Using a definition of sma angle angle formed between these two vessels is called the aortomesenteric angle. In certain situations, the syndrome can result from a retroaortic or circumaortic left renal vein. Pain management specialists should include nutcracker syndrome in the differential when the symptoms indicate, and not assume atypical left flank pain is chronic myofascial or due to lower rib pathology. A narrow aortomesenteric angle causes compression or entrapment of the left renal vein. Nutcracker syndrome is a vascular compression disorder that refers to the. 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 angle formed between these two vessels is called the aortomesenteric angle. 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 ncp nutcracker syndrome ncs in figure 1. Superior mesenteric artery syndrome and nutcracker.
Superior mesenteric artery sma syndrome is a rare cause of gastrointestinal obstruction, caused by external compression of the third part of the duodenum by the sma. These syndromes, the pathogenesis of some of which remains controversial, can result in nonspecific symptoms of epi. Jan 23, 2019 ct imaging showing sma syndrome and nutcracker syndrome. For patients with debilitating pain or those who require red blood cell transfusions due to nutcracker phenomenoninduced anemia, intervention is key. Pdf superior mesenteric artery syndrome accompanying.
Nutcracker syndrome accompanying with superior mesenteric. 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. Superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction. The aortomesenteric space normally averages between 4 and 5 mm in width. A diagnosis of nutcracker syndrome was made and a left renal vein transposition. A drawing shows a normal aortomesenteric angle with the lrv blue circle between the aorta and the ams. Nutcracker syndromean unusual case of chronic left upper. Nutcracker phenomenon and nutcracker syndrome ncbi. If that aortomesenteric angle is reduced, the arteries begin to pinch the left renal vein like a nutcracker, preventing blood from returning back to the heart.
On ct, the beak sign is an abrupt narrowing of the lrv between the aorta and sma, with proximal dilatation of the lrv. An abdominal ultrasound revealed a reduced aortomesenteric angle and an engorged left renal vein supplementary a. Renal nutcracker syndrome genetic and rare diseases. Radiographic features are similar on ultrasound, doppler ultrasound, ct, mri, and conventional angiography. Nutcracker syndrome in children with orthostatic proteinuria. Nutcracker syndrome ncs describes left renal vein compression between the. High index of suspicion, in the presence of known risk factors, is the key to early diagnosis. 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. Ama aortomesenteric angle, amd aortomesenteric distance, ct computed tomography, ivc inferior vena cava, pda pancreaticoduodenal arcade. Given that both the duodenum and the lrv lie within the aortomesenteric angle, two different types of compression syndrome can arise. Left renal vein transposition for nutcracker syndrome. Renal vein entrapment the nutcracker syndrome brandon t. Ncs can be suspected from the clinical history and a urine erythrocyte morphology of predominantly isomorphic erythrocytes.
Some people may not have symptoms, while others develop severe and. Superior mesenteric artery syndrome coexists with nutcracker. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. Meissner the nutcracker syndrome is characterized by entrapment and compression of the left renal vein between the abdominal aorta and the superior mesenteric artery sma figure 1. Nutcracker syndrome ncs describes left renal vein compression between the superior mesenteric artery and the aorta. Nutcracker syndrome may be an important cause of orthostatic proteinuria in children. Collateral veins may be evident on pelvic and abdominal dus or venography. Nutcracker syndrome is a rare condition with unknown prevalence given that many patients with the phenomenon are asymptomatic. The importance of retroperitoneal fat in maintaining a wide aortomesenteric angle was suggested by stavros et al.
Doppler sonography of the left renal vein may be a useful screening tool in patients with suspected orthostatic proteinuria to evaluate whether the cause is nutcracker syndrome. Superior mesenteric artery syndrome accompanying with. Aug 25, 2016 renal vein entrapment the nutcracker syndrome brandon t. Thurner syndrome, nutcracker syndrome, superior mesenteric artery sma syndrome, upj obstruction, ovarian vein syndrome ovs. We report two patients in whom a hybrid approach was adopted in the process combining the strengths of each. The compression of the left renal vein in the aortomesenteric. The angle between the aa a and the sma b was less than 35 measured at c the third portion of the duodenum was compressed by the decreased aortomesenteric angle in the transverse plane.
Superior mesenteric artery syndrome radiology reference. Therefore, further anthropometric studies should be performed to identify these. Its usually around 45 degrees, with the renal vein cushioned by a bit of fat in the mesentery. However, it is estimated to be relatively more common in females and usually presents in the 3rd or 4th decade of life. 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.
The duodenum and the left renal vein occupy the vascular angle made. Open and endovascular interventions have both been performed to relieve the compression. The superior mesenteric artery sma syndrome is a rare cause of proximal intestinal obstruction in which the third part of the duodenum is compressed between sma and the aorta due to narrowing in aortomesenteric angle. A case report of a young girl with recurrent hematuria.
Degree of left renal vein compression predicts nutcracker. Nutcracker syndrome is a rare anomaly resulting from compression of the left renal vein between the aorta and the superior mesenteric artery. Cooccurring superior mesenteric artery syndrome and nutcracker. The aortomesenteric angle created by the sma and aorta is. The angle between the aa a and the sma b was less than 35 measured at c the third portion of the. In addition, the aortomesenteric distance is 28 millimeters, as opposed to the typical 1020.
