Nutcracker syndrome is a rare condition that can be successfully treated with renal vein stenting via an endovascular approach. The nutcracker syndrome is a rare clinic condition associated with severe hematuria and left flank pain due to the entrapment of the left renal vein between the superior mesenteric artery and the aorta. Endovascular treatment of nutcracker syndrome pubmed. Symptoms were hematuria, proteinuria or flank pain. Nutcracker syndrome ncs describes left renal vein compression. Nutcracker syndrome and radiographic evaluation of loin. Nutcracker syndrome, vascular imaging with ultrasound longdom. Results of endovascular treatment for patients with nutcracker.
We report on 3 patients who presented with nutcracker syndrome, including one patient with a circumaortic lrv resulting in posterior nutcracker syndrome, who underwent successful endovascular treatment with renal vein stenting. We describe the case of a 43yearold woman with a duplicated inferior vena cava who was found to have lrv compression on magnetic. In some cases, this compression syndrome arises in the setting of unusual vascular anatomic variants. Nutcracker syndrome is a rare vascular disorder that involves compression of the left renal vein lrv, most often at the level of the aortomesenteric angle. Although endovascular stenting is an attractive option, it is not without complications. Left renal vein entrapment syndrome, characterized by the compression of the left renal vein lrv between the superior mesenteric artery sma and the abdominal aorta was first described in 1950. Endovascular stenting is an alternative treatment option. Current trends in the diagnosis and management of renal. Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. The patient was treated with stenting of her left renal vein. To retrospectively assess the therapeutic value of endovascular stenting for treatment of the nutcracker syndrome ncs in longterm followup and to explore the selection of the size of stents in chinese patients with ncs. Jul 24, 2015 nutcracker syndrome is an entity resulting from left renal vein compression by the aorta and the superior mesenteric artery, which leads to symptoms of hematuria or left flank pain. Nutcracker syndrome ncs is a rare pathology manifested by pain or hematuria in males and females alike.
Hartung o, grisoli d, boufi m, marani i, hakam z, et al. Nutcracker syndrome results from compression of the left renal vein between the superior mesenteric artery and the aorta, leading to symptoms of hematuria and left flank pain. At the threeweek followup, she reported near resolution of nausea and abdominal pain. Nutcracker syndromean unusual case of chronic left upper ab. On ultrasound evaluation 1523 patients, the mean ratio of lrv peak. Management options include endovascular or laparoscopic extravascular stent placement, which is very appealing given the minimally invasive nature of these procedures. Nutcracker phenomenon ncp, also known as left renal vein entrapment, is characterized by impeded outflow from the left renal vein lrv into the inferior vena cava ivc due to extrinsic lrv compression, often accompanied by demonstrable lateral hilar dilatation and medial mesoaortic narrowing schematic representation of ncpnutcracker syndrome ncs in figure 1. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Thrombosis, stent migration, fracture and restenosis are the complications of the endovascular stenting but they are rare. Both open and endovascular approaches to the treatment of this condition have been described in the literature.
Endovascular treatment of nutcracker syndrome request pdf. Nutcracker syndrome servier phlebolymphologyservier. Nutcracker syndrome is a clinical entity leading to renal venous hypertension due to extrinsic compression of the left renal vein by the superior mesenteric artery. A simplified approach with gonadal vein transposition posted on june 12, 2016 june 12, 2016 by docpark the patient is a young woman in her twenties who developed severe right sided abdominal and back pain about 4 months prior to. Nutcracker syndrome is an entity resulting from left renal vein compression by. A simplified approach with gonadal vein transposition posted on june 12, 2016 june 12, 2016 by docpark the patient is a young woman in her twenties who developed severe right sided abdominal and back pain about 4 months prior to presentation associated with bouts of bloody urine. Renal vein stenting for nutcracker syndrome endovascular. Findings confirm compression of the duodenum wilkie syndrome and compression of the left renal vein nutcracker syndrome. Nov 06, 2014 endovascular stenting is an alternative treatment option.
