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)

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)

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 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)

Posterior Nutcracker Syndrome Associated with Interrupted Left Inferior Vena Cava with Azygos Continuation and Retroaortic Right Renal Vein

Various anatomic anomalies have been considered the causes of nutcracker syndrome (NCS). Posterior NCS refers to the condition, in which vascular narrowing was secondary to the compression of the retroaortic left renal vein while it is crossing between the aorta and the vertebral column. Here, we report an unusual case of posterior NCS associated with a complicated malformation of the interrupted left inferior vena cava with azygos continuation and retroaortic right renal vein, diagnosed by both color Doppler ultrasonography and CT angiography.