Contributory factors included are an asthenic body habitus, decreased retroperitoneal and mesenteric fat tissue, left renal ptosis and spinal lordosis. 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. The nutcracker syndrome ns is believed to be the result of the compression of the left renal vein lrv in the aortomesenteric angle ama. Steep angulation of the sma relative to the aorta can cause a short aortomesenteric distance amd representing the anterior nutcracker syndrome. Nutcracker syndrome is a rare disease entity that is caused by entrapment of the left renal vein between the aorta and superior. The symptoms vary from asymptomatic hematuria to severe pelvic congestion.
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. This condition is known as willkie syndrome or superior mesenteric artery. 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. Multidetector ct of vascular compression syndromes in the. Superior mesenteric artery syndrome is an uncommon cause of duodenal obstruction, and its manifestations are generally associated with compression on the third part of the duodenum between the. Nutcracker phenomenon and nutcracker syndrome undiagnosed.
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. The mechanism of ncs is similar to duodenal obstruction in sma syndrome 4. Nutcracker syndrome refers to the entrapment of the left renal vein in the mesoaortic angle with elevation of left renal vein pressure. Stretching the lrv secondary to a posteriorly ptotic left kidney, excess fibrous. This rare, potentially lifethreatening syndrome is typically caused by an angle of 625 between the aa and the sma, in comparison to the normal range of 3856, due to a lack of retroperitoneal and visceral fat mesenteric fat.
For the first time it was described in 1950 by elsadr and mina as a possible reason for a varicocele by congestion 1. Using a definition of sma angle syndrome mals, maythurner syndrome, nutcracker syndrome, superior mesenteric artery sma syndrome, upj obstruction, ovarian vein syndrome ovs, and other forms of ureteral compression. The mean ratios of pv in the lrv between the aortomesenteric angle and the. The aortomesenteric compression of the duodenum is a rare radiological finding aortomesenteric angle, hematuria, left renal vein, nutcracker syndrome background. A sagittal ct view of the aortomesenteric region demonstrating an angle. 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. They had a significant difference in both the hilar and amp peak flow velocity p. The nutcracker phenomenon left renal vein lrv entrapment syndrome refers to compression of the lrv most commonly between abdominal aorta and superior mesenteric artery.
Angiographic studies have indicated that patients with sma syndrome have an abnormal aortomesenteric angle 622, and a shortened aortomesenteric distance 28 mm compared to normal patients. Sma syndrome and nutcracker syndrome have common features that result from narrowed aortomesenteric angle. 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. Nutcracker syndrome associated with celiacomesentric trunk anomaly. If you continue browsing the site, you agree to the use of cookies on this website. Imaging findings and clinical features of abdominal. Nutcracker syndrome associated with celiacomesentric trunk. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. To our knowledge, there are few reports in the literature describing the coexistence of these two conditions and so we take this. 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. Ct imaging showing sma syndrome and nutcracker syndrome. Several treatment options have been described to relieve associated symptoms. Ama aortomesenteric angle, amd aortomesenteric distance, ct computed tomography, ivc inferior vena cava.
The syndrome is characterized by compression of the third portion of the duodenum due to narrowing of the space. Superior mesenteric artery syndrome and nutcracker phenomenon. Although our previous report suggested that changes of body mass index in children with nutcracker syndrome might influence the aortomesenteric angle, there has been no report describing the relationship between nutcracker syndrome and physical characteristics. Hybrid intervention for treatment of the nutcracker syndrome. In the majority of patients, the normal angle between the superior mesenteric artery and the aorta is between 38 and 65. The severity of nutcracker syndrome is variable, and affected individuals may be completely asymptomatic or, in the most extreme. It has been referred to by a variety of other names, including cast syndrome, wilkie syndrome, arteriomesenteric duodenal obstruction, and chronic duodenal ileus. Surgical options include renocaval reimplantation, resection of fibrous tissue and placement of wedge at aortomesenteric angle. Pdf the nutcracker and superior mesenteric artery sma syndromes are rare conditions where the left renal vein or. Imaging findings and clinical features of abdominal vascular. Patients may present with hematuria, proteinuria, collateral vessel formation and pain.
The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. C, oblique sagittal reformation image with sma and aorta in same plane shows aortomesenteric angle lines of 35. The exact prevalence of nutcracker syndrome is unknown, likely because of the variable presenting features. 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. The type of nutcracker syndrome in this case report is known as anterior nutcracker 1,2. Superior mesenteric artery syndrome and nutcracker syndrome. Each of these interventions has strengths and weaknesses. The nutcracker syndrome ncs is the clinical equivalent of nutcracker phenomenon. The aortomesenteric distance is normally 10 to 28 mm. Nutcracker syndrome ns refers to symptomatic compression of the left. It may be associated with the nutcracker phenomenon. Nutcracker syndrome combined with superior mesenteric artery. In addition to an appropriate clinical history, ct findings indicating decreased aortomesenteric angle and a shortened aortomesenteric distance can suggest the diagnosis for both the superior mesenteric artery syndrome and accompanying nutcracker syndrome.
1506 881 1052 621 781 5 865 703 1 405 202 393 869 768 90 350 666 81 520 1270 876 903 927 1083 468 779 1303 546 1467 475 1392 1240 120 706 161 1296 1293 427 939 1069 1129 475