Nutcracker syndrome: a case-based review.

The nutcracker phenomenon, also known as left renal vein entrapment, occurs when there is extrinsic compression of the left renal vein, most often between the abdominal aorta and the superior mesenteric artery. Nutcracker syndrome refers to the constellation of clinical symptoms that may arise from the nutcracker phenomenon, typically inclusive of haematuria, flank/pelvic pain, orthostatic proteinuria and (in male patients) varicocele. We provide a short review of the nutcracker syndrome including various diagnostic and therapeutic modalities. We utilise our own experience with a patient as a case study and highlight the modern management option of endovascular stenting.

Combined superior mesenteric artery syndrome and nutcraker syndrome presenting as acute pancreatitis: a case report.

Superior mesenteric artery syndrome designates compression of the third part of the duodenum between the superior mesenteric artery and the aorta. This condition has a low incidence, being more common in thin young women. Nutcracker syndrome is compression of the left renal vein between the superior mesenteric artery and the aorta. Both entities are rare, and their coexistence has been reported in a few cases. Conservative treatment targeting weight gain is sufficient in most cases. An association between the superior mesenteric artery syndrome and acute pancreatitis has rarely been reported. We intend to describe the case of an 18-year-old girl who was admitted to the emergency room with epigastric pain and emesis. Our investigation revealed acute acalculous pancreatitis. During work-up, we discovered superior mesenteric artery syndrome and a compressed left renal vein. The patient is on conservative treatment, and her symptoms have improved.

A síndrome da artéria mesentérica superior designa compressão da terceira parte do duodeno pela artéria mesentérica superior e a aorta. Essa condição tem uma baixa incidência, sendo mais comum em mulheres jovens magras. A síndrome de quebra-nozes resulta da compressão da veia renal esquerda pela artéria mesentérica superior e a aorta. Ambas as entidades são raras, e a sua coexistência foi descrita em poucos casos. Tratamento conservador com o objetivo de ganho ponderal é suficiente na maioria dos casos. A associação entre a síndrome da artéria mesentérica superior e a pancreatite aguda foi raramente relatada. Pretendemos descrever o caso de uma jovem de 18 anos que recorreu ao serviço de urgência com epigastralgia e vômitos. A investigação realizada revelou pancreatite aguda alitiásica. Durante o estudo complementar, foi identificada a síndrome da artéria mesentérica superior, bem como uma veia renal esquerda comprimida. A paciente encontra-se em tratamento conservador, apresentando melhora clínica.

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.

Robot-assisted extravascular stent for nutcracker syndrome: A case report.

Nutcracker syndrome (NCS) is a rare condition caused by compression of the left renal vein (LRV) between the aorta and superior mesenteric artery. Surgical treatment is reserved for patients with severe symptoms and failure of conservative treatment. A 31-year-old woman diagnosed in adolescence with NCS has had recurrent pain since age 15. For 30 days, she has had severe left flank pain and microhematuria. The patient underwent extravascular stent placement around the LRV with the Da Vinci Xi. In 30 months of follow-up, the patient has no pain and the LRV remained without compressions or thrombosis.

Wilkie's and Nutcracker's syndromes overlapping a case of functional dyspepsia

We present the case of a patient suffering from a mixed-type functional dyspepsia who markedly reduced his diet to improve his symptoms leading him to malnourishment and a subsequent Wilkie's and Nutcracker's syndromes which exacerbated his pain. Our aim by presenting this case is to raise awareness as to what extent a so-called functional dyspepsia can evolve and of the possible overlap with these two entities in case of severe malnutrition.

Presentamos el caso de un paciente diagnosticado de dispepsia funcional de tipo mixto, el cual redujo marcadamente su dieta para mejorar sus síntomas, llevándolo a una desnutrición y a un posterior síndrome de Wilkie y del Cascanueces que agudizó su dolor. Nuestro objetivo con la presentación de este caso es concienciar de hasta qué punto puede evolucionar una denominada dispepsia funcional y del posible solapamiento de estas dos entidades en caso de desnutrición severa.

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

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.

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.

Síndrome de cascanueces y agenesia renal derecha. Una rara asociación / Nutcracker Syndrome and Right Renal Agenesis. A Rare Association

Introducción: El síndrome de cascanueces se considera una anomalía infrecuente y poco pensada en la práctica médica; su incidencia no está bien establecida debido a su sintomatología variada. La agenesia renal unilateral se estima entre 1/2500 y 1/4000 nacidos vivos. Objetivos: Describir una paciente de 18 años con sospecha prenatal de agenesia renal derecha confirmada por imágenes después del nacimiento, que ingresó por proteinuria. Presentación del caso: Paciente de 18 años con agenesia renal derecha conocida, en la que un examen de orina detectó proteinuria desde los 13 años. En su estudio se clasificó como proteinuria ortostática y evolutivamente refirió dolor lumbar izquierdo ligero y transitorio. En el ultrasonido renal, realizado para valorar crecimiento del riñón único, se detectó dilatación de la vena renal izquierda. Se repitió el estudio ecográfico para precisar ángulo aorto-mesentérico y dilatación de vena renal izquierda, y se confirmó el síndrome de cascanueces. Conclusiones: La asociación entre agenesia renal derecha y síndrome de cascanueces, resulta extremadamente rara, y, cuando se presenta con proteinuria ortostática y dolor lumbar ocasional, debe seguirse en forma expectante, pero no se necesita tratamiento quirúrgico en la mayoría de los casos(AU)

