Nutcracker syndrome refers to symptomatic compression of the left renal vein lrv by the aorta and superior mesenteric artery sma. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. Some people may not have symptoms, while others develop severe and persistent symptoms. The nutcracker syndrome in a venous or even broader scope, vascular compression syndromes is based upon decreased space between the superior mesenteric artery and the aorta in the area of the left renal vein. The nutcracker syndrome results most commonly from the compression of the left renal vein between the abdominal aorta and superior mesenteric artery, although other variants exist. This can cause renal venous hypertension high blood pressure in the kidney veins and veins to rupture. 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 the pancreas and intestines. Degree of left renal vein compression predicts nutcracker.
Nutcracker syndrome is caused by stenosis of the left renal vein lrv as it passes anteriorly to the aorta through the fork formed by both abdominal aorta aa and superior mesenteric artery sma. Nutcracker syndrome ns is the constellation of symptoms associated with left renal vein entrapment because of nutcracker phenomenon 7,8. My daughter has nutcracker syndrome testicular disorders. In nutcracker syndrome, the renal vein coming from your left kidney becomes compressed and blood cant flow through it normally. Left renal vein compression or nutcracker syndrome occurs when the left renal vein is squeezed or compressed by surrounding arteries of the body. Renal nutcracker syndrome genetic and rare diseases nih. The term syndrome refers to a group of signs and symptoms primarily occurring together, characteristic of a specific condition. 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. Left renal vein transposition for nutcracker syndrome. Nutcracker syndrome refers to the entrapment of the left renal vein in the mesoaortic angle with elevation of left renal vein pressure. Nutcracker syndrome an overview sciencedirect topics.
Renal nutcracker syndrome genetic and rare diseases. Anterior nutcracker syndrome results from compression of the left renal vein between the superior mesenteric artery and the aorta, with subsequent development of venous varicosities of the renal pelvis, ureter, and the gonadal vein. Your left renal vein carries blood out of your left kidney and into your inferior vena cava, your bodys largest vein, so it can flow back up to your heart. Renal vein entrapment the nutcracker syndrome brandon t. Normally, the angle between the sma and aa is approximately 450. The nutcracker syndrome ncs is the clinical equivalent of. When nutcracker syndrome causes the compression of the left renal vein, it tends to force the blood backward that leads to its flowing into other veins, resulting in swelling. Diagnosis and surgical treatment of nutcracker syndrome. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Several treatment options have been described to relieve associated symptoms. The condition is called nutcracker syndrome because of how the arteries look as they compress the vein, like a nutcracker crushing a nut. The anteroposterior ap diameter and peak velocity pv were measured at the hilar portion of the left renal vein lrv and at the lrv between the aorta and the superior mesenteric artery. 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. However, nutcracker syndrome ns is characterized by coexisting of the nutcracker anatomy and characteristic symptoms.
Left renal vein transposition is an efficient surgical treatment for nutcracker syndrome. Entrapment of the left renal vein lrv between the abdominal aorta and superior mesenteric artery sma causing the classic clinical triad of hematuria, varicocele, and left abdominal or flank pain was first described in 1950. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Nutcracker syndrome refers to the compression of the left renal vein between the aorta and the superior mesenteric artery, which results in. Interventional radiology coding case studies prepared by. 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. Retroaortic left renal vein has an estimated prevalence of 2% 3. What each clinical anatomist has to know about left renal.
Nutcracker syndrome ncs refers to the clinical manifestations of ncp. This is the most common type of nutcracker syndrome. She had surgery, which is left renal vein transposition, where. Normally, the left renal vein brings blood out of the left kidney and. Nutcracker syndrome wikimili, the free encyclopedia. This leads to left renal vein hypertension that may result in rupture of the thinwalled vein into the renal calyceal fornix, with the clinical presentation of intermittent gross or microscopic hematuria. Nutcracker syndrome ncs is a rare vascular compression disorder that involves compression of the left renal vein most commonly between the aorta and the. Treatment of nutcracker syndrome florida surgical clinic. Nutcracker phenomenon refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood. Nutcracker syndrome complicated by left renal vein thrombosis. Anticoagulation was started and three weeks later, cta showed complete disappearance. The left renal vein receives up to 1215% of cardiac output via the left kidney, and with outflow obstruction, drains the blood through small collaterals. A rare, syndromic renal disease characterized by the entrapment of left renal vein lrv between the superior.
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 is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric. Furthermore, the venous return from the left gonadal vein returning to the left renal vein is blocked, thus causing testicular pain. 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. The left renal vein normally helps in bringing blood out of the left kidney and sends it to the vena cava, largest vein of the body.
