Síndrome de cascanueces: una causa infrecuente de hematuria / Nutcracker Syndrome: a Rare Cause of Hematuria

La hematuria en pediatría responde habitualmente a etiologías benignas. Una causa poco frecuente es el síndrome de cascanueces, que se define como la compresión de la vena renal izquierda entre la aorta y la arteria mesentérica superior, que deriva en una presión elevada en la vena renal izquierda con el desarrollo de venas colaterales y dilataciones varicosas. La prevalencia de este síndrome se desconoce. Cuando es sintomático ocasiona hematuria, proteinuria y dolor pélvico crónico. En la pubertad, el crecimiento rápido y el desarrollo de los cuerpos vertebrales pueden producir un estrechamiento del ángulo entre la aorta y la arteria mesentérica superior. Se describe el caso de una adolescente con diagnóstico de carcinoma mucoepitelial metastásico óseo múltiple que presenta hematuria anemizante. Se arribó al diagnóstico de síndrome de cascanueces y se discutieron las opciones de tratamiento. Finalmente, con el uso de realce del calzado para corrección de la escoliosis, se atenuó significativamente la hematuria. (AU)

In pediatrics, hematuria usually responds to benign etiologies. A rare cause is nutcracker syndrome, defined as compression of the left renal vein between the aorta and the superior mesenteric artery, resulting in elevated pressure in the left renal vein with the development of collateral veins and varicose dilatation. The prevalence of this syndrome is unknown. When symptomatic, it causes hematuria, proteinuria, and chronic pelvic pain. At puberty, the rapid growth and development of the vertebral bodies can produce a narrowing of the angle between the aorta and the superior mesenteric artery.We describe the case of a teenage girl diagnosed with multiple metastatic mucoepithelial carcinoma of bone who presented anemia-producing hematuria. The diagnosis of nutcracker syndrome was arrived at with consideration of the therapeutic options. Finally, with shoe enhancement for scoliosis correction, hematuria was significantly lessened. (AU)

Causa rara de dolor abdominal recurrente; coexistencia del síndrome de Wilkie y el síndrome del cascanueces / A rare cause of recurrent abdominal pain; the coexistence of Wilkie's syndrome and nutcracker syndrome

El síndrome del cascanueces es un síndrome que presenta síntomas clínicos como hematuria, proteinuria ortostática, congestión pélvica, varicocele del lado izquierdo, hipertensión y dolor en fosa renal. Estos síntomas se producen por la compresión de la vena renal izquierda entre la aorta y la arteria mesentérica superior. En el síndrome de Wilkie, la tercera porción del duodeno está comprimida entre la arteria mesentérica superior y la aorta abdominal, lo que provoca diversos síntomas gastrointestinales. La coexistencia de estos dos síndromes constituye una afección rara y se incluye como casos clínicos en la bibliografía. En este artículo, se presentan los resultados clínicos y radiológicos de un paciente de 17 años que presentaba dolor abdominal recurrente debido al síndrome de Wilkie, acompañado del síndrome del cascanueces que le provocaba proteinuria, por lo que el paciente fue derivado a los consultorios externos de reumatología pediátrica con un diagnóstico preliminar de fiebre mediterránea familiar.

Nutcracker syndrome is a syndrome that has clinical symptoms such as hematuria, orthostatic proteinuria, pelvic congestion, left-sided varicocele, hypertension, and flank pain. These symptoms occur because of the compression of the left renal vein between the aorta and the superior mesenteric artery. In Wilkie's syndrome, the third part of the duodenum is compressed between the superior mesenteric artery and the abdominal aorta, causing various gastrointestinal symptoms. The coexistence of these two syndromes is a rare condition and is included as case reports in the literature. This article presents the clinical and radiological results of a 17-year-old male patient who had recurrent abdominal pain due to Wilkie's syndrome, which was accompanied by nutcracker syndrome that caused proteinuria, and for this reason, the patient was referred to the Pediatric Rheumatology outpatient clinic with a preliminary diagnosis of familial Mediterranean fever.