Nutcracker syndrome is an entity resulting from left renal vein compression by the aorta and the. Nutcracker syndrome ncs is caused by compression of left renal vein lrv, usually between the aorta and the superior mesenteric artery sma. Carotid artery disease doctors and departments mayo clinic. Endovascular treatment of nutcracker syndrome journal of. The selection of the imaging modality is a stepwise process. Imaging findings and clinical features of abdominal vascular. Of those, 40% were found to have ncp by renal doppler ultrasonography dus.
Introduction nutcracker syndrome ncs, or renal vein entrapment syndrome, is a rare and easily overlooked condition. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to confirm. Endovascular angioplasty with stenting cas treatment. Clinicians place an endovascular stent in the left renal vein. Ncs refers to a syndrome caused by significant compression of lrv between the abdominal aorta and sma, resulting in hypertension of the lrv and hematuria due to rupture of the thinwalled veins close to the calyceal fornices 10,11. Carotid artery angioplasty with stenting cas is a minimally invasive procedure in which a very small hollow tube, or catheter, is advanced from a blood vessel in the groin to the carotid arteries. Current surgical therapy involves placement of an oversized renal vein stent with partial protrusion into the inferior vena cava ivc to relieve stenosis and prevent stent migration. Thoracoabdominal aneurysm surgery, thoracic endovascular aneurysm repair, carotid artery reconstruction. In one of the largest series of endovascular stents, 75 young patients.
In patients with instent stenosis, the diameter of the residual patent lumen and thickness of. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Our protocol for transabdominal pelvic vein duplex ultrasound. Despite potential serious consequences, diagnosing ncs is often challenging, circuitous and commonly delayed. Endovascular stenting in the treatment of pelvic vein congestion caused by nutcracker syndrome. Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound dus, computed tomography ct, magnetic resonance imaging mri, and venography.
It can be preferred to open surgery because of the long period of renal congestion, additional anastomoses and extensive dissection requirement of the open surgery. Endovascular treatment of deep vein thrombosis associated. Radiographic features are similar on ultrasound, doppler ultrasound, ct, mri, and. Its diagnostic criteria are not well defined, often causing delayed or misdiagnosis. Endovascular treatment of nutcracker syndrome sciencedirect.
Stents in the renal vein can cause fibromuscular hyperplasia, proximal migration. The stent will be placed in your vein by the surgeon after the surgeon makes a small puncture in the leg. Endovascular stenting for nutcracker syndrome article pdf available in journal of the chinese medical association 766 april 20 with 159 reads how we measure reads. The alternative option of endovascular or extravascular stenting is very appealing because of the minimal invasive procedures. Endovascular treatment by stenting was proposed and is not very invasive, but followup is too short to establish the role of this technique in the management of the nutcracker syndrome. Left renal vein transposition for nutcracker syndrome journal of. Some people may not have symptoms, while others develop severe and. Extravascular stent management for migration of left renal.
Chronic pelvic pain typically presents in young women in their 20s and 30s and may affect up to 40% of the female population during their lifetime. Results are encouraging at followup periods beyond two. Stenting to treat the nutcracker syndrome a stent is a small mesh tube which is used to hold the left renal vein open so that there is proper blood flow. Nutcracker syndrome is a rare diagnosis and may manifest in unusual ways.
Staff skilled in dozens of specialties work together to ensure quality care and successful recovery. Minimally invasive treatments for venous compression syndromes. In most cases, compression of the left renal vein is between the abdominal aorta the main artery in the abdomen and the superior mesenteric artery, which brings blood. Endovascular stent placement for nutcracker phenomenon. Unusual confluence of two rare syndromes results in. Imaging exams, including vascular mri, angiotomography, doppler. Once the catheter is in place, a balloon may be inflated to open the artery and a stent is placed. Since some of these causes are potentially serious and may be treatable if identified early, it is important to establish a.
