Shin ji, park jm, lee js, kim mj 2007 effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. Micro and macroscopic hematuria caused by renal vein entrapment. Doppler ultrasonographic assessment of left renal vein diameter and peak velocity or computed tomography. Symptoms include hematuria, orthostatic proteinuria, flank pain, abdominal pain, varicocele, dyspareunia, dysmenorrhea, fatigue, and orthostatic intolerance 2.
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. Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. The frequency of hematuria due to nutcracker syndrome in children appears to be highest in asia. Loin pain hematuria syndrome lphs is a poorly defined disorder characterized by recurrent or persistent loin flank pain and hematuria that appears to represent glomerular bleeding. Its main abdominal branches are the celiac trunk, superior mesenteric artery, bilateral renal arteries and inferior mesenteric artery. Using doppler ultrasonography to examine the left renal vein, okada et al. Fitoz s, ekim m, ozcakar zb, elhan ah, yalcinkaya f, et al. Nutcracker syndrome in an 84yearold woman with hematuria. Doppler sonographic findings in 47 children with orthostatic proteinuria and in 27 healthy. Patient is a 43 year old woman who had been having bouts of severe left sided abdominal pain for several years with worsening episodes of nausea and vomiting resulting in several visits to the emergency room. Nutcracker syndrome symptoms, treatment, causes, surgery. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. 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.
Hematuria and flank pain can also cause renal vein thrombosis, 54. In a patient with an unremarkable workup for the common causes of hematuria andor flank pain, a multimodality approach of doppler us followed by either ct or mr venography will often suggest a diagnosis of ncs. This might be due to the absence of both reliable and easily applicable diagnostic methods in diagnosing nutcracker syndrome in children. All imaging modalities have been used during diagnostic workup. Radiographic features are similar on ultrasound, doppler ultrasound, ct, mri. 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. To assess diagnosis of the nutcracker renal vein entrapment syndrome with doppler ultrasonography us.
Nutcracker syndrome is a rare cause of recurrent gross hematuria in. Nutcracker syndrome and radiographic evaluation of loin pain. Cae e urg naie roe 21 oue 1 ue 5 the diagnosis of nutcracker syndrome is difficult to establish with routine diagnostic methods 11. The term nutcracker syndrome ncs is used for patients with clinical symptoms while the nutcracker phenomenon describes the anatomical variation without symptoms. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal doppler ultrasound as a. The patients condition improved with intravenous infusion of hemostat and better nutrition. Superimposition of nutcracker syndrome in a hematuric child. Renal nutcracker syndrome genetic and rare diseases. To identify factors affecting spontaneous resolution of hematuria in children with nutcracker syndrome, 20 patients diagnosed as having nutcracker syndrome using renal doppler ultrasound us were analyzed retrospectively. Ct angiography revealed a compressed left renal vein with dilatation and hence a diagnosis of nutcracker syndrome was made. Nutcracker syndrome in children presenting with recurrent. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Superimposition of nutcracker syndrome in a hematuric.
The most common symptoms of nutcracker syndrome can include. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting. Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound dus, computed tomography ct, magnetic resonance imaging mri, and venography. Effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. Doppler sonographic evaluation of the left renal veincheon je, kim ws, kim io, et al seoul natl univ, republic of korea pediatr radiol 36. To assess the role of lrv ultrasonography in the diagnosis of the nutcracker syndrome in children. A case report of a young girl with recurrent hematuria.
