Diagnostic and treatment criteria are not well established, and the natural history of ncs is not well understood. 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. Left renal vein transposition for nutcracker syndrome. We explain the underlying pathophysiology that results in these clinical syndromes so that the patient subset who will benefit from treatment can be identified. This article describes the typical imaging findings and clinical features that are. Isolated microscopic hematuria is very rarely the presenting scenario of wilms tumor 3.
Differing views on definitive diagnostic criteria and varied symptomatology have led to sparse epidemiological data in the literature. 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. The widely used diagnostic method of ncp is renal venography. Nutcracker phenomenon is a rare cause of haematuria resulting from nontraumatic compression of. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared. 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. Ncs typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to. Diagnostic value of computed tomographic findings of 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. 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. We finally went to mayo clinic and she was diagnosed there.
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. Nutcracker syndrome can be difficult to diagnose because its symptoms are similar to many other urological and gynecological disorders. 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. Some people may not have symptoms, while others develop severe and. 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 reason why this happens is a medical mystery but it is believed that abnormal organogenesis during fetal life is the. 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.
This article describes the typical imaging findings and clinical features that are associated with four abdominal vascular compression syndromes. In this article, we refer to this heterogeneous group of. Diagnostic and treatment criteria are not well established, and the natural history of ncs is not. Doppler ultrasonography us has been commonly used for the diagnosis of ncs. The nutcracker syndrome ncs results most commonly from the compression of the left renal. However, an incidental finding of nutcracker phenomenonsyndrome was made. 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. My daughter has nutcracker syndrome testicular disorders. 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 purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. 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. Nutcracker syndrome is a rare condition, but its rarity doesnt make it any less painful or difficult to deal with. 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.
Abstract background nutcracker phenomenon ncp describes the compression of the left renal vein between the aorta and the superior. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. 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 syndrome involves the compression or narrowing of the renal vein, which causes the slowing of blood flow from the left kidney. Common signs and symptoms when the condition shows no symptoms, its usually known. A differential diagnosis of haematuria following a motor. Nutcracker syndrome can be hard for doctors to diagnose, but once its diagnosed, the outlook is often good.
The canadian ct head rule cchr was developed to help physicians determine which minor head injury patients need head ct imaging. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. Nutcracker syndrome was suspected based on the combination of clinical examination, ultrasound, ct imaging, and mri imaging. To our knowledge, this is the first reported case of chronic fatigue syndrome attributable to ns in an adult. Several treatment options have been described to relieve associated symptoms. 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. 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. American college of radiology acr appropriateness criteria. Nutcracker syndrome refers to symptomatic compression of the left renal vein lrv by the aorta and superior mesenteric artery sma. Nutcracker phenomenon and nutcracker syndrome ncbi. Nutcracker syndrome ncs is a syndrome caused by compression of. After investigation no trauma related injuries were identified. A young female presented to the emergency department following a motor vehicle collision. 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 ncs describes left renal vein compression between the superior mesenteric artery and the aorta. It may be associated with the nutcracker phenomenon. The roles of the liver and pancreas in renal nutcracker. One patient presented with orthostatic proteinuria and 1 with pelvic congestion syndrome. 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.
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. Nutcracker syndrome refers to the compression of the left renal vein between. To our knowledge, there are few reports in the literature describing the coexistence of these two conditions and so we take this. 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. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. 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 is rare and refers to the symptom. The selection of the imaging modality is a stepwise process. Computed tomography ct is not appropriate in the initial evaluation of isolated nonpainful, nontraumatic. Ncs is challenging because the diagnostic algorithm and criteria are. Nutcracker syndrome wikimili, the free encyclopedia. Renal nutcracker syndrome genetic and rare diseases. Nutcracker syndrome, vascular imaging with ultrasound longdom. Nutcracker syndrome is diagnosed through imaging such as doppler.
The type of nutcracker syndrome in this case report is known as anterior nutcracker 1,2. Images c and d show stent placement ct images in the axial and coronal plane, respectively. Computed tomography ct revealed nutcracker phenomenon. 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. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. Other types of ncp are a combination of anterior nutcracker and compression of the third portion of duodenum. She complained of left flank pain and was found to have haematuria. Imaging findings and clinical features of abdominal vascular. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous.
Diagnosis and surgical treatment of nutcracker syndrome. 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. 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. Nutcracker syndrome definition of nutcracker syndrome by. Multidetector ct of vascular compression syndromes in the. Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound dus, computed tomography ct, magnetic resonance imaging mri, and venography.
Some people may not have symptoms, while others develop severe and persistent symptoms. Dus is the initial choice after clinical suspicion based on symptoms. Doppler us and ct diagnosis of nutcracker syndrome ncbi. For the diagnosis of ncp, several diagnostic tools are applied, but definite diagnostic criteria have not yet been established. Pdf nutcracker syndrome diagnosed with 3dimensional. He subsequently underwent left renal vein stent insertion with improvement of both fatigue and abdominal pain. 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. I just recently had a major surgery for something called nutcracker syndrome. The nutcracker phenomenon left renal vein lrv entrapment syndrome refers to compression of the lrv most commonly between abdominal aorta and superior mesenteric artery.
The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. We report a case of nutcracker syndrome diagnosed with 3dimensional computed tomography angiography 3d cta. There is a wide spectrum of clinical presentations and diagnostic criteria are not well defined, which frequently results in delayed or incorrect diagnosis. Nutcracker syndrome renal vein entrapment findings. Nutcracker syndrome, vascular imaging with ultrasound a case report. Ncs typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain, without. Degree of left renal vein compression predicts nutcracker. Renal vein entrapment the nutcracker syndrome brandon t. Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut. Following are the common symptoms that have been studied in the nutcracker syndrome. She had surgery, which is left renal vein transposition, where. Imaging findings and clinical features of abdominal. See more ideas about superior mesenteric artery, pelvic congestion syndrome and superior mesenteric artery syndrome. This then presents as abdominal pain and discomfort, or.
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