RESUMEN
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)
Asunto(s)
Humanos , Femenino , Adolescente , Síndrome de Cascanueces Renal/diagnóstico por imagen , Hematuria/etiología , Aparatos Ortopédicos , Tomografía , Cistoscopía , Síndrome de Cascanueces Renal/cirugía , Reparación Endovascular de Aneurismas , Hematuria/orina , Anemia/terapiaRESUMEN
There are few data on endovascular treatment of anterior nutcracker syndrome and pelvic varices in patients with anterior and posterior renal veins. Our objective is to report a case, identify occurrences and compare diagnosis and treatments. A 42-year-old woman presented with flank and pelvic pain and hematuria. She had anterior nutcracker syndrome and pelvic varices with an anterior and a posterior renal vein. A successful complete endovascular approach was done with stent implantation in the anterior renal vein and left gonadal vein embolisation. After 12-month follow-up, the patient remained asymptomatic with good results on CT. Only two case reports of patients with nutcracker syndrome with anterior and posterior renal veins were identified. In both, a self-expanding stent was implanted in the anterior renal vein. In conclusion, endovascular treatment represents a safe and successful option in patients with nutcracker syndrome and pelvic varices with an anterior and a posterior renal vein.
Asunto(s)
Procedimientos Endovasculares , Síndrome de Cascanueces Renal/cirugía , Venas Renales/anomalías , Várices/cirugía , Adulto , Femenino , Humanos , Síndrome de Cascanueces Renal/complicaciones , Várices/complicacionesRESUMEN
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.
Asunto(s)
Humanos , Masculino , Niño , Procedimientos Endovasculares , Síndrome de Cascanueces Renal/cirugía , Venas Renales , Síndrome de Cascanueces Renal/complicaciones , Síndrome de Cascanueces Renal/diagnóstico , Hematuria/complicaciones , Anemia/complicacionesRESUMEN
BACKGROUND: Nutcracker syndrome is a rare entity, and in the majority of cases is the result of extrinsic compression of the left renal vein between the superior mesenteric artery and the aorta, associated with functional stenosis. OBJECTIVE: To present the case of a 19-year-old female with no significant medical history with confirmed diagnosed of nutcracker syndrome treated successfully by endovascular means. CLINICAL CASE: She was referred to the Vascular Surgery Department with a 6-month history of macroscopic haematuria, after other aetiologies were ruled out. Abdominal computed tomography angiography revealed compression of the left renal vein; the patient underwent endovascular treatment, and a 12×16 mm balloon expandable stent was placed with immediate angiographic improvement, decreased pressure gradients and progressive resolution of haematuria. At one year, she remains symptom-free. CONCLUSION: Nutcracker syndrome is uncommon, and a high index of suspicion is needed. Macroscopic haematuria is not always present, and in our case stent placement demonstrated effectiveness in the resolution of symptoms at 12 months' follow--up. We also present a brief review of the literature.
Asunto(s)
Procedimientos Endovasculares/métodos , Hematuria/etiología , Síndrome de Cascanueces Renal/cirugía , Femenino , Humanos , Síndrome de Cascanueces Renal/complicaciones , Venas Renales/fisiopatología , Stents , Presión Venosa , Adulto JovenRESUMEN
Nutcracker syndrome refers to the complex of clinical symptoms caused by the compression of the left renal vein (LRV) between the abdominal aorta and the superior mesenteric artery, leading to stenosis of the aortomesenteric portion of the LRV and dilatation of the distal portion. Hematuria, proteinuria, flank pain, varicocele and pelvic congestion may occur, occurring more frequently in young adults. Conservative management, might be the option whenever it is possible. When surgical treatment is required, classically open surgery have been performed, with major surgeries as LRV transposition or bypass techniques. The main caveats regards the fact that these are large and risky surgeries. Endovascular surgery with venous stent placement has gained some space as it is minimally invasive alternative. However, venous stents are associated with a high number of trombotic complications and in many cases requirement of life-long anticoagulants. External stenting of the LRV with this "shield technique" is a minimally invasive alternative, with good medium term results. We herein demonstrate our second experience with the technique of this surgery in a patient with 12 months of follow up and excellent results.