Caracterización del síndrome de cascanueces / Characterization of Nutcracker Syndrome

Introducción: El síndrome de cascanueces es una anomalía vascular poco buscada y diagnosticada, por su baja incidencia y por la dificultad de sospecharla ante manifestaciones clínicas frecuentes. Objetivo: Examinar las características clínicas e imagenológicas de los pacientes diagnosticados con el síndrome de cascanueces. Método: Estudio descriptivo retrospectivo y prospectivo en pacientes atendidos en el Hospital Pediátrico Universitario William Soler entre enero de 2013 - diciembre de 2017. Cuando se sospechó el síndrome se realizó ultrasonido buscando la dilatación de la vena renal izquierda y el ángulo de salida de la arteria mesentérica superior. En 7 de los pacientes se realizó angio-TAC. Se revisaron variables epidemiológicas, clínicas, de laboratorio e imagenológicas. Presentación de los casos: Del total de 9 pacientes en que se diagnosticó la anomalía vascular, 6 (66,7 por ciento) pertenecen al sexo masculino. La edad media al diagnóstico fue 11,4 años (rango: 7-15). Se diagnosticó cascanueces anterior en 7 (77,7 por ciento), posterior en 1 (11,1 por ciento) y síndrome de Wilkie con fenómeno cascanueces asociado en 1 (11,1 por ciento). En el 100 por ciento de los casos hubo dolor abdominal o lumbar, asociado a hematuria en 4, orinas espumosas en 4 y varicocele izquierdo en 2 de los 6 varones (33,3 por ciento). Conclusiones: El síndrome de cascanueces debe buscarse en los pacientes que presentan dolor abdominal, lumbar o en flanco izquierdo en forma recurrente, episodios de hematuria recurrente o proteinuria ortostática. En el varón con varicocele izquierdo debe descartarse esta posibilidad. El fenómeno cascanueces, puede diagnosticarse cuando se investiga al paciente por causa no relacionada(AU)

Introduction: Nutcracker syndrome (NCS) is a vascular anomaly which is rarely searched and diagnosed due to its low incidence and because of the difficulty to suspect on it among other frequent clinical manifestations. Objective: To examine clinical and imaging characteristics of patients diagnosed with NCS. Methods: Descriptive, retrospective and prospective study in patients admitted in William Soler University Pediatric Hospital in the period from January, 2013 to December, 2017. When it was suspected the presence of the syndrome, it was made an ultrasound looking for dilation of the left renal vein and the output angle of the superior mesenteric artery. In 7 patients it was made an angio-TAC. The epidemiologic, clinical, laboratory and imaging variables were reviewed. Presentation of cases: From the total of 9 patients diagnosed with a vascular anomaly; 6 (66,7 percent) were males. The mean age was 11,4 years (range 7-15 years). It was diagnosed front NCS in 7 patients (77.7 percent), back NCS in 1 patient (11.1 percent) and Wilkie syndrome with associated NCS in 1 patient (11.1 percent). 100 percent of the cases presented abdominal or lumbar pain related to hematuria (4 patients), foaming urine (4 patients) and left varicocele in 2 of the male patients (33.3 percent). Conclusions: Nutcracker syndrome should be searched in patients presenting frequent abdominal, lumbar or left side pain, recurrent hematuria or orthostatic proteinuria episodes. In males with left varicocele should be discounted this possibility. Nutcracker phenomenon can be also diagnosed when the patient is being investigated due to not related causes(AU)

Hematúria levando ao diagnóstico de duas condições singulares na prática clínica - síndrome de quebra-nozes e traço falcêmico: um relato de caso / Hematuria leading to the diagnosis of two singular conditions in clinical practice - renal nutcracker syndrome and sickle cell trait: a case report

