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)

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)

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 Nutcracker syndrome: case report.

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.

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

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(AU)

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(AU)

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