Renal vein entrapment the nutcracker syndrome brandon t. Normally, the angle between the sma and aa is approximately 450. However it can be potentially fatal if left completely untreated. The term nutcracker syndrome ncs is used for patients with clinical symptoms while the nutcracker phenomenon describes the anatomical variation without symptoms. Although often asymptomatic, this condition may occur with haematuria more frequently microhaematuria, flank or abdominal pain. Renal nutcracker syndrome genetic and rare diseases. The syndrome involves the compression or narrowing of the renal vein, which causes the slowing of blood flow from the left kidney. Renal nutcracker syndrome is a condition where the left renal vein is compressed by the abdominal aorta and the superior mesenteric artery. The term nutcracker syndrome ns refers to symptoms that arise from the nutcracker phenomenon. 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. Red or dark colored urine, also known as macroscopic hematuria, suggest nutcracker syndrome along with many other.
To assess the frequency and significance of presence of the liver and pancreas at the left renal vein lrv level in patients with suspected renal nutcracker syndrome ncs. Posterior nutcracker syndrome with left renal vein. Endovascular treatment of nutcracker syndrome journal of. 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. They said my colon doesnt function properly so thats what they think is causing it but i also have a compressed renal vein which can cause nutcracker syndrome. 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.
The nutcracker syndrome causes compression of the left renal vein by the arteries near the kidney. It is characterized by left renal vein entrapment between the abdominal. Symptoms usually dont become obvious until the third or fourth decade. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. 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. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. The phenomenon results in left renal venous hypertension. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. Physical examination was normal, and there was no stigma of chronic disease. 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. Both open and endovascular approaches to the treatment of this condition have been described in the literature. Commonly undiagnosed diseases in related medical categories.
Pain management specialists should include nutcracker syndrome in. Using doppler ultrasonography to examine the left renal vein, okada et al. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. A rare, syndromic renal disease characterized by the entrapment of left renal vein lrv between the superior mesenteric artery sma and. Read more about causes of renal nutcracker syndrome. Left renal vein entrapment syndrome, characterized by the compression of the left renal vein lrv between the superior mesenteric artery sma and the abdominal aorta was first described in 1950. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting. Nutcracker syndrome is a rare cause of haematuria caused by the left renal vein lrv entrapment 15, most usually between the aorta and the superior mesenteric artery sma, known as anterior nutcracker syndrome.
Nutcracker phenomenon refers to entrapment of the left renal vein between the. We finally went to mayo clinic and she was diagnosed there. Pelvic congestion in women, or varicose veins in the lower abdomen that may cause pain and heaviness in the pelvis or genital area. 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 underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. Ncp can be due to a variety of causes, including renal or ureteric calculi or tumors, intrinsic kidney disease, and loin pain hematuria syndrome. Nutcracker, maythurner and pelvic congestion explained. 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.
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. Several treatment options have been described to relieve associated symptoms. 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. 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. In nutcracker syndrome, your left renal vein gets compressed by two nearby arteries in your abdomen. Nutcracker syndrome wikimili, the free encyclopedia. Nutcracker syndrome, maythurner syndrome and pelvic congestion syndrome explained by prof mark whiteley of the whiteley clinic. It is associated with the formation of the left renal vein from the aortic collar. Some people may not have symptoms, while others develop severe and persistent symptoms.
When the left renal vein is compressed between the aorta and the superior mesenteric artery it causes anterior nutcracker syndrome. At the upmc heart and vascular institute, our surgeons in the division of vascular surgery are experts in treating nutcracker syndrome. The pain gets highly aggravated by standing, walking, riding, and sitting. Furthermore, the venous return from the left gonadal vein returning to the left renal vein is blocked, thus causing testicular pain. Nutcracker syndrome can cause both microscopic and exerciseinduced hematuria view in chinese exerciseinduced hematuria is nutcracker syndrome, which refers to compression of the left renal vein between the aorta and proximal superior mesenteric artery. Nutcracker phenomenon refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood. The reason why this happens is a medical mystery but it is believed that abnormal organogenesis. What happens if nutcracker syndrome is left untreated. In absence of other causes, the presence of compression of the left renal vein nutcracker syndrome is considered the likely cause of hematuria. 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 an overview sciencedirect topics. A 29yearold female was transferred to our hospital for management of duodenal obstruction. Nutcracker syndrome treatment nutcracker syndrome treatments can vary based on your age, symptoms, and the severity of your condition.