Introduction: Nutcracker syndrome is considered an infrequent and poorly thought out anomaly in medical practice; its incidence is not well established due to its varied symptomatology. Unilateral renal agenesis is estimated to be between 1/2500 and 1/4000 live births. Objectives: To describe an 18-year-old female patient with suspected pre-natal imaging-confirmed right renal agenesis after birth, who was admitted due to proteinuria. Case presentation: An 18-year-old female patient with known right renal agenesis, in whom a urine test detected proteinuria from the age of 13. In the study it was classified as orthostatic proteinuria and evolutionarily she referred mild and transient left low back pain. Renal ultrasound, performed to assess single kidney growth, showed dilation of the left renal vein. The ultrasound study was repeated to specify aorto-mesenteric angle and left renal vein dilation, and nutcracker syndrome was confirmed. Conclusions: The association between right renal agenesis and nutcracker syndrome is extremely rare, and, when it presents with orthostatic proteinuria and occasional low back pain, it should be followed expectantly, but surgical treatment is not needed in most cases(AU)

Combined superior mesenteric artery syndrome and nutcraker syndrome presenting as acute pancreatitis: a case report / Síndrome da artéria mesentérica superior em combinação com síndrome de quebra-nozes manifestando-se como pancreatite aguda: relato de caso

Abstract Superior mesenteric artery syndrome designates compression of the third part of the duodenum between the superior mesenteric artery and the aorta. This condition has a low incidence, being more common in thin young women. Nutcracker syndrome is compression of the left renal vein between the superior mesenteric artery and the aorta. Both entities are rare, and their coexistence has been reported in a few cases. Conservative treatment targeting weight gain is sufficient in most cases. An association between the superior mesenteric artery syndrome and acute pancreatitis has rarely been reported. We intend to describe the case of an 18-year-old girl who was admitted to the emergency room with epigastric pain and emesis. Our investigation revealed acute acalculous pancreatitis. During work-up, we discovered superior mesenteric artery syndrome and a compressed left renal vein. The patient is on conservative treatment, and her symptoms have improved.

Resumo A síndrome da artéria mesentérica superior designa compressão da terceira parte do duodeno pela artéria mesentérica superior e a aorta. Essa condição tem uma baixa incidência, sendo mais comum em mulheres jovens magras. A síndrome de quebra-nozes resulta da compressão da veia renal esquerda pela artéria mesentérica superior e a aorta. Ambas as entidades são raras, e a sua coexistência foi descrita em poucos casos. Tratamento conservador com o objetivo de ganho ponderal é suficiente na maioria dos casos. A associação entre a síndrome da artéria mesentérica superior e a pancreatite aguda foi raramente relatada. Pretendemos descrever o caso de uma jovem de 18 anos que recorreu ao serviço de urgência com epigastralgia e vômitos. A investigação realizada revelou pancreatite aguda alitiásica. Durante o estudo complementar, foi identificada a síndrome da artéria mesentérica superior, bem como uma veia renal esquerda comprimida. A paciente encontra-se em tratamento conservador, apresentando melhora clínica.

Síndrome de cascanueces acompañando un caso de síndrome de Wilkie: a propósito de un caso / Nutcracker Syndrome Combined with Wilkie Syndrome: Case Report

Resumen Objetivo: describir la presentación clínica, el diagnóstico y el tratamiento quirúrgico de un paciente con síndrome de la arteria mesentérica superior o síndrome de Wilkie combinado con síndrome de cascanueces o síndrome de compresión de la vena renal izquierda, en un centro de alta complejidad de un país latinoamericano. Descripción del caso: paciente masculino de 25 años, procedente de Estados Unidos, quien consultó por un año de pérdida de peso y episodios de obstrucción intestinal de etiología desconocida tras múltiples estudios de imagen. Se le realizaron estudios endoscópicos sin hallazgos; en la sala de recuperación desarrolló dolor abdominal que requirió ingreso al servicio de urgencias. La enterotomografía mostró dilatación de asas de colon e intestino delgado, con disminución del ángulo aortomesentérico y la serie gastrointestinal con paso filiforme del medio de contraste. Se intentó el manejo conservador como terapia inicial, con intolerancia al soporte nutricional entérico. Finalmente, se optó por el tratamiento quirúrgico, con un proceso de recuperación tórpido inicialmente, pero al final con resolución de los síntomas y aumento de peso. Conclusión: el síndrome de Wilkie es una enfermedad rara y un desafío diagnóstico en pacientes con pérdida de peso y dolor abdominal. Describimos un caso de compresión de la arteria mesentérica superior en el que se logró el diagnóstico con múltiples estrategias diagnósticas y resolución completa luego del tratamiento quirúrgico. La disminución del ángulo aortomesentérico puede comprimir la arteria mesentérica superior, así como la vena renal izquierda, que en ese caso resultó en un síndrome combinado de Wilkie y de cascanueces.