It is due to the compression of the distal segment of the left renal vein lrv between the superior mesenteric artery sma and the aorta also called left renal vein entrapment. A high index of suspicion and proper imaging is needed to reach a proper diagnosis and avoid the psychological and financial stress on the family. Effect of renal doppler ultrasound on the detection of. The purpose of this study was to evaluate the clinical characteristics of 44 pediatric patients who were diagnosed as having nutcracker syndrome ncs. Diagnosis of nutcracker phenomenon should be suspected when anteroposterior diameter and peak velocity at these two points exceeds by a factor of five. Nutcracker syndrome in children with gross haematuria. Additional symptoms related to pelvic congestion syndrome also may be present. Diagnostic value of renal doppler ultrasonography for. Mar 22, 2018 the aortasma angle was measured at 15. Renal doppler ultrasound was also performed on 32 healthy normal children. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or. Rare causes of hematuria associated with various vascular. Ncs typically presents as pelvic pain, flank pain, hematuria, gonadal varices varicocele or ovarian vein syndrome, orthostatic proteinuria, and. Nutcracker syndrome is a complex of clinical symptoms due to compression of the left renal vein between abdominal aorta and superior mesenteric artery.
Two children who presented to us with recurrent gross hematuria were evaluated. Of those, 40% were found to have ncp by renal doppler. This can lead to renal venous hypertension, resulting in rupture of thinwalled veins into. 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. Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. The consequences may vary between symptomfree conditions through moderate proteinuria and hypertension to severe hematuria.
Aortomesenteric angle, hematuria, left renal vein, nutcracker syndrome background. Nutcracker syndrome accompanying pelvic congestion syndrome. Diagnosis of the nutcracker syndrome with color doppler. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. Kirby sebro 1, lester goetz 1, satyendra persaud 2 1 division of surgical sciences, university of the west indies, st augustine, trinidad and tobago. Nutcracker syndrome ncs refers to the clinical manifestations of ncp. The sensitivity and specificity of color doppler sonography for revealing the nutcracker syndrome were 78% and 100%, respectively, when color flow in collateral veins was included in the diagnostic criteria. A simplified approach with gonadal vein transposition posted on june 12, 2016 june 12, 2016 by docpark the patient is a young woman in her twenties who developed severe right sided abdominal and back pain about 4 months prior to presentation associated with bouts of bloody urine. The peak velocity pv was measured at the hilar portion of the left renal vein lrv and. The nutcracker syndrome is caused by tight compression of the left renal vein between the superior mesenteric artery and the abdominal aorta.
The velocity of the lrv flow proximal to the narrowing was 23 cmsec with an increase to 350 cms at the narrowing. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to confirm. Left renal vein entrapment syndrome, also known as nutcracker syndrome, involves compression of the left renal vein and can present with abdominopelvic pain, hematuria, and symptoms of pelvic congestion syndrome. Nutcracker syndrome and radiographic evaluation of loin. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. 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. The aorta is the largest artery in the human body, it begins at heart and branches out to supply oxygenated blood to all the viscera and extremities. We successfully obtained doppler spectra from the lrv at the aortomesenteric and hilar portions in 36 of 44 patients. Factors affecting spontaneous resolution of hematuria in. The left renal vein normally runs between the superior mesenteric artery and aorta, resembling a nut between the jaws of a nutcracker. Kimeffect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria eur j. These cases are rare, and it is not clear whether the nutcracker syndrome is really the etiology.
A history of recent substantial weight loss also is implicated in cases of nutcracker syndrome 57. Nutcracker syndrome is an easily missed cause of hematuria in children. 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 management of ncs depends upon the clinical presentation and the severity of the lrv hypertension. Posterior ncs occurs when the persistent posterior branch of fetal periaortic vascular ring gets compressed between aorta and vertebral body. Ab to identify factors affecting spontaneous resolution of hematuria in children with nutcracker syndrome, 20 patients diagnosed as having nutcracker syndrome using renal doppler ultrasound us were. Clinical course of children with nutcracker syndrome. Nutcracker syndrome is a rare cause of recurrent gross hematuria in children. Nutcracker syndrome symptoms and diagnosis nutcracker syndrome symptoms. 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.
