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.

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.

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.

Síndromes compresivos vasculares: un desafío diagnóstico / Vascular Compressive Syndromes: A Challenge Diagnosis

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.

Progresses of imaging diagnosis of nutcracker syndrome / 中国医学影像技术

Nutcracker syndrome (NCS) refers to symptomatic compression of the left renal vein between the abdominal aorta and the superior mesenteric artery with a serial of clinical symptoms including hematuria, proteinuria, lateral abdominal pain and varicocele. Imaging methods such as Doppler ultrasonography, CTA, MRA, intravascular ultrasound and renal venography play an important role in the diagnosis of NCS. The imaging findings and diagnostic criteria of NCS were reviewed in this paper.

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.

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 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.