RESUMO: A hematúria é definida como o achado de mais que duas hemácias por campo de aumento na análise microscópica de urina coletada por jato médio. Na sua forma macroscópica, caracteriza-se por sua coloração típica (avermelhada ou marrom), acompanhada pela presença de mais de 106 hemácias/ml na sedimentoscopia. É uma condição que necessita de investigação da causa e apresenta-se com prevalência incerta e definição às vezes imprecisa, mas com vasto diagnóstico diferencial. Dentre as possíveis causas, encontramos a presença do traço falciforme, aparentemente subdiagnosticado, e a síndrome de quebra-nozes, possibilidade mais rara. No caso relatado a seguir, foram diagnosticadas simultaneamente as duas causas acima citadas, descobertas após a investigação do primeiro episódio de hematúria macroscópica em uma mulher jovem previamente hígida. Com o objetivo de chamar a atenção para a concomitância de duas possíveis e incomuns causas de hematúria numa mesma paciente, relatamos o caso a seguir. (AU)

ABSTRACT: Hematuria is defined as the finding more than two red blood cells per field of analysis in the microscopic analysis of the urine collected by the medium jet. In its macroscopic form, it is characterized by its typical coloration (reddish or brown), accompanied by the presence of more than 106 red cells/ml in urinary sediment. It is a condition that needs investigation and it presents itself sometimes with imprecise definition, but with vast differential diagnosis. Among the possible etiologies, there is the presence of the sickle cell trait, apparently underdiagnosed, and the renal nutcracker syndrome, a rarer possibility. In the case reported below, these two findings were diagnosed simultaneously, following an investigation of the first episode of macroscopic hematuria in a young and previously healthy woman. In order to draw attention to the simultaneous presence of two unusual causes of hematuria in the same patient, we report the following case. (AU)

Tratamento endovascular da síndrome do quebra-nozes em adolescente: relato de caso / Endovascular treatment of a teenager with nutcracker syndrome: a case report

Resumo A síndrome do quebra-nozes (ou síndrome de nutcracker) é causada pela compressão da veia renal esquerda pela artéria mesentérica superior e aorta, e está associada a uma sintomatologia característica, como dor no baixo ventre, varicocele e hematúria. O diagnóstico é frequentemente difícil e, portanto, demorado. O tratamento invasivo é controverso, especialmente nos pacientes pediátricos; no entanto, em casos de hematúria severa associada a anemia, insuficiência renal funcional, severa dor pélvica ou ineficácia de tratamento conservador, ele é indicado. É relatado o caso de uma criança do sexo masculino, 12 anos, com quadro de hematúria maciça por 12 horas, sem evidências de alterações à investigação inicial, que evoluiu com anemia intensa e retenção urinária. Investigações futuras evidenciaram imagens sugestivas da síndrome de nutcracker e foi optado pelo tratamento endovascular por implante de stent smart control seguido de balonamento. Paciente cessou a hematúria após o procedimento e permanece assintomático há 5 anos.

Abstract The nutcracker syndrome is caused by compression of the left renal vein by the superior mesenteric artery and aorta and is associated with characteristic symptoms, such as lower abdominal pain, varicocele, and hematuria. Diagnosis is often difficult and, therefore, is often delayed. Invasive treatment is controversial, particularly in pediatric patients. However, it is indicated in cases of gross hematuria associated with anemia, renal function impairment, severe pelvic pain, or ineffective conservative treatment. We report the case of a 12-year-old boy presenting with severe hematuria for 12 hours, with no abnormal findings at a first evaluation, who progressed with severe anemia and urinary retention. Further investigation provided images suggestive of nutcracker syndrome, and endovascular stenting (smart control stent) followed by balloon dilatation was the treatment of choice. Hematuria ceased after the procedure, and the patient is still asymptomatic at 5-year follow-up.

Compressões das veias renal e ilíaca comum esquerdas em tomografias computadorizadas: com que frequência são detectadas? / Compression of left renal vein and left common iliac vein on CT scans: how often are they detected?