Evaluation of microscopic hematuria in children uptodate. Computed tomography angiography cta was used in 8 patients for the diagnostic purposes along with dus due to visualize the am portions of the lrv. Posterior nutcracker syndrome with left renal vein. 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. Color doppler ultrasound cdus and computed tomography ct images showed reduction of the angle between the. However, in some cases, no cause is identified unless rarer pathologic states are considered. 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 is characterized by gross haematuria caused by left renal vein lrv entrapment. Effect of renal doppler ultrasound on the detection of nutcracker. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. In this paper a case of 12yearold girl with rare association of nutcracker and alport syndrome is presented who admitted for persistent severe microscopic hematuria, recurrent gross hematuria, occasional vomiting, loin pain and positive family history of microscopic hematuria associated with hearing deficit. 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. In conclusion, ncp might be one of the leading causes of orthostatic proteinuria, and non. If the data is obtained, it can be used as the criteria for ncp, and doppler us will become a powerful diagnostic tool in the diagnosis of ncp. Nutcracker phenomenon and nutcracker syndrome mayo clinic. Left renal vein transposition for nutcracker syndrome. Hematuria and flank pain are common clinical symptoms 58. Doppler ultrasound measurements of the anteriorposterior ap.
Flank pain, hematuria, nutcracker syndrome introduction nutcracker syndrome is a clinical condition that develops as a result of the compression of the left renal vein. Recently, however, renal doppler ultrasound has been developed to diagnose nutcracker syndrome. Renal parenchymal disease and abnormalities in the urinary tract were ruled out. We describe a patient with loin pain and hematuria who proved to be a diagnostic dilemma for several years before the diagnosis of nutcracker syndrome was established. Nutcracker syndrome in children with orthostatic proteinuria. Jul 14, 2017 posts about nutcracker syndrome written by docpark. The diagnosis of the nutcracker syndrome was suspected based on clinical examination, doppler scan, duplex ultrasound scan, computed tomography scan, and magnetic resonance imaging. Nutcracker syndrome is commonly found in thin young females.
All patients underwent lrv dus as the initial diagnostic test with suspected nutcracker syndrome. 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. A 30year old male patient presented with hematuria and leftflank pain that was aggravated by exercise. Instead, blood can flow backwards into other veins and cause them. Nutcracker syndrome has many symptoms for both adults and children, although some especially children dont have any symptoms. The nutcracker syndrome can exist in either nondistended or distended left renal veins. Some people may not have symptoms, while others develop severe and persistent symptoms. Although spontaneous resolution of hematuria in children with nutcracker syndrome is obscure, our findings suggest the increase in bmi may be a possible hemodynamic factor. 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 is an uncommonly diagnosed cause of loin pain and hematuria. 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. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. 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. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared.
Recommended protocol in instances of suspected nutcracker phenomenon. Current trends in the diagnosis and management of renal. A diagnosis of nutcracker syndrome was made figures 3 and 4. Several treatment options have been described to relieve associated symptoms. We report a 6yearold girl with episodic macroscopic hematuria who was diagnosed as ncs with doppler ultrasound and 3d computerized tomography angiography. Most patients present with both manifestations, but some present with loin pain or hematuria alone. Nutcracker syndrome servier phlebolymphologyservier. Doppler ultrasonography us has been commonly used for the diagnosis of ncs. 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. Patients with lphs have persistent and reoccurring loin pain on one or both sides of their body, and blood in their urine. In 1974, pastershank 31 reported the first case of surgical treatment of a patient with the nutcracker syndrome in english literature. Nutcracker syndrome is a vascular compression disorder that refers to the. Doppler ultrasound measurements of the anteriorposterior ap diameter and peak velocities of the left renal vein may be helpful in diagnosing nutcracker syndrome. Doppler ultrasound showed left renal vein compression while 3 d.
Dec 21, 2009 patients therefore often undergo multiple investigations and procedures. Nutcracker syndrome refers to the compression of the left renal vein. 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. Doppler ultrasound, computed tomography ct and selective left. In nutcracker syndrome, the renal vein coming from your left kidney becomes compressed and blood cant flow through it normally. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. Fortyfour patients with hematuria of unknown origin underwent color doppler sonography, renal arteriography, retrograde left renal venography, and renocaval.
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