Síndrome de cascanueces y agenesia renal derecha. Una rara asociación

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.

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.

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)

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

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.

A Case of Secondary Hypertension Associated with the Nutcracker Phenomenon

A 25-year-old Korean woman was referred for uncontrolled hypertension. Laboratory examination revealed increased plasma renin activity and microscopic hematuria. Computed tomography demonstrated compression of the left renal vein (LRV) between the aorta and superior mesenteric artery; however, both renal arteries were intact and there was no adrenal mass. Renal vein catheterization showed external compression with a pressure gradient of up to 8 mm Hg between the LRV and the inferior vena cava. Plasma renin activity in the LRV was almost five times higher than that in the right renal vein. In this patient, renin-dependent hypertension was caused by renal congestion due to LRV obstruction.

Tratamento endovascular da Sindrome de Quebra-nozes (Nutcracker) / Endovascular treatment of Nutcracker syndrome

A Síndrome do Quebra-nozes se refere a um conjunto de sinais secundários à compressão da veia renal esquerda, mais comumente entre a artéria mesentérica superior e a aorta, levando ao comprometimento do fluxo para a veia cava. O diagnóstico desta síndrome frequentemente é difícil e, por esta razão, o diagnóstico é demorado, em muitos casos. É relatado, nesta publicação, o caso de uma mulher de 51 anos, tratada com sucesso pela embolização da veia ovariana associada ao implante de stent autoexpansível na veia renal esquerda. São discutidos os aspectos técnicos e as dificuldades do procedimento.

Nutcracker syndrome refers to signs and symptoms secondary to compression of the left renal vein, most commonly between the superior mesenteric artery and the aorta, leading to impaired outflow to the vena cava. Diagnosis of this syndrome is often difficult and as result is late in most cases. We report on the case of a 51-year-old woman successfully treated with embolization of the ovarian vein and insertion of a self expandable stent in the left renal vein. Technical details and pitfalls are discussed.

Fenómeno y síndrome de cascanueces asociado a hematuria y proteinuria ortostática / Nutcracker phenomenon and syndrome associated to hematuria and orthostatic proteinuria

El fenómeno cascanueces es una compresión de la vena renal izquierda, lo más frecuente es el ángulo formado por la arteria aorta y la mesentérica superior, por una emergencia anormal de la mesentérica. Cuando aparecen síntomas derivados de esta anomalía se le denomina síndrome de cascanueces. Este síndrome puede producir síntomas y signos muy variados, pero entre ellos, la hematuria, la proteinuria ortostática, el varicocele, la congestión pélvica crónica, el dolor abdominal y en flanco, y la intolerancia ortostática son los más frecuentes. La hematuria y la proteinuria ortostática son 2 manifestaciones que frecuentemente tienen que enfrentar el médico general integral y el pediatra, y es necesario tener en cuenta al síndrome de cascanueces en el diagnóstico diferencial de estas alteraciones. La hematuria es muy frecuente y la proteinuria ortostática tiene como causa principal el síndrome cascanueces. Por tal motivo consideramos importante esta breve revisión del tema, para poder enfrentar estas situaciones teniendo en cuenta todas sus posibilidades diagnósticas

Nutcracker phenomenon is left renal vein compression, more frequently in the angle formed by the aorta artery and the superior mesenteric artery due to abnormal emergency of the mesentery. When symptoms derived from this anomaly occur, this situation is called nutcracker syndrome. It may cause very varied symptoms and signs such as hematuria, orthostatic proteinuria, varicocele, chronic pelvic congestion, abdominal pain and flank pain, and orthostatic intolerance as the most common ones. Hematuria and orthostatic proteinuria are two frequent manifestations that the family physician and the pediatrician must face, so it is necessary to take the nutcracker syndrome into account for the differential diagnosis of these alterations. Hematuria is more frequent and orthostatic proteinuria is mainly caused by the nutcracker syndrome. Therefore, we consider that this brief review on this topic is important to cope with these situations and to bear in mind all their diagnostic possibilities

Significant Proteinuria Caused by Posterior Nutcracker Phenomenon

Posterior nutcracker phenomenon (PNP) was found to be the cause of significant proteinuria in a 10-year-old female. PNP was documented by Doppler ultrasonography and abdominal 3D CT as a cause of her proteinuria. Despite treatment with ACE inhibitor for several months, her persistent and progressive proteinuria lead us to perform a left renal biopsy, which revealed no significant finding except for focal effacement of foot processes. We speculate that nutcracker phenomenon can induce not only orthostatic proteinuria but also significant proteinuria by focal effacement of foot processes.

