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 can be hard for doctors to diagnose, but once its diagnosed, the outlook is often good. Nutcracker syndrome is diagnosed through imaging such as doppler. I just recently had a major surgery for something called nutcracker syndrome. Ncs is challenging because the diagnostic algorithm and criteria are. Nutcracker syndrome definition of nutcracker syndrome by.
Axial contrastenhanced ct of the abdomen demonstrated compression of the left renal vein between the superior mesenteric artery sma and the aorta as the left renal vein crosses midline figure 1. Images c and d show stent placement ct images in the axial and coronal plane, respectively. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. The roles of the liver and pancreas in renal 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. Ncs refers to a syndrome caused by significant compression of lrv between the abdominal aorta and sma, resulting in hypertension of the lrv and hematuria due to rupture of the thinwalled veins close to the calyceal fornices 10,11. 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. The canadian ct head rule cchr was developed to help physicians determine which minor head injury patients need head ct imaging.
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. 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 syndrome ncs is a syndrome caused by compression of. It may be associated with the nutcracker phenomenon. Hematuria was the most common presenting symptom 21 of 23 patients. For the diagnosis of ncp, several diagnostic tools are applied, but definite diagnostic criteria have not yet been established. 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. 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. My daughter has nutcracker syndrome testicular disorders. Nutcracker syndrome ncs describes left renal vein compression between the superior mesenteric artery and the aorta. The roles of the liver and pancreas in renal nutcracker.
A differential diagnosis of haematuria following a motor. Nutcracker phenomenon and nutcracker syndrome ncbi. The original validation trial and multiple subsequent studies stiell 2001, stiell 2005, stiell 2010 each found the cchrs high risk criteria to be 100% sensitive for injuries requiring neurosurgical. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. 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. All patients met the criteria for establishing the diagnosis of the nutcracker syndrome. Isolated microscopic hematuria is very rarely the presenting scenario of wilms tumor 3. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared. American college of radiology acr appropriateness criteria. It is associated with the formation of the left renal vein from the aortic collar during the 6th8th week of gestation and abnormal angulation of the sma from the aorta. One patient presented with orthostatic proteinuria and 1 with pelvic congestion syndrome. There is a wide spectrum of clinical presentations and diagnostic criteria are not well defined, which frequently results in delayed or incorrect diagnosis. She had surgery, which is left renal vein transposition, where.
Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric. Nutcracker syndrome is rare and refers to the symptom. Doppler us and ct diagnosis of nutcracker syndrome ncbi. In this article, we refer to this heterogeneous group of. Nutcracker syndrome, vascular imaging with ultrasound longdom. Nutcracker syndrome wikimili, the free encyclopedia. Nutcracker syndrome was suspected based on the combination of clinical examination, ultrasound, ct imaging, and mri imaging. We explain the underlying pathophysiology that results in these clinical syndromes so that the patient subset who will benefit from treatment can be identified.
However, an incidental finding of nutcracker phenomenonsyndrome was made. She complained of left flank pain and was found to have haematuria. Computed tomography ct revealed nutcracker phenomenon. Ncs typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain. Following are the common symptoms that have been studied in the nutcracker syndrome. Other types of ncp are a combination of anterior nutcracker and compression of the third portion of duodenum. 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. The widely used diagnostic method of ncp is renal venography. Some people may not have symptoms, while others develop severe and persistent symptoms. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to. Common signs and symptoms when the condition shows no symptoms, its usually known. Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound dus, computed tomography ct, magnetic resonance imaging mri, and venography.
Objective to study the pathophysiology of the nutcracker syndrome and to describe a new method of treatment subjects and methods computerized tomography ct was used to compare the anatomical relations of the left renal vein with the aorta and the superior mesenteric artery in three patients with the nutcracker syndrome and in a control group of 12 healthy kidney donors. A young female presented to the emergency department following a motor vehicle collision. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. Nutcracker syndrome had been confirmed by conventional venography until recent years. Nutcracker syndrome renal vein entrapment findings. The nutcracker syndrome ncs results most commonly from the compression of the left renal. Diagnosis and surgical treatment of nutcracker syndrome. 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. Pdf nutcracker syndrome diagnosed with 3dimensional.
