Duplex ultrasound diagnosis and followup of nutcracker. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. They have not been typeset and the text may change before final. 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. Triplex ultrasound nutcraker syndrome dr ahmed esawy. Dus is the initial choice after clinical suspicion based on symptoms. Diagnosis of the nutcracker syndrome with color doppler. Maythurner syndrome mts is an anatomically and pathologically variable condition leading to venous outflow obstruction as a result of extrinsic venous compression in the iliocaval venous territory. Radiographic features are similar on ultrasound, doppler ultrasound, ct, mri, and conventional angiography. Nutcracker phenomenon and nutcracker syndrome the nutcracker phenomenon is by far the most frequent vascular compression syndrome. The selection of the imaging modality is a stepwise process. Nutcracker syndrome refers to the compression of the left renal vein.
The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal doppler ultrasound as a screening examination has a substantial effect on. 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. 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. The nutcracker syndrome in a venous or even broader scope, vascular compression syndromes is based upon decreased space between the superior mesenteric artery and the aorta in the area of the left renal vein. Criteria for defining significant central vein stenosis with duplex ultrasound. Diagnosis of the nutcracker syndrome with color doppler sonography. Current trends in the diagnosis and management of renal nutcracker syndrome. Common signs and symptoms when the condition shows no symptoms, its usually known. Maythurner syndrome also known as iliac vein compression syndrome is one of them. The nutcracker syndrome is a rare clinic condition associated with severe hematuria and left flank pain due to the entrapment of the left renal vein between the superior mesenteric artery and the.
Svensson christina, department of medicine and health science. The purpose of this study was to evaluate the clinical characteristics of 44 pediatric patients who were diagnosed as having nutcracker syndrome ncs. Common signs and symptoms when the condition shows no. Usg shows compression of the left renal vein between the aorta and the superior mesenteric artery so. Jun 22, 2017 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. Color duplex sonography of the nutcracker syndrome. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal doppler ultrasound as a. Evidence of secondary pelvic congestion syndrome nutcracker syndrome or maythurner syndrome patient preparation for a pelvic congestion syndrome ultrasound exam. Multidetector ct of vascular compression syndromes in the. Diagnosing nutcracker syndrome and pelvic congestion syndrome. Fitoz s, ekim m, ozcakar zb, elhan ah, yalcinkaya f, et al. It occurs when arteries, most often the abdomens aorta and superior mesenteric artery, squeeze the left renal kidney vein.
Other types of ncp are a combination of anterior nutcracker and compression of the third portion of duodenum that courses in front of the lrv between the aorta and the sma. In addition, intrinsic anatomic differences in the amp and hilar portion of the lrv could be directly responsible for the onset of varicocele. Worlds first surgical procedure for nutcracker syndrome was very. It is basically caused and aggravated by the upright, bipedal human gait. Our protocol for transabdominal pelvic vein duplex ultrasound. Doppler ultrasound measurements of the anteriorposterior ap. This typical human posture is the reason for a swinging configuration of the human spine that is characterized by a socalled lordosis of the lumbar spine. Some people may not have symptoms, while others develop severe and. Mar 22, 2018 nutcracker syndrome ncs refers to the clinical manifestations of ncp. Effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria 19 october 2006 european journal of pediatrics, vol.
Color duplex sonography of the nutcracker syndrome aortomesenteric left renal vein compression a. Nutcracker syndrome symptoms, treatment, causes, surgery. Left renal vein transposition for nutcracker syndrome. Nutcracker syndrome ultrasound sunday, november 06, 2011 nutcracker syndrome young male with painless hematuria. A case report of a young girl with recurrent hematuria. Color doppler ultrasound to look for flow away from the lrv has a diagnostic sensitivity of 78% and specificity of 100% for nutcracker syndrome 9. Hematuria was the most common presenting symptom 21 of 23 patients. This condition is known as willkie syndrome or superior mesenteric artery. Shin ji, park jm, lee js, kim mj 2007 effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. Therefore, we carried out a prospective study of routine renal doppler ultrasound in children presenting with isolated hematuria at our nephrology unit. Usg shows compression of the left renal vein between the aorta and the superior mesenteric artery so nutcracker syndrome. However, normal flow also can exist in distended left renal veins. Nutcracker phenomenon scholbach sonographic perfusion. One patient presented with orthostatic proteinuria and 1 with pelvic congestion syndrome.
Radiographic features are similar on ultrasound, doppler ultrasound, ct, mri, and. Several anatomical anomalies can result in deep vein thrombosis dvt. To assess the detection rate of nutcracker syndrome in children with isolated hematuria, renal doppler ultrasound examinations were routinely performed on 216 consecutive children 176 microscopic hematuria and 40 gross hematuria. Nutcracker syndrome is a vascular compression disorder that refers to the. No food or drink 8 hours prior to to the pcs exam and schedule a morning appointment to reduce the amount of overlying bowel gas. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared. Triplex ultrasound nutcraker syndrome dr ahmed esawy youtube. Worlds first surgical procedure for nutcracker syndrome was very successful. The term nutcracker phenomenon ncp refers to compression of the left renal vein lrv between the aorta and the superior mesenteric artery sma.
