It should be considered when patients present with pain in the left flank and intermittent macroscopic hematuria or pelvic congestion syndrome. The posterior nutcracker syndrome is very rare and is considered when the left renal vein is compressed between the aorta and vertebral column. We report a 6yearold girl with episodic macroscopic hematuria who was diagnosed as ncs with doppler ultrasound and 3d computerized tomography angiography. Nutcracker syndrome is defined as compression of the left renal vein between the superior mesenteric artery and the aorta and is associated with urinary abnormalities, fig 8a. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Duplex scanning and ct angiography show compression of the left renal vein.
The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. 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. With anterior nutcracker syndrome, the left renal vein is compressed between. 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 is caused by a compression of the left renal vein between the aorta and the superior mesenteric artery. Several treatment options have been described to relieve associated symptoms. Anterior nutcracker syndrome as a rare cause of hematuria in an 8 year old boy. 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. Nutcracker syndrome is described as the symptomatic compression of left renal vein between the aorta and the superior meteric artery, resulting in outflow congestion of the left kidney.
Degree of left renal vein compression predicts nutcracker. Nutcracker syndrome ncs is a syndrome caused by compression of the left renal vein lrv, between the abdominal aorta and the superior mesenteric artery, resulting in hypertension of the lrv and hematuria. Left renal vein transposition is an efficient surgical treatment for nutcracker syndrome. Diagnosis and surgical treatment of nutcracker syndrome. Several renal vascular pathological processes have been associated with hematuria. This syndrome causes impaired blood flow, along with swelling of the distal area of the vein.
Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. With anterior nutcracker syndrome, the left renal vein is compressed between the aorta and the superior mesenteric artery. Posterior nutcracker syndrome with left renal vein duplication. 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. No consensus diagnostic criteria exist to guide endovascular treatment. She was recently diagnosed w nutcracker syndrome and fibro muscular dysplasia, has a history of making kidney stones that are surgically removed, experiences severe pain in her kidneys and liver even though tests show them to be normal, and also experiences severe neurological. To our knowledge, this is the first case of roboticassisted laparoscopic surgery of a nutcracker. 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. 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. 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. The nutcracker phenomenon left renal vein lrv entrapment syndrome refers to compression of the lrv most commonly between abdominal aorta and superior mesenteric artery. A case of persistent hematuria and pain in the presence of a leftsided inferior vena cava. 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.
Nutcracker syndrome is a disorder caused by compression of the left renal vein between the aorta and the superior mesenteric artery sma. Anterior nutcracker syndrome as a rare cause of hematuria. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. It results most commonly from the compression of the left renal vein between the abdominal aorta and superior mesenteric artery. 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. 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.
Nutcracker syndrome ncs is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Left renal vein transposition for nutcracker syndrome. Nutcracker syndrome is a rare condition of left renal vein entrapment manifesting with hematuria and flank pain. Nutcracker syndrome caused by compression of the left renal vein between the. Recall that the longer left renal vein has to pass in front of the aorta and behind the sma before it reaches the inferior vena cava. The ncs or left venous hypertension is a rare clinical manifestation often caused by the compression of left renal vein lrv between abdominal. The nutcracker syndrome or nutcracker phenomenon is, basically, the compression of the left renal vein, usually between the superior mesenteric artery and the aorta.
Current management approach for left renal vein entrapment. Posterior nutcracker syndrome with left renal vein. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. In some cases however pelvic pain remains following left renal vein transposition. It results in left renal venous hypertension, and the subsequent development of venous varicosities of the renal pelvis, ureter. Nutcracker syndrome an overview sciencedirect topics. Nutcracker syndrome is an easily missed cause of hematuria in children. Common signs and symptoms when the condition shows no. Renal nutcracker syndrome genetic and rare diseases. An unusual course of segmental renal artery displays a.
Cystoscopy showed hematuria originating from the left ureter. Hematuria might occur secondary to the rupture of septum separating veins from the collecting system. Nutcracker syndrome definition of nutcracker syndrome by. Some people may not have symptoms, while others develop severe and persistent symptoms. We aimed to evaluate the specificity of lrv compression to ns symptoms. The clinical presentation varies according to the severity of the renocaval pressure gradient but the presence of well developed collaterals or presence of the. The phenomenon is attributable to the idiopathic decrease in the angle between the aorta and the superior mesenteric artery with consequent compression of the left renal. Nutcracker syndrome is a constellation of symptoms resulting from renal vein entrapment or mesoaortic compression of the left renal vein. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. We report a case of nutcracker syndrome, where a roboticassisted laparoscopic transposition of the left renal vein was performed.
Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Renal vein stenting for nutcracker syndrome endovascular. Renal nutcracker syndrome definition of renal nutcracker. Since its description in 1972, the nutcracker syndrome or aortomesenteric left renal vein entrapment syndrome has been mentioned in the literature as an. Some people may not have symptoms, while others develop severe and. Policha a, lamparello p, sadek m, berland t, maldonado t 2016 endovascular treatment of nutcracker syndrome. A case report of a young girl with recurrent hematuria. Rare causes of hematuria associated with various vascular.
These include renal artery aneurysm raa, fibromuscular dysplasia fmd, renal nutcracker syndrome rns, renal. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. The phenomenon results in left renal venous hypertension, which leads to left renal vein and left gonadal vein varices and unilateral hematuria. The presence of hematuria, proteinuria, and pain albeit atypically right sided made it nutcracker syndrome. Nutcracker syndrome and radiographic evaluation of loin. Etiology and evaluation of hematuria in adults uptodate.
Nutcracker syndrome can cause both microscopic and. What each clinical anatomist has to know about left renal. Mesoaortic compression of the left renal vein nutcracker syndrome. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. In this report, we are presenting a case of rare variant. Nutcracker syndrome occurs when nearby arteries compress the left renal kidney vein. This phenomenon with its associated symptoms of unilateral hematuria and left flank pain is known as the nutcracker syndrome. 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, also called renal nutcracker syndrome is a rare vascular condition. Gross haematuria in patients with nutcracker syndrome nefrologia. Nutcracker syndrome ncs, the left renal vein hypertension due to compression between aorta and superior mesenteric artery, may present with hematuria, flank pain and proteinuria. The nutcracker syndrome is associated with left renal vein compression in its passage between the abdominal aorta and superior. Ive been helping take care of a 52 yearold woman who has been chronically ill for some years. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. It has been postulated that the mechanism that produces the hematuria is an increase in left renal vein pressure, which may cause minute rupture of thinwalled veins. Left renal venous hypertension from extrinsic left renal vein compression should be considered in the differential diagnosis of hematuria, especially in. This can lead to renal venous hypertension, resulting in rupture of thinwalled veins into the collecting system with resultant hematuria. Nutcracker syndrome is a variation of nutcracker phenomenon or renal vein.
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. The condition is called nutcracker syndrome because of how the arteries look as they compress the vein, like a nutcracker crushing a nut. The nutcracker syndrome refers to compression of the left renal vein between the aorta and proximal superior mesenteric artery. This patient had gross hematuria secondary to posterior nutcracker syndrome characterized by compression of the anomalous retroaortic left renal vein between the aorta and the spine. 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. Roboticassisted laparoscopic transposition of the left. Symptoms of nutcracker syndromes may include intermittent left flank pain associated with hematuria, proteinuria, and sometimes with symptoms of pelvic congestion. 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. Nutcracker phenomenon or renal vein entrapment is classically seen as a compression of renal vein in between abdominal aorta and superior mesenteric artery with patients being asymptomatic or clinically manifested in the form of nutcracker syndrome as proteinuria, hematuria, flank pain, pelvic congestion in women, and varicocele in men. Left renal vein hypertension, also called nutcracker phenomenon or nutcracker syndrome, is a rare vascular abnormality responsible for gross hematuria. Renal vein entrapment the nutcracker syndrome brandon t. Importance symptoms of nutcracker syndrome are complex and include left flank and abdominal pain, macroscopic or microscopic hematuria, left renaltogonadal vein reflux resulting in pelvic congestion syndrome in females, varicoceles in males, orthostatic proteinuria, lower limb varices, and chronic fatigue symptoms. Since its description in 1972, the aortomesenteric renal vein entrapment has been considered a rare cause of hematuria, with impaired blood outflow, renal venous hypertension and development of collateral circulation.
Persistent or recurrent blood in the urine hematuria is causing anemia. This type of haematuria originating from the left collecting system and is secondary to compression of the left renal vein between the superior mesenteric artery and. The compression causes renal vein hypertension, leading to hematuria which can lead to anemia and abdominal pain classically left flank or pelvic pain. Although posterior ncs is a rare anomaly of renal vein, it can be a cause of hematuria. The left renal vein normally runs between the superior mesenteric artery and aorta, resembling a nut between the jaws of a nutcracker. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Nutcracker syndrome is an uncommonly diagnosed cause of loin pain and hematuria.
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