Dilated left gonadal vein and pelvic varices indicate left renal vein lrv ouflow obstruction by the superior mesenteric artery sma i ordered a renal duplex and a ct venogram for procedural planning. Diseases associated with the renal vein include renal vein thrombosis rvt and nutcracker syndrome renal vein entrapment syndrome. Urological symptoms can be caused by increased pressure in the renal vein resulting in venous hypertension. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. 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. Nutcracker syndrome ncs is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Nutcracker syndrome definition of nutcracker syndrome by. In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome. The name nutcracker syndrome comes from the image of the left renal vein being compressed, or cracked, between the aorta and superior. A case report of a young girl with recurrent hematuria. In anterior nutcracker syndrome, the left renal vein is compressed between the aorta and another abdominal artery. What each clinical anatomist has to know about left renal vein. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis.
If there is both a vein passing in front of and one behind the aorta this is called a circumaortic renal vein. Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. In some people, the left renal vein can become wedged between the abdominal aorta and the superior mesenteric artery. Instead, blood can flow backwards into other veins and cause them.
The nutcracker syndrome is an unusual problem where the left renal vein is narrowed as it passes between an angle formed between the abdominal aorta and the superior mesenteric artery. In nutcracker syndrome, symptoms are most often caused when the left renal vein coming from the left kidney becomes compressed and blood. These findings are consistent with a nutcracker syndrome. There is, however, no abnormal drainage of a renal vein through retroperitoneal or lumbar collaterals. Nutcracker phenomenon refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood outflow often accompanied by distention of the distal portion of the vein. Its natural history is not well understood and there is a wide spectrum of clinical presentations. Nutcracker syndrome is an easily missed cause of hematuria in children. Renal vein entrapment, or more commonly nutcracker syndrome, is a phenomenon occurs when there is an obstruction somewhere along the left renal vein that prevents the circulation from passing through unopposed into the inferior vena cava and eventually, back to the heart.
Retroaortic left renal vein radiology reference article. In posterior nutcracker syndrome, the left renal vein is typically compressed between the aorta and the spine. 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. It was first described in 1950 by elsadr and mina and was named in 1971 1,2,3,4.
The development of ncs is associated with the formation of the left renal vein lrv from the aortic collar during the sixth to eighth week of gestation and abnormal angulation of the superior mesenteric artery from the aorta. Renal vein stenting for nutcracker syndrome endovascular. Compression of the left renal vein lrv between the superior mesenteric artery sma and the aorta was first described by grant in 1937 who found the anatomy analogous to a nut in a nutcracker. Differing views on definitive diagnostic criteria and varied symptomatology have led to sparse epidemiological data in the literature. Following the left renal venogram and pressure measurements, samples for renin. Nutcracker syndrome symptoms, treatment, causes, surgery. In some cases however pelvic pain remains following left renal vein transposition. The presence of hematuria, proteinuria, and pain albeit atypically right sided made it nutcracker syndrome. The term nutcracker phenomenon ncp refers to compression of the left renal vein lrv between the aorta and the superior mesenteric artery sma. We finally went to mayo clinic and she was diagnosed there. 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. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. As a result the superior mesenteric artery lies just over the left renal vein. 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.
Nutcracker syndrome gets its name because this compression is like a nutcracker crushing a nut. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. Some people may not have symptoms, while others develop severe and. 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 ncs results most commonly from the compression of the left renal vein lrv between the abdominal aorta aa and superior. In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. Nutcracker phenomenon and nutcracker syndrome ncbi.
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. It results most commonly from the compression of the left renal vein between the abdominal aorta and superior mesenteric artery. Pdf left renal vein entrapment syndrome nutcracker. Nutcracker syndrome is a constellation of symptoms resulting from renal vein entrapment or mesoaortic compression of the left renal vein. This phenomenon with its associated symptoms of unilateral hematuria and left flank pain is known as the nutcracker syndrome. At the upmc heart and vascular institute, our surgeons in the division of vascular surgery are experts in treating nutcracker syndrome. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. The nutcracker syndrome left renal vein entrapment, demonstrated by ultrasonic imaging and doppler flow scanning renal manifestations of sickle cell disease view in chinese hematuria has been attributed to the greater length of the left renal vein and the nutcracker phenomenon, in which compression of the left renal vein between. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and aa appear to be a nutcracker crushing a nut. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared. The small intestines hang like baggy sausages off the branched stems of the superior mesenteric artery sma, and in some individuals, the sma compresses the left renal vein against the aorta. Nutcracker syndrome, also called renal nutcracker syndrome is a rare vascular condition.
N2 imaging description the nutcracker phenomenon, also known as left renal vein entrapment, refers to the compression of the left renal vein between the aorta and the superior mesenteric artery figures 96. Patients may present with hematuria, proteinuria, collateral vessel formation and pain. The reason why this happens is a medical mystery but it is believed that abnormal organogenesis during fetal life is the. This can lead to renal venous hypertension, resulting in rupture of thinwalled veins into.