Venous Compressive Disorders.

Venous compressive disorders are a heterogenous group of vascular syndromes characterized by extrinsic venous compression that can lead to complications of venous hypertension or venous thrombosis. Endovascular damage secondary to deep venous thrombosis (DVT) can result in post-thrombotic syndrome (PTS), a potentially debilitating condition that can be associated with significant morbidity in the pediatric population. Here we discuss 4 venous compressive disorders: iliac vein compression (May-Thurner syndrome [MTS]); subclavian vein compression at the venous thoracic inlet (Paget-Schroetter syndrome); left renal vein compression (nutcracker syndrome); and popliteal vein compression (popliteal entrapment syndrome) with a focus on clinical evaluation and diagnostic methods. Where endovascular therapy is appropriate, specific procedural considerations including procedure indications, equipment, procedural steps, technical challenges, complications, clinical follow-up and expected outcomes are discussed.

Outcomes of conservative management in patients with nutcracker syndrome.

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.

A systematic review on nutcracker syndrome and proposed diagnostic algorithm.

OBJECTIVE: Nutcracker syndrome (NCS) is an increasingly recognized venous compressive disease. Although people with NCS can experience debilitating symptoms, making an accurate diagnosis can often be challenging owing to the broad spectrum of clinical presentations and radiologic findings. In the present systematic review, we assessed the most frequent clinical and radiologic criteria used in establishing a diagnosis of NCS and have proposed a comprehensive framework for clinical decision-making. METHODS: We performed a systematic review to identify all observational studies or case series conducted within the previous 10 years that had studied patients with a clinical and radiologic diagnosis of NCS. The extracted data included details related to the study design, participant demographics, presenting clinical features, and radiologic criteria. These details were compared between studies and synthesized to establish a comprehensive diagnostic framework that would be applicable to clinical practice. RESULTS: In the present review, we included 14 studies with a total of 384 patients with NCS. The most common clinical features of NCS were hematuria (69.5%), left flank or abdominal pain (48.4%), pelvic pain (23.1%), and varicocele (15.8%). Computed tomography and ultrasound were the most commonly used imaging modalities, with a threshold for left renal vein stenosis of >80% the most frequently used diagnostic parameter. Eight studies had used venography, with the renocaval pressure gradient the most commonly measured parameter. Two studies had reported using intravascular ultrasound. The findings from our review have shown that a thorough clinical workup of NCS should include critical evaluation of the presenting clinical features and exclusion of alternative diagnoses. All patients should undergo duplex ultrasound with or without the addition of computed tomography or magnetic resonance imaging. Any patient considered for therapeutic intervention should also undergo diagnostic venography with measurement of the renocaval pressure gradient and, if available, intravascular ultrasound with measurement of the left renal vein diameters. CONCLUSIONS: NCS is a challenging condition in terms of the diagnosis and management both. We have proposed a comprehensive diagnostic framework based on the currently available literature to aid in clinical decision-making.

The Journey and Barriers to Treatment of Patients With Renal Nutcracker Syndrome.

OBJECTIVE: To better understand renal nutcracker syndrome (NCS) from a patients' perspective starting at presentation and followed through to diagnosis and management METHODS: This descriptive study was conducted on a national level via a self-selected online survey distributed via river sampling by a post on the Facebook Page 'Renal Nutcracker Syndrome Support Group.' RESULTS: Of the 22 responses collected, 95.5% were female and 91% self-identified as White. 43% experienced symptoms as teenagers and 62% were diagnosed as young adults. Prior to receiving a definitive diagnosis, over half of the respondents were worked up for kidney stones (57%) and ovarian cysts (48%) and saw at least 10-15 providers. Nearly 80% experienced constant pain throughout the day. Pain management included prescription oral pain relievers (38%), prescription patches (29%), and physical therapy (19%). Surgical procedures included nephrectomy with auto transplant (38%), left renal vein transposition (10%), and laparoscopic extravascular stent placement (10%). Respondents had high healthcare utilization for management of NCS. Nearly 30% were unable to work and had filed for disability. CONCLUSION: Awareness of NCS should increase among healthcare providers of all specialties to improve quality of care to those living with NCS. It is crucial to keep NCS within the differential diagnosis in patients presenting with gross hematuria and unusual abdominal and/or flank pain.