The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and aa with some imagination appear to be a nutcracker crushing a nut the renal vein. The nutcracker syndrome ncs is the clinical equivalent of nutcracker phenomenon. Anterior nutcracker syndrome is caused by abnormal branching of the superior mesenteric artery that decreases the aortamesenteric angle to below 90, and posterior nutcracker syndrome is caused by the compression of the left renal vein between the abdominal aorta and the vertebral body. They also employed intravascular ultrasound to enhance localization of the. Nutcracker syndrome or mesoaortic compression of the left renal vein lrv with associated symptoms related to venous hypertension in the left kidney is a rare entity that may result in severe symptoms requiring operative intervention. Patient is a 43 year old woman who had been having bouts of severe left sided abdominal pain for several years with worsening episodes of nausea and vomiting resulting in several visits to the emergency room. Methods we report the case of a 26yearold woman with 4 months of intermittent flank pain and hematuria, diagnosed with nutcracker syndrome by both duplex ultrasound and axial based imaging. We explain the underlying pathophysiology that results in these clinical syndromes so that the patient subset who will benefit from treatment can be identified.
This article describes the typical imaging findings and clinical features that are associated with four abdominal vascular compression syndromes. Surgical treatment of posterior nutcracker syndrome presented. Both stenting and open surgical interventions may relieve symptoms. Renal nutcracker syndrome genetic and rare diseases. Pelvic congestion syndrome pcs results from ovarian, parauterine, or gluteal vein incompetence and remains an. Jun 22, 2017 endovascular stenting in the treatment of pelvic vein congestion caused by nutcracker syndrome. Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric artery sma and aorta, although other variations can exist 1 this can lead to renal venous hypertension, resulting in rupture of thinwalled veins into the collecting system with resultant hematuria. Endovascular management of recurrent stenosis following left renal vein transposition for the treatment of nutcracker syndrome donald t. Endovascular management of recurrent stenosis following left. Nutcracker syndrome, caused by compression of the left renal vein lrv between the. After a successful endovascular stenting and a 6month period of antiplatelet and. Imaging findings and clinical features of abdominal. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on nutcracker syndrome.
Nutcracker syndrome, first reported in 1972, is characterized by hematuria and back pain caused by increased pressure in the lrv as a result of compression between the aorta and the sma. Endovascular treatment of nutcracker syndrome scielo. Endovascular treatment of posterior nutcracker syndrome with. The exact prevalence of nutcracker syndrome is unknown, likely because of the variable presenting features. This can lead to obstruction of flow into the inferior vena cava and secondary left renal venous hypertension. The first case report of endovascular stent placement for renal vein hypertension was in 1996 by neste et al who described a 58yearold man successfully treated with a wallstent boston scientific corporation, natick, ma. Ncs typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain, without. Current management approach for left renal vein entrapment. There are various underlying pathologic causes, including renal or ureteric calculi or tumors, intrinsic kidney disease including glomerulonephritis, and loin pain hematuria syndrome.
Jan 01, 20 the nutcracker syndrome is a rare clinic condition associated with severe hematuria and left flank pain due to the entrapment of the left renal vein between the superior mesenteric artery and the aorta. Followup was completed by clinical exams and duplex ultrasound at 3, 6 and 12 months. Though lrv transposition and now lrv stenting are commonly employed methods for treatment of nutcracker syndrome, judicious management of ncs should take into consideration patients age, severity, and chronicity of symptoms along with current lack of data regarding longterm outcomes of stent placement. Endovascular stenting in the treatment of pelvic vein congestion caused by. Conclusion nutcracker syndrome is a rare diagnosis and may manifest in unusual ways. Typically, a selfexpanding nitinol or stainless steel stent is used.
Venous stents may lead to fibromuscular hyperplasia, embolization and proximal migration which may be fatal. The nutcracker syndrome ncs results most commonly from the compression of the left renal vein lrv between the abdominal aorta aa and superior mesenteric artery sma, although other variants exist. A case report of a young girl with recurrent hematuria. More recently, endovascular stenting has been described in several small series but have reported stent fracture, thrombosis, and migration. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to. Maythurner syndrome mts is an entity caused by the compression of the left iliac vein that predispose to acute deep vein thrombosis dvt of left lower limb.