Several treatment options have been described to relieve associated symptoms. Aims this paper aims to provide education and ultrasound protocol for clinicians and sonographers who encounter patients with the symptoms of ncs during their course of practice. Sonographic evidence of nutcracker phenomenon, an incidental finding in a patient referred to investigate cholecystitis. It can cause many symptoms in both children and adults, such as flank pain and blood in the urine. Nutcracker syndromeultrasound sunday, november 06, 2011 nutcracker syndrome young male with painless hematuria. 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. Diagnosed as both nutcracker ns and wilkie ws syndrome. This can lead to obstruction of flow into the inferior vena cava and secondary left renal venous hypertension. She had surgery, which is left renal vein transposition, where. Pelvic congestion syndrome ultrasound exam, risk factors. Effect of renal doppler ultrasound on the detection of. 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 syndrome, vascular imaging with ultrasound longdom. Effect of renal doppler ultrasound on the detection of nutcracker.
Renal nutcracker syndrome genetic and rare diseases. My daughter has nutcracker syndrome testicular disorders. Nutcracker syndrome ncs is caused by compression of left renal vein lrv, usually between the aorta and the superior mesenteric artery sma. Feb 19, 2015 16 year old female patient, presenting with nausea, vomiting, abdominal pain and hematuria. 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. Imaging findings and clinical features of abdominal. To assess diagnosis of the nutcracker renal vein entrapment syndrome with doppler ultrasonography us. The nutcracker syndrome can exist in either nondistended or distended left renal veins.
Nutcracker syndrome ncs describes left renal vein compression between the. Common treatments for nutcracker syndrome include stenting, surgery, and. Nutcracker syndrome ncs describes left renal vein compression between the superior mesenteric artery and the aorta. The proposed ultrasound techniques are a valid protocol extension to the ultrasound examination to help diagnose ncp. 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. Transverse ultrasound image demonstrates compression of the left renal vein lrv between the superior mesenteric artery sma and aorta ao courtesy of ahmad alomari, md. Accepted manuscript manuscripts that have been selected for publication. Despite potential serious consequences, diagnosing ncs is often challenging, circuitous and commonly delayed. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal doppler ultrasound as a screening examination has a substantial effect on the detection of nutcracker syndrome. The type of nutcracker syndrome in this case report is known as anterior nutcracker 1,2. 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. Which specialists are essential to meet, what tests are needed and other useful information for the diagnosis of nutcracker syndrome. We also investigated the left renal vein doppler ultrasonography dus results, to determine whether or not there was an association between clinical symptoms and dus findings among these patients.
Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound dus, computed tomography ct, magnetic resonance imaging mri, and venography. Triplex ultrasound median arcuate ligament syndrome dr ahmed. Nutcracker syndrome is an easily missed cause of hematuria in children. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. Compression of the left renal vein lrv between aorta and superior mesenteric artery sma, resulting haematuria, flank pain, varicocele, etc. Stents, embolisation, ultrasoundcolour doppler, ctangiography, catheter. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks. Doppler ultrasound measurements of the anteriorposterior ap diameter and peak velocities of the left renal vein may be helpful in diagnosing nutcracker syndrome.
Nutcracker syndromeultrasound sumers radiology blog. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Nutcracker syndrome ncs, also referred to as left renal vein entrapment, is characterized by left renal vein compression between the superior mesenteric artery and aorta. Nutcracker syndrome ncs refers to the clinical manifestations of ncp. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. However, studies show that among these diagnostic tools, doppler ultrasonography which is a noninvasive modality should be the initial assessment tool after nutcracker syndrome is suspected clinically, or when there is an increased diameter ratio between the distended and narrow portions on ct scan and mr imaging 2. Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut.
Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Oct 04, 2019 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. Our findings indicate that nutcracker syndrome is a frequent finding in varicoceleaffected patients and should be routinely excluded as a possible cause of varicocele. 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. Following are the common symptoms that have been studied in the nutcracker syndrome. The patient stated that he did not feel an ache in his scrotum and that his blood pressure was normal. The peak velocity pv was measured at the hilar portion of the left renal vein. In addition, intrinsic anatomic differences in the amp and hilar portion of the lrv.
Nutcracker syndrome ncs is a rare clinical condition that occurs when the left renal vein lrv experiences venous outflow obstruction secondary to external vascular compression, most often between the aorta and superior mesenteric artery sma. On ct, the beak sign is an abrupt narrowing of the lrv between the aorta and sma, with proximal dilatation of the lrv. In this article, we refer to this heterogeneous group of. Renal doppler ultrasound was also performed on 32 healthy normal children. Treatment usually involves renal vein percutaneous angioplasty with or without stent placement. The ability of color doppler sonography to reveal color flow in collateral veins is useful for the noninvasive diagnosis of the nutcracker syndrome. Nutcracker syndrome was suspected based on the combination of clinical examination, ultrasound, ct imaging, and mri imaging. We finally went to mayo clinic and she was diagnosed there. Color duplex sonography of the nutcracker syndrome aortomesenteric left renal vein compression, journal of ultrasound in medicine, vol. The term syndrome refers to a group of signs and symptoms primarily occurring together, characteristic of a specific condition. 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 ncs or left renal vein lrv entrapment is rare and may be caused by compression of the vein between the aorta. Oct 19, 2006 recently, however, renal doppler ultrasound has been developed to diagnose nutcracker syndrome, and it is now possible to apply this diagnostic method to children 1012. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Imaging findings and clinical features of abdominal vascular. 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. With partial venous obstruction, the condition can be asymptomatic, but progression with symptoms related to chronic venous hypertension or. Nov 22, 2017 triplex ultrasound nutcraker syndrome dr ahmed esawy. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong.
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