Resumo Contexto As síndromes de nutcracker e May-Thurner são raras e, apesar de muitas vezes subdiagnosticadas, podem causar sintomas limitantes de gravidade variável. Frequentemente são consideradas diagnóstico de exclusão e não há consenso na literatura quanto a prevalência, incidência e critérios diagnósticos. Objetivos Estimar a frequência da compressão das veias ilíaca comum e renal esquerdas em tomografias computadorizadas de abdome e pelve. Métodos Estudo descritivo, quantitativo e transversal. Para veia renal esquerda, foram considerados como critérios de compressão a relação diâmetro hilar/aortomesentérico > 4 e o ângulo aortomesentérico < 39° e, para veia ilíaca comum esquerda, o diâmetro < 4 mm. Resultados Foram analisadas tomografias computadorizadas de 95 pacientes; destes, 61% eram mulheres e 39% eram homens. A compressão da veia renal esquerda foi encontrada em 24,2% da amostra, com idade média de 48,8 anos, ocorrendo em 27,6% das mulheres e 18,9% dos homens (p = 0,3366). A compressão da veia ilíaca comum esquerda foi detectada em 15,7% da amostra, com idade média de 45,9 anos, ocorrendo em 24,10% das mulheres e 2,7% dos homens (p = 0,0024). Em 7,4% dos pacientes, ambas compressões venosas foram detectadas. Conclusões A compressão da veia renal esquerda ocorreu em mulheres e homens com frequência semelhante, enquanto a compressão da veia ilíaca comum esquerda foi mais frequente em mulheres. Ambas as compressões venosas foram mais frequentemente encontradas em pacientes com idade entre 41 e 50 anos.

Abstract Background The nutcracker and May-Thurner syndromes are rare and, although often underdiagnosed, they can cause limiting symptoms. They are frequently considered only after exclusion of other diagnoses and there is no consensus in the literature on prevalence, incidence, or diagnostic criteria. Objectives To estimate the frequency of compression of the left common iliac vein and left renal vein in CT scans of the abdomen and pelvis. Methods Descriptive, quantitative, cross-sectional study. The criteria used to define compression of the left renal vein were a hilar/aortomesenteric diameter ratio > 4 and aortomesenteric angle < 39° and the criterion for compression of the left common iliac vein was a diameter < 4mm. Results CT scans of 95 patients were analyzed; 61% were women and 39% were men. Left renal vein compression was observed in 24.2% of the sample, with a mean age of 48.8 years, occurring in 27.6% of the women and 18.9% of the men (p = 0.3366). Compression of the left common iliac vein was detected in 15.7% of the sample, with a mean age of 45.9 years, occurring in 24.1% of the women and 2.7% of the men (p = 0.0024). Both veins were compressed in 7.4% of the patients. Conclusions Left renal vein compression was detected in women and men at similar frequencies, whereas left common iliac vein compression was more frequent in women. Both venous compressions were most frequently found in patients aged 41 to 50 years.

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.

A minimally invasive alternative for the treatment of nutcracker syndrome using individualized three-dimensional printed extravascular titanium stents / 中华医学杂志(英文版)