Left renal vein entrapment syndrome (report of 10 cases) / 中华泌尿外科杂志

Objective To evaluate the diagnosis and treatment of left renal vein entrapment syndrome. Methods The clinical data of 10 cases of left renal vein entrapment syndrome were retrospectively analyzed.Their mean age was 24 years (range,14-37 years).Of them 7 cases presented with non-glomerular origin hematuria;2,with orthostatic proteinuria;1 with hematuria accompanied by proteinuria.Color Doppler sonography showed that the dilated segment of the left vein was three-fold than the strictured segment in diameter,and magnetic resonance angiography (MRA) showed that the left renal vein was compressed in 9 cases. Results Stent placement across the left renal vein was performed in 7 cases.Superior mesenteric arteriectomy and replantation were performed in 3 cases.The operations were all successful.Follow-up of 1-5 years showed that the clinical symptoms were improved and urine tests were normal. Conclusions Non-glomerular hematuria or orthostatic proteinuria is frequently found in left renal vein entrapment syndrome.Left renal vein entrapment syndrome can be diagnosed by Doppler sonography,MRA and cystoscopy in the presence of hematuria or proteinuria.Generally,conservative treatment and careful follow-up are conducted in these patients.Surgical and radiologic interventional procedures are effective alternatives for those with glomerular dysfunction and complications.

Surgical management for left renal vein entrapment syndrome (report of 4 cases) / 中华泌尿外科杂志

Objective To study the clinical features,the diagnosis and treatment of left renal entrapment syndrome. Methods Four male patients with left renal entrapment syndrome were reported.Their age ranged from 16 to 22 years.The clinical features were characterized by recurrent episodes of gross hematuria,and 1 case had left varicocele concomitantly.Cystoscopy showed hematuria from the left ureter orifice.B-ultrasonography and CT scan showed that the left vein was clearly compressed at the included angle of abdominal aorta and superior mesenteric artery and the proximal vein was dilated in all the 4 patients.The characteristics of onset and imaging, and treatment of left renal entrapment syndrome were reviewed and discussed with the relevant literature. Results The diagnosis was established in the 4 cases based on B-ultrasonography and CT scan.Left renal vein reconstruction was performed on them.The patients were cured and discharged,with renal vein outflow being normal and no hematuria occurring.At 6 and 9 months after operation,CT and ultrasonography showed that the kidney contour was normal,the reconstructed veins were patent,and the routine urine test results were normal. Conclusions Left renal entrapment syndrome clinically results in high pressure of renal vein,hematuria and left varicocele.Autotransplantation of the left renal vein is an effective treatment option.

Nutcracker Syndrome Associated with Non-glomerular Hematuria in Childhood / 대한신장학회잡지

PURPOSE: This study was designed to aid the diagnosis and to predict the outcorne by understanding the clinical course of nutcracker syndrome in childhood. METHODS: The clinical, laboratory, radiological and cystoscopic data from the medical records of eleven children who were diagnosed as nutcracker syndrome by gross hematuria and pressure gradient criteria (>3mrnHg) were studied retrospectively and analyzed. RESULTS: Sex ratio of the cases was 7:4, and the median age of onset was 12.8 (3-14.3) years. Six cases showed persistent and 5 cases manifested interrnittent, exercise induced hematuria. Left flank pain (64%), abdominal pain (18%), left varicocele (9%) were associated in some of the children, but hematuria was the only symptom in 36Yo. Left renal vein entrapment was documented in 10 cases by ultrasonography. Out of the 5 cases studied by renal Doppler ultrasonography, 4 and 5 cases showed higher (>5) mean left renal vein diameter ratio (Distal/ Aortomesenteric portion) and mean peak velocity ratio respectively. Unilateral bleeding from left ureteral orifice was documented in 7 of the 9 cases at cystoscopy. The mean pressure gradient between proximal left renal vein and inferior vena cava was 4.4+/-1.6 (3-7) mmHg. Hematuria of 25% and 57% of the cases disappeared spontaneously in 3 and 5 years after onset respectively. Proteinuria disappear- ed in 3 of the 5 initial proteinuric cases. CONCLUSION: Nutcracker syndrome must be considered in the differential diagnosis of non-glomerular, especially gross hematuria in childhood, and Doppler ultrasonography can aid diagnosis non-invasively. The renal function remained stable, but 4396 of the cases continued to show hematuria still 5 years after onset.