Nutcracker syndrome caused by compression of the left renal vein between the. 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. Diagnosis and surgical treatment of nutcracker syndrome. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine.
Nutcracker syndrome associated with severe anemia and mild. Scholbach t 2007 from the nutcrackerphenomenon of the left renal vein to the midline congestion syndrome as a cause of migraine, headache, back and abdominal pain and functional disorders of pelvic organs. Ive been helping take care of a 52 yearold woman who has been chronically ill for some years. 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. At the upmc heart and vascular institute, our surgeons in the division of vascular surgery are experts in treating nutcracker syndrome. Several renal vascular pathological processes have been associated with hematuria. We aimed to evaluate the specificity of lrv compression to ns symptoms. It results in left renal venous hypertension, and the subsequent development of venous varicosities of the renal pelvis, ureter. Nutcracker syndrome can cause both microscopic and. 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. Mesoaortic compression of the left renal vein nutcracker syndrome.
In some cases however pelvic pain remains following left renal vein transposition. 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. 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. What each clinical anatomist has to know about left renal. 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 phenomenon results in left renal venous hypertension, which leads to left renal vein and left gonadal vein varices and unilateral hematuria. 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. 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. Posterior nutcracker syndrome with left renal vein duplication. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension.
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 posterior nutcracker syndrome is very rare and is considered when the left renal vein is compressed between the aorta and vertebral column. In this report, we are presenting a case of rare variant. These include renal artery aneurysm raa, fibromuscular dysplasia fmd, renal nutcracker syndrome rns, renal. Since its description in 1972, the nutcracker syndrome or aortomesenteric left renal vein entrapment syndrome has been mentioned in the literature as an. No consensus diagnostic criteria exist to guide endovascular treatment. Some people may not have symptoms, while others develop severe and. 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. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. Nutcracker syndrome ncs is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. 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 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. Several treatment options have been described to relieve associated symptoms. Nutcracker syndrome and radiographic evaluation of loin. A case report of a young girl with recurrent hematuria. Nutcracker syndrome is a rare condition of left renal vein entrapment manifesting with hematuria and flank pain. 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. 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. Rare causes of hematuria associated with various vascular. This can lead to renal venous hypertension, resulting in rupture of thinwalled veins into the collecting system with resultant hematuria. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations.
The left renal vein normally runs between the superior mesenteric artery and aorta, resembling a nut between the jaws of a nutcracker. Anterior nutcracker syndrome as a rare cause of hematuria. 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. Renal nutcracker syndrome genetic and rare diseases. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. 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. This syndrome causes impaired blood flow, along with swelling of the distal area of the vein. 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.
Anterior nutcracker syndrome as a rare cause of hematuria in an 8 year old boy. Left renal vein transposition for nutcracker syndrome. Left renal vein hypertension, also called nutcracker phenomenon or nutcracker syndrome, is a rare vascular abnormality responsible for gross hematuria. Nutcracker syndrome, also called renal nutcracker syndrome is a rare vascular condition. 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. Nutcracker syndrome is a constellation of symptoms resulting from renal vein entrapment or mesoaortic compression of the left renal vein. Nutcracker syndrome is caused by a compression of the left renal vein between the aorta and the superior mesenteric artery. Left renal venous hypertension from extrinsic left renal vein compression should be considered in the differential diagnosis of hematuria, especially in. With anterior nutcracker syndrome, the left renal vein is compressed between the aorta and the superior mesenteric artery. Cystoscopy showed hematuria originating from the left ureter. 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.
Some people may not have symptoms, while others develop severe and persistent symptoms. This phenomenon with its associated symptoms of unilateral hematuria and left flank pain is known as the nutcracker syndrome. We report a 6yearold girl with episodic macroscopic hematuria who was diagnosed as ncs with doppler ultrasound and 3d computerized tomography angiography. Renal vein entrapment the nutcracker syndrome brandon t. Nutcracker syndrome definition of nutcracker syndrome by. Duplex scanning and ct angiography show compression of the left renal vein. 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. It should be considered when patients present with pain in the left flank and intermittent macroscopic hematuria or pelvic congestion syndrome. 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. Etiology and evaluation of hematuria in adults uptodate.
Left renal vein transposition is an efficient surgical treatment for nutcracker syndrome. The nutcracker syndrome refers to compression of the left renal vein between the aorta and proximal superior mesenteric artery. With anterior nutcracker syndrome, the left renal vein is compressed between. 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. Persistent or recurrent blood in the urine hematuria is causing anemia. Common signs and symptoms when the condition shows no.
It is characterized by left renal vein entrapment between the. 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. Nutcracker syndrome is a variation of nutcracker phenomenon or renal vein. 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. The condition is called nutcracker syndrome because of how the arteries look as they compress the vein, like a nutcracker crushing a nut. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. 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. Degree of left renal vein compression predicts nutcracker. Renal vein stenting for nutcracker syndrome endovascular. An unusual course of segmental renal artery displays a. The nutcracker syndrome is associated with left renal vein compression in its passage between the abdominal aorta and superior. 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.
A case of persistent hematuria and pain in the presence of a leftsided inferior vena cava. The presence of hematuria, proteinuria, and pain albeit atypically right sided made it nutcracker syndrome. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. 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 nutcracker syndrome definition of renal 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. The ncs or left venous hypertension is a rare clinical manifestation often caused by the compression of left renal vein lrv between abdominal. Nutcracker syndrome is a disorder caused by compression of the left renal vein between the aorta and the superior mesenteric artery sma. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Nutcracker syndrome is an uncommonly diagnosed cause of loin pain and hematuria.
To our knowledge, this is the first case of roboticassisted laparoscopic surgery of a nutcracker. Symptoms of nutcracker syndromes may include intermittent left flank pain associated with hematuria, proteinuria, and sometimes with symptoms of pelvic congestion. Roboticassisted laparoscopic transposition of the left. Posterior nutcracker syndrome with left renal vein. The nutcracker syndrome or nutcracker phenomenon is, basically, the compression of the left renal vein, usually between the superior mesenteric artery and the aorta. Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. Current management approach for left renal vein entrapment. Nutcracker syndrome an overview sciencedirect topics. The compression causes renal vein hypertension, leading to hematuria which can lead to anemia and abdominal pain classically left flank or pelvic pain. Nutcracker syndrome occurs when nearby arteries compress the left renal kidney vein. Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut. Although posterior ncs is a rare anomaly of renal vein, it can be a cause of hematuria. Gross haematuria in patients with nutcracker syndrome nefrologia.
1323 173 1179 1166 160 1297 4 787 861 1498 75 1473 688 989 1220 1595 306 774 272 363 575 239 1580 1125 328 1186 505 696 519 856 178 1116 280 1215 706 942 605 854