BACKGROUND@#This study was designed to evaluate the clinical and radiographic outcomes of patients with nutcracker syndrome (NCS) who were treated with three-dimensional printing (3DP) extravascular titanium stents (EVTSs). The 3DP EVTS was expected to release the hypertension of the left renal vein (LRV) produced by its compression between the superior mesenteric artery (SMA) and the aorta without causing any complications.@*METHOD@#The pre-operative kidney model of each patient was printed out to enable surgical planning. After that, the EVTS was designed based on the LRV's primitive physiologic structure using computer-aided design software, and each stent was printed out with a precision setting of 20 μm. Seventeen patients who had been suffering from NCS underwent laparoscopic 3DP EVTS placement. The surgical procedure was designed for the placement of EVTS, taking great care in positioning and fixing the stent. Surgical data, which included patient demographic characteristics as well as pre- and post-operative test results, were collected and analyzed.@*RESULTS@#The mean duration of surgery was 75 ± 9 min, and the mean blood loss was 20 ± 5 mL. Computed tomography examinations revealed that the pre- and post-operative angle between the SMA and the aorta ranged from 18.7° ± 4.3° to 48.0° ± 8.8° (P < 0.05); in patients with left varicocele, the mean diameter of the left spermatic vein ranged from 3.7 ± 0.5 to 1.3 ± 0.2 mm (P < 0.05). Moreover, Doppler ultrasound examinations showed that the peak velocity of blood flow at the hilar area ranged from 12.4 ± 3.3 to 18.5 ± 3.4 cm/s (P < 0.05). No side effects were observed in the 24 to 42 months following surgery.@*CONCLUSION@#The findings after 2 years of follow-up suggest that the 3DP EVTS is a safe and effective minimally invasive alternative for the treatment of NCS.

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

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

Síndrome de cascanueces en combinación con nefropatía IgA: causa de hematuria y proteinuria persistentes. Reporte de un caso y revisión de la literatura / Nutcracker syndrome combined with IgA Nephropathy: a cause of recurrent hematuria and proteinuria. Case report

RESUMEN El síndrome de cascanueces se produce por la obstrucción de la vena renal izquierda, debido al atrapamiento entre la aorta y la arteria mesentérica superior. Se manifiesta por dolor, hematuria, varicocele o síntomas urinarios bajos o pelvianos. La prevalencia de este síndrome es desconocida, pero se ha incrementado con el advenimiento de estudios no invasivos para su diagnóstico. Por su parte, la nefropatía IgA es la glomerulopatía más común en el mundo y representa cerca del 15 % de las causas de hematuria en niños. La combinación de nefropatía IgA y el síndrome de cascanueces es inusual, con pocos casos descritos en la literatura. Describimos el caso de un escolar de 9 años con hematuria y proteinuria persistente en el curso de una púrpura de Henoch-Schönlein, en quien se realiza una biopsia renal documentando nefropatía IgA. Sin embargo, durante el seguimiento, por la persistencia de hematuria y proteinuria aunadas a otros síntomas, se realizan estudios adicionales que confirman un síndrome de cascanueces. Es clave en los pacientes con la persistencia de estos síntomas descartar la asociación de estas dos enfermedades para evitar intervenciones innecesarias.

SUMMARY The nutcracker syndrome is caused by the obstruction of the left renal vein secondary to its compression between the superior mesenteric artery and the aorta. Clinically, this syndrome manifests with pain, hematuria, varicocele or lower urinary tract symptoms. The prevalence of this syndrome is currently unknown; however, the diagnosis of this condition has increased thanks to the availability of non-invasive studies that allow its recognition. On the other hand, IgA nephropathy is the most common type of glomerular disease worldwide. Almost 15% of the causes of hematuria in children are secondary to this condition. The combination of IgA nephropathy and the nutcracker syndrome is rare. In the current literature, few cases have been described. We described the case of a 9-year-old scholar with hematuria and persistent proteinuria in the course of a purpura of Henoch-Schonlein in whom renal biopsy documented IgA nephropathy. However, during follow-up, due to the persistence of hematuria and proteinuria, in addition to other symptoms, additional studies are carried out confirming a Nutcracker syndrome. It is key in patients with persistent symptoms to rule out the association of these two diseases to avoid unnecessary interventions.