Nov 22, 2017 although endovascular stenting is an attractive option, it is not without complications. Jul 14, 2017 posts about nutcracker syndrome written by docpark. Nutcracker syndrome is an entity resulting from left renal vein compression by the aorta and the superior mesenteric artery, which leads to symptoms of hematuria or left flank pain. Loin pain and hematuria are common symptoms, presenting in patients of all ages. Endovascular stent placement as a treatment for renal venous hypertension. Nutcracker syndrome is a variation of nutcracker phenomenon or renal vein entrapment syndrome, in which the arteries near the kidney compress the left renal kidney vein. Policha a, lamparello p, sadek m, berland t, maldonado t 2016 endovascular treatment of nutcracker syndrome. Nutcracker syndrome european journal of vascular and. Results of endovascular treatment for patients with. Venous stents may lead to fibromuscular hyperplasia, embolization and proximal migration which may be fatal 20. The use of lowflow color doppler, power doppler, advanced dynamic flow, or an equivalent is required to demonstrate flow defects representing echolucent instent stenosis in most cases. Extravascular stent management for migration of left renal vein endovascular stent in nutcracker syndrome lu tian1, shanwen chen2, gaoyue zhang3, hongkun zhang1, wei jin1 and ming li1 abstract background.
Nutcracker syndrome, caused by compression of the left renal vein lrv between the superior mesenteric artery and the aorta, results in left renal and gonadal venous hypertension. 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. Hansraj n, hamdi a, khalifeh a, wise e, sarkar r, toursavadkohi s. Several treatment options have been described to relieve associated symptoms. Daily r matteo j loper t northup m nutcracker syndrome. The nutcracker syndrome ncs comprises symptoms and findings such as. Presented at the peripheral vascular surgical society spring meeting, san diego.
Endovascular stent placement for the treatment of nutcracker syndrome. However, it is estimated to be relatively more common in females and usually presents in the 3rd or 4th decade of life. We report a case of migration of a laparoscopically. Examination in long axis using color doppler will reveal the presence of a velocity gradient from the perihilar to mesoaortic region 8. Endovascular stenting of the renal vein has been reported for nutcracker syndrome with a promising safety and efficacy profile. Mayo clinic has one of the largest and most experienced practices in the united states, with campuses in arizona, florida and minnesota. It is characterized by external compression of the out. Nutcracker syndrome is an important cause of pelvic pain and consists of a set of signs. Stents in the renal vein can cause fibromuscular hyperplasia, proximal migration or. Nutcracker syndrome, vascular imaging with ultrasound a case report.
As more cases are reported, endovascular repair is becoming an alternative treatment for nutcracker syndrome. It can be easily overlooked, and should be considered in young men or women with symptoms of extended duration. Endovascular stenting for treatment of nutcracker syndrome. Posts about nutcracker syndrome written by docpark. Nutcracker syndrome ncs is a rare pathology manifested by pain or. Nutcracker syndrome ncs is a rare entity defined by the symptomatic hypertension of the left renal vein lrv compressed in the majority of cases between the superior mesenteric artery and the aorta. Endovascular stenting for nutcracker syndrome sciencedirect. We present a case of a 54yearold female with chronic lower abdominal pain radiating to the left thigh of 4 years in duration. Chen and colleagues 2011 retrospectively evaluated the endovascular stenting of 61 individuals with nutcracker syndrome and a median age of 26 years. Nutcracker syndrome ns is a welldefined pathologic process in which the therapeutic approach of first choice, although not globally accepted, is currently stenting of the left renal vein lrv with a selfexpanding stent, which may be associated with left gonadal vein lgv embolization. Renal nutcracker syndrome ncs is a condition that occurs when the left renal vein the vein that carries blood purified by the left kidney becomes compressed. Left renal vein transposition for nutcracker syndrome. Nutcracker syndrome is an easily missed cause of hematuria in children. Nutcracker phenomenon and nutcracker syndrome ncbi.
Nutcracker syndrome occurs more frequently in women by the 3rd or 4th. Endovascular management of nutcracker syndrome after. Cgsurg106 venous angioplasty with or without stent. Syncope is one of the more rarely reported symptoms associated with nutcracker syndrome.
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