Abstract Objective: to describe the clinical presentation, diagnosis, and surgical treatment of a patient with superior mesenteric artery syndrome or Wilkie syndrome combined with the nutcracker syndrome or left renal vein compression syndrome in a tertiary referral center in a Latin American country. Case description: a 25-year-old male patient from the United States who attended for a year of weight loss and intestinal obstruction episodes of unknown etiology after multiple imaging studies. Endoscopic studies were performed without findings. While in the recovery room, he developed abdominal pain requiring admission to the emergency service. The CT enterography showed dilation of the colon loops and small intestine with a decrease of the aortomesenteric (AOM) angle and the gastrointestinal series with the filiform passage of the contrast medium. Conservative management was attempted as initial therapy with intolerance to enteric nutritional support. Finally, we initially opted for surgical treatment, with a slow recovery process, but in the end, with a resolution of symptoms and weight gain. Conclusion: Wilkie syndrome is a rare disease and a diagnostic challenge in patients with weight loss and abdominal pain. We described a superior mesenteric artery compression case in which diagnosis was achieved with multiple diagnostic strategies and complete resolution after surgical treatment. The decreased aortomesenteric angle may compress the superior mesenteric artery and the left renal vein. In this case, it resulted in a combined Wilkie and nutcracker syndrome.

Endovascular treatment of a patient with nutcracker syndrome and pelvic varices involving anterior and posterior renal veins.

We have reported a case of a 36-year-old woman with flank and pelvic pain and hematuria. She had posterior nutcracker syndrome and pelvic varices involving one anterior and three posterior renal veins (including one major vein). We used a complete endovascular approach, which included stent implantation in the major posterior renal vein and left gonadal vein embolization. During a 12-month follow-up period, the patient had had no symptoms and good computed tomography results. Endovascular treatment represents a safe and successful option for patients with nutcracker syndrome and pelvic varices involving the anterior and posterior renal veins.

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)

Combined Nutcracker and Ehlers-Danlos Syndromes: A Case Report.

INTRODUCTION: Nutcracker syndrome refers to the clinical manifestations of left renal vein compression between the superior mesenteric artery and the abdominal aorta, causing urinary changes and low back pain. REPORT: A 44 year old woman presented with low back and pelvic pain. Following the diagnosis of nutcracker syndrome, she underwent endovascular treatment with renal vein stent placement; however, the patient continued to complain of pain. Further examinations revealed left renal vein compression by the portal vein. The patient underwent a second procedure; however, improvement was temporary and her pain returned. Further investigation revealed previously undetected nephroptosis and hyperelasticity. A diagnosis of Ehlers-Danlos syndrome made, possibly explaining the mobility of viscera and unusual compression of the left renal vein by the portal vein. CONCLUSION: Ehlers-Danlos syndrome can cause nutcracker syndrome and may give rise to visceral pain of mixed origin.

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)

Prevalence of left renal vein compression (nutcracker phenomenon) signs on computed tomography angiography of healthy individuals.

OBJECTIVE: We evaluated the frequency of significant left renal vein (LRV) compression, also called the nutcracker phenomenon (NCP), in a normal asymptomatic population. METHODS: The present retrospective descriptive anatomic study analyzed the data from high-definition renal computed tomography (CT) angiography of living kidney donors. A total of 324 CT examinations were evaluated for signs of LRV compression, including the beak sign, aortomesenteric angle <41°, LRV diameter ratio ≥4.9, and beak angle ≥32°. The presence of pelvic varicose veins and the left gonadal vein in the proximal and mid-portion (considered dilated if >0.5 cm) were also evaluated. Anthropometric and laboratory (urine erythrocyte count) data were collected from the medical records. Statistical inference was calculated using Fisher's exact test and Student's t test. RESULTS: The mean aortomesenteric angle was 53.1° in women and 58.7° in men (P = .044). The beak sign and beak angle were present in 15.3% and 9.8%, respectively, and both had a greater prevalence in the women (P = .01). An aortomesenteric angle <41° was identified in 30.5%, with a greater prevalence in women (P < .01). The diameter ratio was positive in 0.7% of the cases, with no difference between the sexes. A left gonadal vein >0.5 cm was more prevalent in women in both the proximal and the mid-portions (P < .01). Although analysis stratified by positive criteria (3 or 4) showed no difference between the sexes, a positive correlation was found with younger age (P < .01). The limitations included the absence of a nutcracker syndrome (NCS) population; the lack of a renocaval pressure gradient, because of the need for intervention; the absence of other types of imaging studies, such as duplex ultrasound scan; and the absence of female parity data. CONCLUSIONS: The NCP and NCS CT criteria were present with a high frequency in healthy individuals. Women and younger individuals showed a greater prevalence of compression findings in the aortomesenteric axis. Revision of the current NCP and NCS criteria with a distinct categorization between sex, age, and body mass index is recommended to better evaluate LRV compressive events.

Endovascular treatment of a teenager with nutcracker syndrome: a case report.

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.

Compression of left renal vein and left common iliac vein on CT scans: how often are they detected?

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.

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.

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.