Role of physical exercise in an overlooked nutcracker syndrome occurred in a patient with diaphragmatic relaxation: a case report.

Background: Nutcracker syndrome (NCS) is caused by extrinsic compression of the left renal vein (LRV), usually between the abdominal aorta (AA) and superior mesenteric artery (SMA). This rare disease includes symptoms such as hematuria, left flank pain or abdominal pain, varicocele in males, proteinuria, anemia, gynecological symptoms (dyspareunia, dysmenorrhea). Case report: We report the case of a 48-year-old female patient, who experienced left abdominal colic after intensive physical exercise, finally resulting in a diagnosis of NCS. This abdominal pain was disabling for daily activities, it was controlled by analgesic drugs and led to hospital admissions. In-depth examinations were recommended to the patient to investigate the etiology of these attacks. A bad rotated and ectopic left kidney, which was located superior to the spleen, at the level of the left hemithorax base, was found due to the presence of a diaphragmatic relaxation in the posterior area, which caused an upward displacement of the kidney, part of the colon and omental fat. Because of the presence of a compression of the LRV by the SMA and the AA, the nephrologist diagnosed a NCS, presenting with abdominal pain following physical exercise, proteinuria and dysmenorrhea. Conservative treatment was chosen for the patient. Conclusions: The patient was recommended to engage in a moderate and regular physical activity, avoiding acute and intense exercise: hypopressive abdominal gymnastics was suggested. The role of physical exercise in triggering painful attacks and its role in rehabilitation to prevent the same attacks was crucial for the patient.

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.

Two Peculiarities of the Nutcracker Syndrome: Rare Association with Multiples Thrombosis and Successful Medical Treatment (Pentoxifylline).

The nutcracker syndrome (NCS) is a rare condition characterized by the entrapment of the left renal vein between the superior mesenteric artery and the aorta. Clinically, it presents with hematuria, flank pain, and symptoms of pelvic venous congestion. It is more frequent in females. Conservative treatment is usually proposed children but in adults, NCS has to be treated by surgical or endovascular methods. Drug therapy is not proposed for the treatment of NCS. We report a case of a young patient who presented with recurrent gross hematuria. Our patient was treated with pentoxifylline three times daily and anti-coagulation, with a favorable outcome.

Combined Superior Mesenteric Artery Syndrome and Nutcracker Syndrome in a Young Patient: A Case Report and Review of the Literature.

BACKGROUND Superior mesenteric artery syndrome is the compression of the third portion of the duodenum between the superior mesenteric artery and the aorta causing abdominal pain and vomiting. Nutcracker syndrome is the compression of the left renal vein between the superior mesenteric artery and the aorta causing symptoms related to renal congestion. Both entities, although well described in the literature, are individually rare, and even though they might share a common etiology, their co-existence has been reported in only a few case reports. CASE REPORT An 18-year-old male, previously healthy, presented with postprandial abdominal pain relieved by bilious vomiting that started shortly after he lost weight fasting. Our investigation revealed superior mesenteric artery syndrome as well as a compressed left renal vein. He was started on an enriched fluid diet which was progressed gradually as he regained weight. His left renal vein compression at the time was asymptomatic; it will be followed up for possible resolution after the patient's weight returns to normal. CONCLUSIONS Superior mesenteric artery syndrome is to be suspected in patients with abdominal pain following weight loss. Conservative treatment with a focus on weight regain will cure most cases. Asymptomatic or mildly symptomatic nutcracker syndrome is treated conservatively. For patients requiring intervention, laparoscopic extravascular titanium stent placement seems to be the least invasive promising option today, awaiting further definitive studies.

Nutcracker Syndrome-A Rare but Important Cause of Varicocele in Adolescent Boys.

Nutcracker syndrome is a complex of clinical symptoms due to compression of the left renal vein between abdominal aorta and superior mesenteric artery. Hematuria and pelvic or back pain are the most common presenting symptoms with varicocele being an uncommon clinical finding in these patients especially in children. Doppler ultrasound, computed tomography and selective left renal vein phlebography are useful to confirm the diagnosis. Treatment can be conservative or surgical depending on the severity of symptoms. Here, we report a 13-year-old boy with left flank pain and varicocele on examination who on evaluation was found to have nutcracker syndrome as the etiology.