In nutcracker syndrome, symptoms are most often caused when the left renal vein coming from the left kidney becomes compressed and blood. A case report of a young girl with recurrent hematuria. A rare condition where the left renal vein becomes compressed between the abdominal aorta and the superior mesenteric artery. Renal nutcracker syndrome is an anatomic anomaly characterized by the compression of the left renal vein between the superior mesenteric artery and. Nutcracker syndrome ncs is caused by compression of left renal vein lrv, usually between the aorta and the superior mesenteric artery sma. Nutcracker syndrome symptoms, treatment, causes, surgery. Private group for those diagnosed with nutcracker syndrome or renal vein stenosis, and or other vascular compression disorders. Nutcracker syndrome occurs when nearby arteries compress the left renal kidney vein. Causes of renal nutcracker syndrome including triggers, hidden medical causes of renal nutcracker syndrome, risk factors, and what causes renal nutcracker syndrome. Nutcracker syndrome is a rare condition, but its rarity doesnt make it any less painful or difficult to deal with. This can lead to obstruction of flow into the inferior vena cava and secondary left renal venous hypertension. I dont know whats going in but they said not to worry about the renal vein unless my red blood cells are low or peeing out blood life is getting hard. 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 or mesoaortic compression of the left renal vein lrv with associated symptoms related to venous hypertension in the left kidney is a rare entity that may result in severe symptoms requiring operative intervention.
The entrapment of the left renal vein causes distention of the distal portion of the vein, elevated venous pressure and reduced renal perfusion with formation of prominent collateral vessels. The retroaortic or circumaortic renal vein may be compressed between the aorta and the vertebral body, which is called posterior nutcracker. Raising awareness for nutcracker syndrome and venous. 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. Nutcracker phenomenon and nutcracker syndrome mayo clinic.
The most common clinical manifestation of nutcracker syndrome is intermittent macroscopic hematuria, repeated episodes of gross hematuria, and left flank pain. Despite potential serious consequences, diagnosing ncs is often challenging, circuitous and commonly delayed. 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. Current trends in the diagnosis and management of renal. This then presents as abdominal pain and discomfort, or groin pain and pelvic pain. In nutcracker syndrome, the renal vein coming from your left kidney becomes compressed and blood cant flow through it normally. The patient continued to have normal renal function and she was managed conservatively. Renal nutcracker syndrome genetic and rare diseases nih. Furthermore, the venous return from the left gonadal vein returning to the left renal vein is blocked, thus causing testicular pain colloquially referred to as nut pain. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks. Nutcracker syndrome is caused by compression of the left renal vein between the aorta and the superior mesenteric artery where it passes in the fork formed at the bifurcation of these arteries.
Nutcracker syndrome is an easily missed cause of hematuria in children. 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. The syndrome is manifested by left flank and abdominal pain, with or without unilateral. Most problems associated with nutcracker syndrome are dietary.
Left renal vein transposition for nutcracker syndrome. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and. 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. The purpose of this case report is to discuss the diagnosis and conservative management of a patient with both wilkie syndrome and nutcracker syndrome. The most common symptoms of nutcracker syndrome can include. Your left renal vein carries blood out of your left kidney and into your inferior vena cava, your bodys largest vein, so it. Nutcracker phenomenon and nutcracker syndrome ncbi. Learn about the symptoms and treatments for this vascular disease. Renal vein compression syndromes are rare causes of hematuria and can be divided into anterior and posterior nutcracker syndrome. My daughter has nutcracker syndrome testicular disorders. Renal vein stenting for nutcracker syndrome endovascular. Nutcracker syndrome is caused by compression of the left renal vein between the aorta and the superior mesenteric artery where it passes in the fork formed at. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine.