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. Superior mesenteric artery sma syndrome, also known as wilkie syndrome, is a rare acquired vascular compression disorder in which acute angulation of the superior mesenteric artery sma results in compression of the third part of the duodenum, leading to obstruction. This condition is known as willkie syndrome or superior mesenteric artery. No consensus diagnostic criteria exist to guide endovascular treatment. It occurs when arteries, most often the abdomens aorta and superior mesenteric artery, squeeze the left renal kidney vein. Degree of left renal vein compression predicts nutcracker.
Shin ji, park jm, lee js, kim mj 2007 effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. Our findings suggest exercising caution when attributing these rare syndromes to an absolute sma angle. Superior mesenteric artery sma syndrome is an uncommon but well recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum between the aorta and the superior mesenteric artery. The speaker discusses normal anatomy and pathology of the aorta and the mesenteric arteries. Nutcracker phenomenon and nutcracker syndrome ncbi. Nutcracker syndrome refers to the compression of the left renal vein between the aorta and the superior mesenteric artery, which results in. Nutcracker syndrome renal vein entrapment findings. Using a definition of sma angle feb 14, 2019 heidbreder r. The decreased angle between sma and the aorta can cause ncs, this angle was. In certain situations, the syndrome can result from a retroaortic or circumaortic left renal vein nutcracker syndrome should not be confused with superior mesenteric artery syndrome wilkie syndrome also a superior mesenteric artery compression disorder, where the sma compresses the third part of the duodenum the two conditions, however, may be associated. Although uncommon, it is an important diagnosis due to the important morbidity associated with it, including the risk of chronic kidney disease from longterm left renal vein lrv hypertension and the risk of lrv thrombosis.
This article describes the typical imaging findings and clinical features that are associated with four abdominal vascular compression syndromes. The part of the renal vein to pass between the aorta and the sma was seen to be. Nutcracker phenomenon is an uncommon condition characterized by compression of the left renal vein lrv between the abdominal aorta and the superior mesenteric artery sma, the sma and aorta appearing as two arms of a nutcracker compressing the lrv. Nutcracker syndrome ncs describes left renal vein compression between the superior. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. The sma frequently rises at an acute angle from the aorta, but can have a low or lateral origin, and the distance between the sma and the aorta at the level of the lrv is reduced, as shown by fu. Less often, the third portion of the duodenum courses in front of the lrv between the aorta and the sma. It is not clear why some individuals get nutcracker syndrome, or what its treatment should be.
It was first described in 1950 by elsadr and mina and was named in 1971 1,2,3,4. It is associated with the formation of the left renal vein from the aortic collar during the 6th8th week of gestation and abnormal angulation of the sma from the aorta. 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. Differing views on definitive diagnostic criteria and varied symptomatology have led to sparse epidemiological data in the literature. Diagnosis and treatment of the nutcracker syndrome. Nutcracker syndrome ns refers to symptomatic compression of the left renal vein lrv between the abdominal aorta and superior mesenteric artery with potential symptoms including hematuria, proteinuria, left flank pain, and renal venous hypertension.
Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut. For the diagnosis of ncp, several diagnostic tools are applied, but definite diagnostic criteria have not yet been established. It is characterized by compression of the left renal vein lrv between the superior mesenteric artery sma and the abdominal aorta. Nutcracker syndrome is characterized by a set of signs and symptoms secondary to compression of the left renal vein lrv either in the acute anatomic angle between the abdominal aorta aa and the origin of the superior mesenteric artery sma known as anterior ncs 2,3,4,5 or in a retroaortic position between aa and vertebral column, which. Ncp ultrasound criteria, and 7 ultrasound grades with criteria for. We explain the underlying pathophysiology that results in these clinical syndromes so that the patient subset. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. See more ideas about syndrome, nutcracker, superior mesenteric artery. Previous investigators using ct have suggested that nutcracker syndrome can be diagnosed when there is lrv stenosis at the sma, suggesting a reasonable cutoff of 5 mm or less, which would include 21 10. Cooccurring superior mesenteric artery syndrome and nutcracker syndrome requiring rouxeny duodenojejunostomy and left renal vein transposition. Nutcracker syndrome, vascular imaging with ultrasound longdom. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Nutcracker syndrome refers to symptomatic compression of the left renal vein lrv by the aorta and superior mesenteric artery sma.
In addition to enabling evaluation of flow, doppler can also reveal the compressive process caused by the sma. In some cases, this compression syndrome arises in the setting of unusual vascular anatomic variants. Nutcracker syndrome wikimili, the free encyclopedia. Pdf left renal vein entrapment syndrome nutcracker. It may be associated with the nutcracker phenomenon. Ultrasound assessment of the aorta and mesenteric arteries.
We presented this case report in order to discuss the diagnosis of a patient with both sma syndrome and accompanying ncs with computed tomography ct findings. Common treatments for nutcracker syndrome include stenting, surgery, and routine urine tests. Imaging findings and clinical features of abdominal vascular. Nutcracker syndrome ncs is a rare vascular compression disorder that involves compression of the left renal vein most commonly between the aorta and the superior mesenteric artery sma, although variations exist. Imaging findings and clinical features of abdominal. Nutcracker syndrome ncs is characterized by impeded outflow from the left renal vein lrv into the inferior vena cava ivc due to extrinsic lrv compression. 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. The condition occurs when the duodenum the part of the small intestine that connects to the stomach is compressed between two arteries the main artery of the body called the aorta and one of the main branch arteries of the aorta called the superior mesenteric.
