes, often fails to recognize the interrelationship of many pelvic symptoms and their underlying pathophysiology. Based on a perceived need, the American Vein and Lymphatic Society convened an international, multidisciplinary panel charged with the development of a discriminative classification instrument for PeVD. This instrument, the Symptoms-Varices-Pathophysiology ("SVP") classification for PeVD, includes three domains-Symptoms (S), Varices (V), and Pathophysiology (P), with the pathophysiology domain encompassing the Anatomic (A), Hemodynamic (H), and Etiologic (E) features of the patient's disease. An individual patient's classification is designated as SVPA,H,E. For patients with pelvic origin lower extremity signs or symptoms, the SVP instrument is complementary to and should be used in conjunction with the Clinical-Etiologic-Anatomic-Physiologic (CEAP) classification. The SVP instrument accurately defines the diverse patient populations with PeVD, an important step in improving clinical decision making, developing disease-specific outcome measures and identifying homogenous patient populations for clinical trials.

Gonadal vein transposition is a safe and effective treatment of nutcracker syndrome.

BACKGROUND: Nutcracker syndrome (NCS) is a pelvic venous disorder that results from outflow obstruction of the left renal vein, most often due to a decreased aortomesenteric angle, leading to gonadal vein reflux, pain, and varices. Although a number of open and minimally invasive procedures to treat NCS have been described, the optimal management of this condition remains uncertain. To the best of our knowledge, we have presented the largest case series to date using gonadal vein transposition (GVT) to treat NCS. METHODS: Patients considered for intervention to treat NCS underwent a rigorous and standardized workup, including axial imaging studies, catheter-based diagnostic procedures, and urinalysis. GVT has been the institutional first-line treatment of NCS for appropriate patients. With institutional review board approval, a retrospective review of patients who had undergone GVT for NCS was conducted. RESULTS: From 2014 to 2019, 18 GVTs had been performed. Of the 18 patients, none had died or required reintervention, although 2 had required readmission. During a median follow-up of 178 days, complete symptom relief was achieved in 11 patients (61.1%), with 4 patients (22.2%) reporting partial symptom relief and 2 (11.1%) reporting transient symptom relief. CONCLUSIONS: GVT is a safe and effective procedure to treat NCS in appropriately selected patients with outcomes that compare favorably with those of other described procedures. Appropriate patient selection for this procedure is critical and requires a rigorous and standardized approach to diagnosis and management.

Los puntos oscuros de la insuficiencia venosa pélvica / The dark spots of pelvic venous insufficiency

La insuficiencia venosa pélvica es un tema de mucho debate en congresos de nuestro ámbito profesional. Sin embargo, cuanto más se reconoce y se trata esta patología en nuestro medio, más casos encontramos en los que, aplicando un tratamiento estándar de embolización venosa o stenting, se obtiene un resultado desfavorable. Para evitar este patrón de resultados, debemos estudiar con detenimiento la circulación venosa abdominopélvica estableciendo paralelismos con la circulación venosa infrainguinal y, además, aplicar tecnología IVUS en los casos de duda. Con estas dos medidas, podemos afrontar esta patología en su conjunto con unos resultados muy positivos para nuestros pacientes

Pelvic Venous Insufficiency is a topic of much debate in Congresses of our professional field. However, the more this pathology is recognized and treated in our setting, the more cases we find in which applying a standard venous embolization or stenting, treatment results in an unfavorable result. To avoid this type of results, we must carefully study the abdomino-pelvic venous circulation, establishing parallels with the infrainguinal venous circulation, and also apply IVUS technology in cases of doubt. With these two measures, we can face this pathology as a whole with very positive results for our patients

Salvage Renal Autotransplant Following Previous Renal Vein Stenting in Nutcracker Syndrome.

OBJECTIVES: Nutcracker syndrome is rare, and a proportion of patients with this syndrome continue to have intractable pain and symptoms. Due to the heterogeneity of patients' chief complaints and symptoms, the surgeon's preferred approach may be inherently different but is of paramount importance to the outcome. MATERIALS AND METHODS: We present 4 cases in which renal autotransplant with extraction and ligation of previously placed gonadal coils was performed following previously attempted renal vein stenting or combined renal vein transposition followed by renal vein stenting. RESULTS: Autotransplant resulted in flank pain resolution with improvement in symptoms associated with pelvic congestion syndrome. CONCLUSIONS: The approach to such cases requires meticulous and adequate vena cava exposure, with preparation for potential caval reconstruction. No firm inferences can be made from such a small series; however, we believe in renal autotransplant as first-line therapy, and failure after an initial renal vein stent should be salvaged by renal autotransplant over further endovascular attempts.