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.

RENAL VEIN THROMBOSIS DUE TO POSTERIOR NUTCRACKER SYNDROME.

Thrombosis of the left renal vein (LRV) is a rare occurrence usually associated with hypercoagulable disorders. It may also be caused by extrinsic compression due to anatomical variations. Such variations, known as nutcracker phenomenon (NP), are usually completely asymptomatic, and they have been described in three variants: a narrowed aortomesenteric angle (AMA) with entrapment of the LRV; a second, rarer variant with an anomalous retro aortic LRV compressed between the abdominal aorta (AA) and the spine (posterior nutcracker syndrome, PNCS); and a third variant with a duplicated LRV with a pre- and retroaortic course, compressed both anteriorly and posteriorly. The development of symptoms secondary to NP is mainly due to renal congestion, often difficult to identify, and includes flank pain, ipsilateral varicocele, hematuria, and orthostatic proteinuria, among others. The most severe complication of NCS is LRV thrombosis with or without associated predisposing prothrombotic factors. The present case associates a PNCS to LRV thrombosis with a floating thrombus in the inferior vena cava and pulmonary embolism.

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.

[Nutcracker syndrome]. / Síndrome del cascanueces.

Nutcracker syndrome is a vascular anomaly consisting in the compression of the left renal vein between the superior mesenteric artery and the aorta. Clinical features in nutcracker syndrome include pelvic pain, flank pain, haematuria, gonadal varices or simply asymptomatic. We are presenting two cases, one of them with macroscopic haematuria and flank pain and the other was studied for hypertension but with previous antecedents of left renal vein embolization in the setting of varicocele. We discuss the clinical presentation as well as diagnostic and therapeutic aspects related to this syndrome.

El síndrome del cascanueces es una anomalía vascular en la que se comprime la vena renal izquierda a su paso entre la unión de la aorta y la arteria mesentérica superior, debido a un ángulo muy cerrado entre ambas arterias. Clínicamente puede presentarse como hematuria macro o microscópica, dolor episódico en flanco izquierdo, dolor pélvico, várices gonadales o simplemente cursar de forma asintomática. Presentamos dos casos clínicos, uno de ellos con dolor abdominal tipo cólico nefrítico y hematuria macroscópica y otro estudiado por hipertensión pero con antecedentes de embolización de la vena renal izquierda por varicocele izquierdo. Discutimos los aspectos clínicos, diagnósticos y terapéuticos de este síndrome.

Síndrome del cascanueces / Nutcracker syndrome

El síndrome del cascanueces es una anomalía vascular en la que se comprime la vena renal izquierda a su paso entre la unión de la aorta y la arteria mesentérica superior, debido a un ángulo muy cerrado entre ambas arterias. Clínicamente puede presentarse como hematuria macro o microscópica, dolor episódico en flanco izquierdo, dolor pélvico, várices gonadales o simplemente cursar de forma asintomática. Presentamos dos casos clínicos, uno de ellos con dolor abdominal tipo cólico nefrítico y hematuria macroscópica y otro estudiado por hipertensión pero con antecedentes de embolización de la vena renal izquierda por varicocele izquierdo. Discutimos los aspectos clínicos, diagnósticos y terapéuticos de este síndrome.

Nutcracker syndrome is a vascular anomaly consisting in the compression of the left renal vein between the superior mesenteric artery and the aorta. Clinical features in nutcracker syndrome include pelvic pain, flank pain, haematuria, gonadal varices or simply asymptomatic. We are presenting two cases, one of them with macroscopic haematuria and flank pain and the other was studied for hypertension but with previous antecedents of left renal vein embolization in the setting of varicocele. We discuss the clinical presentation as well as diagnostic and therapeutic aspects related to this 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.