Nutcracker syndrome servier phlebolymphologyservier. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. Nutcracker syndrome ncs, also known as left renal vein lrv entrapment syndrome, was first defined anatomically by grant in 1937 as follows. Following are the common symptoms that have been studied in the nutcracker syndrome. To our knowledge, there are few reports in the literature describing the coexistence of these two. Nutcracker syndromean unusual case of chronic left upper. Several treatment options have been described to relieve associated symptoms. We describe the case of a 43yearold woman with a duplicated inferior vena cava who was found to have lrv. Using a definition of sma angle criteria have not yet been established.
The nutcracker syndrome ncs results most commonly from the compression of the left renal. The term nutcracker phenomenon ncp refers to compression of the left renal vein lrv between the aorta and the superior mesenteric artery sma. Nutcracker syndrome complicating with renal abscess. Superior mesenteric artery sma syndrome is a rare condition that involves the small intestine. The term nutcracker phenomenon ncp refers to compression of the left renal vein lrv between the aorta. It can cause many symptoms in both children and adults, such as flank pain and blood in the urine. Nutcracker phenomenon is an uncommon condition that may lead to a syndrome of chronic abdominal pain. Superior mesenteric artery syndrome radiology reference. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension.
Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric. Nutcracker syndrome ncs is a rare vascular compression disorder that involves compression of the left renal vein most commonly between the aorta and the superior mesenteric artery sma. Superior mesenteric artery syndrome combined with renal nutcracker syndrome in a young male. 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 nutcracker syndrome ncs is the clinical equivalent of ncp and is characterised by various symptoms such as haematuria, proteinuria, flank pain, pelvic congestion orvaricocele. The type of nutcracker syndrome in this case report is known as anterior nutcracker 1,2. Nutcracker syndrome ncs describes left renal vein compression between the superior mesenteric artery and the aorta.
They had a significant difference in both the hilar and amp peak flow velocity p. Nutcracker syndrome refers to the compression of the left renal vein between the aorta and the superior mesenteric artery, which results in elevated left renal vein pressure and possible collateral vein development. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. This can lead to renal venous hypertension, resulting in rupture of thinwalled veins into. The nutcracker phenomenon ncp refers to the compression of left renal vein lrv most commonly between the superior mesenteric artery sma and aorta. Nutcracker syndrome associated with celiacomesentric trunk anomaly. Nutcracker syndrome ncs or left renal vein lrv entrapment is rare and may be caused by. Patients with varicocele and nutcracker syndrome did not have a significant difference in either the hilar or amp diameter compared with patients with varicocele without nutcracker syndrome. Left renal venous hypertension nutcracker syndrome. Some people may not have symptoms, while others develop severe and persistent symptoms. The name derives from the fact that, in the sagittal plane andor transverse plane. 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. See more ideas about syndrome, superior mesenteric artery, superior mesenteric artery syndrome. We aimed to evaluate the specificity of lrv compression to ns symptoms.
Multidetector ct of vascular compression syndromes in the. Nutcracker syndrome is a rare disease entity that is caused by entrapment of the left renal vein between. The nutcracker phenomenon left renal vein lrv entrapment syndrome refers to compression of the lrv most commonly between abdominal aorta and superior mesenteric artery. Nutcracker syndrome is an easily missed cause of hematuria in children. We explain the underlying pathophysiology that results in these clinical syndromes so that the patient subset who will benefit from treatment can be identified. Other types of ncp are a combination of anterior nutcracker and compression of the third portion of duodenum that courses in front of the lrv between the aorta and the sma. Transverse ultrasound image demonstrates compression of the left renal vein lrv between the superior mesenteric artery sma and aorta ao courtesy of ahmad alomari, md. Nutcracker syndrome associated with celiacomesentric trunk. Left renal vein transposition for nutcracker syndrome.
Common signs and symptoms when the condition shows no symptoms, its usually known. It should not be confused with nutcracker syndrome which can be an. It is distinct from nutcracker syndrome, which is the entrapment of the left renal vein between the aa and the sma, although it is possible to be diagnosed with both conditions. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting.
Jun 22, 2017 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. This condition is not to be confused with superior mesenteric artery syndrome, which is the. 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. These include irritable bowel syndrome, functional dyspepsia, and opioid bowel dysfunction. There is a wide spectrum of clinical presentations and diagnostic criteria are not well defined, which. Effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria 19 october 2006 european journal of pediatrics, vol. Pdf left renal vein entrapment syndrome nutcracker syndrome. Superior mesenteric artery syndrome presenting with acute massive gastric dilatation, gastric wall pneumatosis, and portal venous gas. Strangulating fibrolymphatic tissue between the aorta and smaa, b, c. Fitoz s, ekim m, ozcakar zb, elhan ah, yalcinkaya f, et al. Superior mesenteric artery syndrome accompanying with.
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. To our knowledge, there are few reports in the literature describing the coexistence of these two conditions and so we take this. This results in chronic, intermittent, or acute complete or partial duodenal obstruction. 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. The nutcracker syndrome is a rare clinical entity caused by compression of the left renal vein lrv by the superior mesenteric artery sma as it passes between the sma and the abdominal aorta anterior nutcracker syndrome. Axial contrastenhanced ct of the abdomen demonstrated compression of the left renal vein between the superior mesenteric artery sma and the aorta as the left renal vein crosses midline figure 1. An update on current diagnostic criteria and management guidelines k. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and. When symptomatic, such compressions can result in a variety of uncommon syndromes in the abdomen and pelvis, including median arcuate ligament syndrome mals, maythurner syndrome, nutcracker syndrome, superior mesenteric artery sma syndrome, upj obstruction, ovarian vein syndrome ovs, and other forms of ureteral compression. The proposed ultrasound techniques are a valid protocol extension to the ultrasound examination to help diagnose ncp. Oct 05, 2014 nutcracker syndrome ncs is characterized by impeded outflow from the left renal vein lrv into the inferior vena cava ivc due to extrinsic lrv compression. Renal nutcracker syndrome genetic and rare diseases.
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