Outcomes of left renal vein stenting in patients with nutcracker syndrome.

BACKGROUND: Nutcracker syndrome (NCS) is a rare condition that can be manifested with hematuria, flank pain, pelvic varicosities, or chronic pelvic congestion related to left renal vein (LRV) compression. Open surgery, specifically LRV transposition, has been the mainstay of treatment, but in the past few years, LRV stenting has emerged as a less invasive alternative without sufficient evidence to support it. This study aimed to assess outcomes of renal vein stenting in the treatment of NCS. METHODS: A retrospective chart review of patients with NCS who underwent LRV stenting between 2010 and 2018 was performed. End points were perioperative adverse outcomes, symptom relief, and stent patency. Symptom resolution was classified as complete, partial, and none on the basis of the interpretation of medical records on clinical follow-up. Standard descriptive statistics and survival analysis were used. RESULTS: Eighteen patients (17 female; mean age, 38.1 ± 16.9 years) diagnosed with NCS and treated with LRV stenting were identified. Five of these had a prior LRV transposition that had failed within a mean of 7.0 ± 4.9 months, manifested by symptom recurrence (or no improvement) along with imaging evidence of persistently severe renal vein stenosis. Twelve patients had coexisting pelvic congestion syndrome treated with gonadal vein embolization. The most frequent sign and symptom were hematuria (10/18 patients) and flank pain (15/18 patients), respectively. All patients received self-expanding stents (mean diameter, 12.8 ± 1.6 mm), the smaller ones typically placed in the previously transposed LRVs. No perioperative complications occurred. Nine patients were discharged on the same day; the remaining patients stayed longer for pain control (mean hospital stay, 1.0 ± 1.3 days). At an average follow-up of 41.4 ± 26.6 months, 13 (72.2%) patients had symptoms resolved or improved (9 complete, 4 partial). Three of the five patients whose symptoms remained unchanged had previous LRV transposition surgery, and two of these three patients eventually required renal autotransplantation. Six of 10 patients who presented with hematuria had it resolved. Three patients underwent a stent reintervention at 5.8 months, 16.8 months, and 51.7 months because of symptom recurrence or stent restenosis. The two early ones required balloon venoplasty and the third one restenting. Two-year primary and primary assisted patency was 85.2% and 100%, respectively. No stent migration occurred. CONCLUSIONS: Endovascular treatment with renal vein stenting is safe, providing encouraging results with good midterm patency rates and symptom relief. Minimally invasive approaches may have a potential role in the treatment of NCS. Larger series and longer follow-up are needed to better assess the comparative performance against LRV transposition.

Renal autotransplantation results in pain resolution after left renal vein transposition.

Left renal vein transposition is often the preferred treatment of nutcracker syndrome. However, pain returns in some patients despite surgery. One solution to this problem is renal autotransplantation. Here we report our initial results of renal autotransplantation in patients with persistent flank pain despite a previous left renal vein transposition. We used the University of Wisconsin loin pain hematuria syndrome test as a diagnostic maneuver to determine who may benefit from renal autotransplantation; this procedure subsequently resulted in complete pain resolution in all three patients. All patients underwent successful renal autotransplantation and remain pain free. These cases support the test as a diagnostic maneuver to determine which patients may benefit from renal autotransplantation.

Robot-Assisted Laparoscopic Left Renal Vein Transposition for the Treatment of Nutcracker Syndrome: A Preliminary Experience.

BACKGROUND: The aim of this study was to describe our robot-assisted laparoscopic left renal vein (LRV) transposition experiences for nutcracker syndrome treatment. METHODS: From August 2016 through May 2017, three patients with nutcracker syndrome underwent robot-assisted laparoscopic LRV transpositions. The patient demographics, surgical outcomes, and postoperative morbidities were reviewed. RESULTS: Successful surgical procedures were performed in all three patients. The operative times for the three cases were 150, 175, and 162 minutes, respectively, while the LRV anastomosis times were 19, 22, and 13 minutes, respectively. No major perioperative complications were encountered, and the hematuria and flank pain were resolved in all three cases. At the 6-month follow-up, the computed tomography scan showed that the LRV narrowing had disappeared in two of the patients. Although one patient still exhibited LRV flattening, his symptoms were also relieved, and the varicose tributaries spontaneously ceased. CONCLUSIONS: Robot-assisted laparoscopic LRV transposition can be a viable minimally invasive treatment option for patients with nutcracker syndrome.

Endovascular treatment of posterior nutcracker syndrome with a new autoexpandable stent.

The endovascular treatment of nutcracker syndrome is currently considered by some to be the preferred treatment option in this pathologic process despite its risks. However, currently, there are few data about the pure endovascular approach in the posterior nutcracker syndrome related to evolution in the midterm. We present two successful cases of a complete endovascular approach in this disease, with follow-up of 9 months and 17 months without complications.

A systematic review on management of nutcracker syndrome.

OBJECTIVE: Although nutcracker syndrome (NS) is rare, patients presenting with symptoms or signs and anatomic compression of the left renal vein (LRV) can be considered for intervention. Open, laparoscopic, and endovascular techniques have been developed to decrease the venous outflow obstruction of the LRV. The paucity of data regarding the management of this uncommon disease process poses a challenge for adequate recommendations of the best treatment modality. Herein, we aim to present a systematic review for the management of NS. METHODS: We used the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement standards to systematically search the electronic databases of MEDLINE from October 1982 to July 2017 for articles about the management of NS. Included were studies in English, Spanish, and German in all age groups. RESULTS: The literature search provided 249 references. After abstract and full review screening for inclusion, 17 references were analyzed. Eight (47%) described the open surgical approach. The LRV transposition was the most commonly reported technique, followed by renal autotransplantation. Seven (41.11%) described the endovascular technique of stent implantation, and two (11.7%) described the minimally invasive laparoscopic extravascular stent implantation. CONCLUSIONS: NS is a rare entity. Multiple techniques have been developed for the treatment of this condition. However, the rarity of this syndrome, the paucity of data, and the short-term follow-up of the existing evidence are the disadvantages that prevent recommendations for the best treatment strategy. Up to now, open surgical intervention, specifically LRV transposition, has been considered by some experts the mainstay for treatment of NS. The endovascular approach is gaining strength as more evidence has become available. However, the long-term patency and durability of this approach remain to be elucidated. Therefore, careful selection of patients is necessary in recommending this technique.

Gonadal Vein Transposition in Nutcracker Syndrome.

BACKGROUND: Nutcracker syndrome, caused by mesoaortic compression of the left renal vein leading to symptoms related to venous hypertension, is an uncommon entity that may require operative intervention. Traditional open transposition of the left renal vein to the vena cava has been shown to have a reintervention rate of up to 30%, while also having additional morbidity associated with laparotomy. More recently, endovascular stenting has been described in several small series but have reported stent fracture, thrombosis, and migration. METHODS: We report the case of a 26-year-old woman with 4 months of intermittent flank pain and hematuria, diagnosed with nutcracker syndrome by both duplex ultrasound and axial based imaging. RESULTS: The patient underwent catheter venography confirming left renal vein compression, which also demonstrated a dilated gonadal vein measuring 11 mm leading to significant pelvic varices. Through a left lower quadrant retroperitoneal exposure, the gonadal vein was transposed to the left common iliac vein with completion venography demonstrating relief of renal venous congestion. The patient was discharged uneventfully with immediate resolution of symptoms and remains symptom-free at 6-month follow-up. CONCLUSIONS: Gonadal vein transposition is an effective alternative surgical treatment for nutcracker syndrome.

Venous Compression Syndromes.

Venous compression syndromes occur due to extrinsic compression causing complications of venous hypertension or venous thrombosis. This review focuses on 4 venous compression syndromes involving the left common iliac vein, subclavian vein, left renal vein, and popliteal vein. Clinical presentation, diagnostic methods, and management options are reviewed. When properly diagnosed and treated, long-term consequences can be avoided.

Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.

BACKGROUND: Nutcracker syndrome (NCS) describes left renal vein compression between the superior mesenteric artery and the aorta. Although uncommon, it is an important diagnosis due to the important morbidity associated with it, including the risk of chronic kidney disease from long-term left renal vein (LRV) hypertension and the risk of LRV thrombosis. METHODS: This article reviews the literature on NCS, particularly with respect to the diagnostic accuracy of different imaging modalities and the success rates, complications, and long-term follow-up data associated with various surgical interventions. RESULTS AND DISCUSSION: The diagnosis of this condition is based on a stepwise work-up with history and clinical examination, followed by Doppler ultrasonography, computed tomography, magnetic resonance imaging, intravascular ultrasound (IVUS) and phlebography with measurement of the renocaval pressure gradient. Management is determined by symptom severity; often symptom resolution occurs following a conservative approach. However, in some cases, surgical management is required, particularly when conservative management is unsuccessful. When it comes to the surgical management of NCS three main pathways exist: open surgery, laparoscopic surgery and endovascular approaches, with the latter 2 becoming increasingly popular due to their minimal invasiveness. Additionally, cases involving the use of robotic surgery in the management of NCS have been reported. CONCLUSION: Despite the rarity of NCS, its recognition and management are important. This article has explored the evidence basis for conservative, medical and surgical options.