Tratamento endovascular da síndrome de quebra-nozes: relato de caso / Endovascular treatment of Nutcracker syndrome: case report

A dor pélvica crônica é uma doença debilitante, com impacto na qualidade de vida e custos para os serviços de saúde. A síndrome de quebra-nozes é uma importante causa dessa dor, e se refere a um conjunto de sinais secundários à compressão da veia renal esquerda, mais comumente entre a artéria mesentérica superior e a aorta. Seu tratamento ainda permanece controverso e varia de acordo com a gravidade clínica do paciente. Contudo, a técnica endovascular com implante de stent em veia renal tem obtido excelentes resultados. Relatamos um caso de uma paciente de 59 anos submetida a correção endovascular com stent autoexpansível de nitinol. São apresentados dados clínicos, detalhes do procedimento e resultados do acompanhamento dessa paciente. O sucesso técnico foi obtido e não houve relato de complicações pós-operatórias. Pôde-se observar alívio dos sintomas e melhora nos exames de imagem realizados no acompanhamento de curto prazo

Chronic pelvic pain is a debilitating disease that directly impacts on quality of life and generates costs for health services. Nutcracker Syndrome is an important cause of pelvic pain and consists of a set of signs secondary to compression of the left renal vein, most commonly between the superior mesenteric artery and the aorta. Treatment remains controversial and varies depending on the patient's clinical severity. However, endovascular treatment with renal vein stenting has achieved excellent results. We report the case of a 59 year-old female treated by endovascular repair with a self-expanding nitinol stent. Clinical data, details of the procedure, and follow-up results are presented. Technical success was achieved and there patient reported no postoperative complications. Short-term, there was relief from symptoms and follow-up imaging tests showed improvement

Revascularização venosa via transposição de veia gonadal esquerda em síndrome de nutcracker posterior: relato de caso / Venous revascularization to treat posterior nutcracker syndrome by transposition of the left gonadal vein: case report

A síndrome de nutcracker é manifesta na presença de um aprisionamento sintomático da veia renal esquerda entre a aorta abdominal e a artéria mesentérica superior. Uma variação mais efêmera desta desordem é dita síndrome de nutcracker posterior, quando a compressão da veia renal não mais ocorre frontalmente à aorta, mas posteriormente a ela, entre esta e a coluna vertebral. A despeito de variáveis opções terapêuticas, as técnicas presentes visam aliviar os sintomas e diminuir a pressão venosa da veia renal esquerda. Este relato descreve um caso de Síndrome de nutcracker posterior, em que a abordagem de escolha foi a cirurgia aberta, transpondo distalmente a veia gonadal esquerda na veia cava inferior

The Nutcracker Syndrome is manifest in the presence of a symptomatic entrapment of the left renal vein between the abdominal aorta and the superior mesenteric artery. In a more ephemeral variation of this disorder, called the Posterior Nutcracker Syndrome, the renal vein is not compressed anterior to the aorta, but posteriorly, between the artery and the spine. Although there are multiple treatment options, current techniques aim to relieve the symptoms and reduce venous pressure on the left renal vein. This report describes a case of Posterior Nutcracker Syndrome in which the management approach chosen was open surgery, transposing the gonadal vein distally, to the inferior cava vein

Diagnóstico e tratamento da síndrome de quebra-nozes (nutcracker): revisão dos últimos 10 anos / Diagnosis and treatment of the Nutcracker syndrome: a review of the last 10 years

A síndrome de quebra-nozes é caracterizada por um grupo de manifestações clínicas que ocorrem por conta da compressão da veia renal esquerda. Seus principais sintomas são macro e micro-hematúria, proteinúria e dor no flanco. O diagnóstico é geralmente realizado após a exclusão de outras causas mais comuns, por conta da ausência de critérios clínicos para diagnóstico. Sua confirmação é feita por exames de imagem, com uso da ultrassonografia Doppler e tomografia computadorizada. O tratamento pode variar com as características do paciente e com a gravidade dos sintomas, e inclui o tratamento conservador, a cirurgia aberta e a cirurgia endovascular. Atualmente, a cirurgia aberta continua sendo a linha de frente, mas abordagens menos invasivas vêm ganhando cada vez mais espaço

The nutcracker syndrome is characterized by a group of clinical manifestations caused by compression of the Left Renal Vein. The main symptoms are: macro and micro hematuria, proteinuria, and flank pain. Diagnosis is usually made after excluding other causes, because there are no clinical criteria for diagnosis. Confirmation is by Doppler ultrasonography or computed tomography. Treatment can vary, depending on patient characteristics and the severity of the symptoms, while conservative treatment, open surgery, and endovascular surgery may be employed. Currently, open surgery is still the first-line treatment, but some less invasive approaches are gaining acceptance