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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.

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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.

RESUMO

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.

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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)

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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.

RESUMO

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.

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Resumen Los síndromes de compresión vascular constituyen un grupo de patologías poco frecuentes con prevalencia incierta, no solo por el desconocimiento que se tiene de estos, sino también por su presentación clínica inespecífica. Eso conlleva a que sean infradiagnosticados o detectados en etapas tardías, con secuelas a largo plazo. Su fisiopatología y clínica dependen del tipo de vaso comprometido, el sitio anatómico afectado y la causa subyacente que lo esté produciendo. La anamnesis, el examen físico dirigido y el uso de métodos complementarios como la ultrasonografía Doppler y la angiografía por tomografía computarizada (ATC) o angiografía por resonancia magnética (ARM), son herramientas vitales para su correcto diagnóstico, planificación terapéutica y seguimiento.

Abstract Vascular compression syndromes constitute a group of rare pathologies with uncertain prevalence not only because of their unawareness, but also for its nonspecific clinical presentation. This leads to their underdiagnosis, or detection in late stages with long-term sequelae. Its pathophysiology and presentation depends on the type of vessel involved, the affected anatomical site and the underlying cause producing it. Clinical history, physical examination and use of complementary methods such as Doppler ultrasonography, Computed Tomography Angiography (CTA) or Magnetic Resonance Angiography (MRA), are essential tools for its proper diagnosis, therapeutic planning and follow-up.

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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.

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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.

RESUMO

The term nutcracker syndrome (NS) refers to the compression of left renal vein between the aorta and the superior mesenteric artery (SMA) causing renal venous hypertension. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. The purpose of this study is to evaluate the clinical characteristics of patients with NS and the correlation between clinical and laboratory findings with Doppler sonographic measurements. Sixty-three patients were evaluated (19 boys and 44 girls) since January 2004 to January 2014. The mean age of the patients was 12.21 ± 3.21 years (range 7-17). Hematuria and proteinuria disappeared during the follow up of 9/63 patients. These nine patients showed statistically significant difference in upright SMA angles (p= 0.035). Doppler sonographic evaluation is a non-invasive method for diagnosis NS. Upright SMA angle measurement is more efficient than supine SMA angle measurement on clinicoradiological correlation.

Se llama ô€€€síndrome del cascanuecesô€€€ a la compresión de la vena renal izquierda entre la aorta y la arteria mesentérica superior (AMS) que causa hipertensión venosa renal. Los síntomas varían desde hematuria asintomática hasta congestión pélvica grave. El objetivo de este estudio es evaluar las características clínicas de pacientes con síndrome del cascanueces y la correlación entre los datos clínicos y analíticos, y los resultados de las ecografías Doppler. De enero de 2004 a enero de 2014, se evaluaron 63 pacientes (19 niños y 44 niñas). La media de la edad de los pacientes fue de 12,21 ± 3,21 años (intervalo: 7-17). Durante el seguimiento, la hematuria y la proteinuria desaparecieron en 9 de 63 pacientes. Estos 9 pacientes mostraron una diferencia estadísticamente significativa del ángulo entre la aorta y la AMS (p= 0,035) en la medición en posición erguida. La evaluación mediante ecografía Doppler es un método no invasivo para el diagnóstico del síndrome del cascanueces. En la correlación clínico-radiológica, la medición del ángulo aorto-mesentérico en posición erguida es más eficaz que en decúbito supino.

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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.

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