The reason why this happens is a medical mystery but it is believed that abnormal organogenesis during fetal life is the. We report a case of nutcracker syndrome diagnosed with 3dimensional computed tomography angiography 3d cta. Multidetector ct of vascular compression syndromes in the. 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. See more ideas about superior mesenteric artery, pelvic congestion syndrome and superior mesenteric artery syndrome. He subsequently underwent left renal vein stent insertion with improvement of both fatigue and abdominal pain. Diagnostic and treatment criteria are not well established, and the natural history of ncs is not. Abstract background nutcracker phenomenon ncp describes the compression of the left renal vein between the aorta and the superior. Nutcracker syndrome can be difficult to diagnose because its symptoms are similar to many other urological and gynecological disorders. The selection of the imaging modality is a stepwise process. To our knowledge, this is the first reported case of chronic fatigue syndrome attributable to ns in an adult. 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.
Several treatment options have been described to relieve associated symptoms. Nutcracker syndrome is a rare condition, but its rarity doesnt make it any less painful or difficult to deal with. To our knowledge, there are few reports in the literature describing the coexistence of these two conditions and so we take this. A computed tomography ct imaging of the abdomen performed at outside institution reported as nutcracker syndrome, and b repeat ct venography to specifically evaluate left renal vein demonstrating no compression. Nowadays, with the development of imaging techniques, color doppler sonogram and 3d cta are replacing venography for the diagnosis of nutcracker. This then presents as abdominal pain and discomfort, or. This article describes the typical imaging findings and clinical features that are. Differing views on definitive diagnostic criteria and varied symptomatology have led to sparse epidemiological data in the literature. 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. Renal vein entrapment the nutcracker syndrome brandon t. Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Ncs typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain, without. The nutcracker syndrome ncs is the clinical equivalent of nutcracker. We finally went to mayo clinic and she was diagnosed there.
The syndrome involves the compression or narrowing of the renal vein, which causes the slowing of blood flow from the left kidney. Imaging findings and clinical features of abdominal. Once other conditions have been ruled out, your vascular surgeon may recommend imaging tests, like an ultrasound, ct scan, or mri to screen for nutcracker syndrome. 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. Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut. Nutcracker syndrome, vascular imaging with ultrasound a case report. The type of nutcracker syndrome in this case report is known as anterior nutcracker 1,2. Imaging findings and clinical features of abdominal vascular. Nutcracker syndrome refers to the compression of the left renal vein between. 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. Nutcracker phenomenon is a rare cause of haematuria resulting from nontraumatic compression of.
Some people may not have symptoms, while others develop severe and. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. Ultrasonography, ct, and mri revealed visible entrapment of the left renal vein between the superior. After investigation no trauma related injuries were identified. Diagnostic value of computed tomographic findings of nutcracker syndrome. Nutcracker syndrome is rare and refers to the symptom complex. This article describes the typical imaging findings and clinical features that are associated with four abdominal vascular compression syndromes.
Left renal vein transposition for nutcracker syndrome. 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. Diagnostic and treatment criteria are not well established, and the natural history of ncs is not well understood. Renal nutcracker syndrome genetic and rare diseases. Nutcracker syndrome refers to symptomatic compression of the left renal vein lrv by the aorta and superior mesenteric artery sma. Raising awareness for nutcracker syndrome and venous.
This syndrome results most commonly from the compression of the left renal vein between the abdominal aorta aa and superior mesenteric artery sma, although other variants exist. Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. When symptomatic, such compressions can result in a variety of uncommon syndromes in the abdomen and pelvis, including median arcuate ligament syndrome mals, maythurner syndrome, nutcracker syndrome, superior mesenteric artery sma syndrome, upj obstruction, ovarian vein syndrome ovs, and other forms of ureteral compression. Degree of left renal vein compression predicts nutcracker. Computed tomography ct is not appropriate in the initial evaluation of isolated nonpainful, nontraumatic. It was first described in 1950 by elsadr and mina and was named in 1971 1,2,3,4. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Dus is the initial choice after clinical suspicion based on symptoms.
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