Síndrome renal del cascanueces / Nutcracker syndrome

El síndrome renal del cascanueces es una enfermedad rara, más frecuente en mujeres entre la tercera y cuarta décadas de la vida. Consiste en la compresión de la vena renal izquierda, entre la arteria mesentérica superior y la aorta abdominal, que provoca un aumento en el gradiente presión de la vena renal izquierda y produce hematuria renal unilateral izquierda. Se presenta una paciente de 55 años de edad con antecedentes patológicos personales de hipertensión arterial hace 15 años, que comienza con cuadro de dolor lumbar izquierdo, asociado a hematuria microscópica y leucocituria. Se interpreta como infección del tracto urinario, recibió tratamiento antibiótico por 10 días, aunque con persistencia de la hematuria, sin otra manifestación clínica. Se le realizó angio-TAC (fase simple, arterial, venosa, excretora) que mostró arterias únicas y normales. Existe ligera compresión de la vena renal izquierda, por disminución del ángulo aorto mesentérico (ángulo 25°), compatible con síndrome renal del cascanueces. Se presenta este caso, dado lo poco frecuente del reporte de este síndrome en Cuba(AU)

Nutcracker syndrome is a rare disease, more frequent in in women between the third and fourth decades of life. It consists of the compression of the left renal vein between the superior mesenteric artery and the abdominal aorta, causing an increase in the pressure gradient of the left renal vein and producing left unilateral renal hematuria. We present a 55-year-old patient with a personal pathological history of arterial hypertension for 15 years, who began with left lumbar pain, associated with microscopic hematuria and leukocyturia. It was interpreted as a urinary tract infection, the patient received antibiotic treatment for 10 days , although hematuria continued, until now, with no other clinical manifestation. The patient underwent a angio-CT (simple, arterial, venous, excretory phase) that showed single and normal arteries. There is slight compression of the left renal vein, due to reduction of the mesenteric aorto angle (angle 25°), compatible with renal nutcracker syndrome. We present this case since this syndrome is rare in Cuba(AU)

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

Microscopic spermatic vein ligation for nutcracker phenomenon complicated with left varicocele / 中华男科学杂志

Objective@#To investigate the clinical effect of microscopic spermatic vein ligation in the treatment of nutcracker phenomenon (NCP) complicated with left varicocele (VC).@*METHODS@#This retrospective study included 31 cases of NCP complicated with left VC treated in our hospital by subinguinal microscopic ligation of the left spermatic vein (group A, n = 11), open retroperitoneal high ligation of the left spermatic vein (group B, n = 11), or conservative therapy (group C, n = 9). The patients were followed up for 6－24 (15.3 ± 5.4) months. We compared the semen parameters, spermatic vein diameter, left testis volume, and recurrence rate among the three groups of patients before and after treatment.@*RESULTS@#Compared with the baseline, the semen quality parameters were significantly improved in both groups A and B at 6 months after treatment (P0.05), B (［9.77 ± 2.03］ vs ［9.96 ± 1.72］ ml, P>0.05), or C (［9.83 ± 1.59］ vs ［10.48 ± 2.05］ ml, P>0.05), nor in the recurrence rate between groups A and B (P>0.05).@*CONCLUSIONS@#Hematuria, proteinuria and other mild symptoms of nutcracker phenomenon complicated with left VC can be treated palliatively by microscopic ligation of the spermatic vein, which can relieve the clinical symptoms, improve the semen quality, and protect the testicular function of the patient.

Microsurgical bypass for varicocele with nutcracker syndrome / 中华男科学杂志

Objective@#To investigate the clinical effect and feasibility of internal spermatic vein-inferior epigastric vein (ISV-IEV) bypass surgery in the treatment of varicocele complicated by left renal vein nutcracker syndrome (NCS).@*METHODS@#We retrospectively analyzed the clinical data about 30 cases of varicocele with left renal vein NCS treated by ISV-IEV bypass surgery in our hospital from June 2014 to February 2017. We reviewed the follow-up data and results of ultrasonography, routine urianlysis and semen routine examination.@*RESULTS@#All the operations were successfully accomplished and postoperative color Doppler ultrasonography showed that varicocele was cured in all the cases. At 6 months after surgery, sperm concentration and the percentage of grade a+b sperm were significantly improved (［34.47 ± 8.60］ ×10⁶/ml and ［63.54% ± 9.58］ %) as compared with the baseline (［19.90 ± 8.97］ ×10⁶/ml and ［37.93 ± 8.73］ %) (P <0.05). Hematuria was cured in 23 and alleviated in 1 of the 24 cases. Proteinuria disappeared in all the 14 cases, with neither scrotal pain symptoms nor obvious complications.@*CONCLUSIONS@#ISV-IEV bypass surgery, with its advantages of safety, effectiveness, minimal invasiveness, and simple operation, deserves wide clinical application in the treatment of varicocele with left renal vein NCS.

Superior Mesenteric Artery Syndrome Combined with Renal Nutcracker Syndrome in a Young Male: A Case Report / 대한소화기학회지

Superior mesenteric artery (SMA) syndrome is one of the rare causes of small bowel obstruction. It develops following a marked decrease in the angle between SMA and the abdominal aorta due to weight loss, anatomical anomalies, or following surgeries. Nutcracker syndrome in the left renal vein may also occur following a decrease in the aortomesenteric angle. Though SMA syndrome and renal nutcracker syndrome share the same pathogenesis, concurrent development has rarely been reported. Herein, we report a 23-year-old healthy male diagnosed with SMA syndrome and renal nutcracker syndrome due to severe weight reduction. The patient visited our outpatient clinic presenting bilious vomiting and indigested vomitus for 3 consecutive days. He had lost 20 kg during military service. We suspected SMA syndrome based on abnormal air-shadow in the stomach and small bowel on abdominal X-ray; we confirmed compression of the third portion of the duodenum with upper gastrointestinal series and abdominal computed tomography (CT). Concurrently, renal nutcracker syndrome was also detected via abdominal CT and Doppler ultrasound. Considering bilious vomiting and no urinary symptoms, SMA syndrome was corrected by laparoscopic duodenojejunostomy, and close observation for the renal nutcracker syndrome was recommended.

Síndrome del cascanueces anterior y posterior acompanado de vena renal izquierda circumaórtica en un adolescente: A propósito de un caso / Anterior and posterior nutcracker syndrome accompanying left circumaortic renal vein in an adolescent: Case report

La vena renal izquierda (VRI) presenta diversas variantes en su desarrollo; las dos más frecuentes son la circumaórtica y la retroaórtica. El síndrome del cascanueces anterior es la compresión de la VRI entre la aorta y la arteria mesentérica superior, mientras que el síndrome del cascanueces posterior se produce entre la columna vertebral y la aorta. Derivaron a un varón adolescente (16 años de edad) a la sala de emergencias debido a dolor en una de las fosas renales. En la tomografía computada se halló la combinación de síndrome del cascanueces anterior y posterior en la vena renal izquierda circumaórtica, circunstancia nunca antes descrita en un adolescente.

The left renal vein (LRV) has many developmental variations; the two most common are the circumaortic and the retrocaval. Anterior nutcracker syndrome is the compression of the LRV between the aorta and superior mesenteric artery, whereas posterior nutcracker syndrome occurs between the vertebral column and the aorta. An adolescent male (aged 16 years) was referred to the emergency department for flank pain. CT findings showed the combination of anterior and posterior nutcracker syndrome in the left circumaortic renal vein, which has not previously been described in an adolescent.

Síndrome del ligamento arcuato medio concomitante con síndrome de cascanueces. Presentación de un caso / Concomitant Medium Arcuate Ligament Syndrome with Nutcracker Syndrome. A Case Report

El síndrome del ligamento arcuato medio y el síndrome de cascanueces son entidades raras, con baja prevalencia, a menudo subdiagnosticadas y asintomáticas en la mayoría de las ocasiones. Se presenta el caso de una paciente con ambas entidades, lo cual probablemente le genera dolor abdominal como síntoma común.

The Arcuate ligament syndrome and the Nutcracker syndrome are rare disorders, with low prevalence, commonly underdiagnosed and asymptomatic in most cases. We present the case of a female patient with both syndromes, which probably causes her abdominal pain as a common symptom.