Signs and symptoms of eisenmenger syndrome and treatment options. Nov 22, 2017 these include hematuria, chronic pelvic pain, varicoceles, orthostatic proteinuria and orthostatic intolerance and fatigue 4,5. Nutcracker syndrome ncs is an uncommon cause of hematuria. For patients 18 years old and below, nonsurgical approach is warranted because there is higher likelihood complete resolution 1. 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. Symptoms vary widely and are often exacerbated by physical activity. Nutcracker syndrome associated with severe anemia and mild. Some cases of nutcracker syndrome during pregnancy have been reported 5, 6. Renal parenchymal disease and abnormalities in the urinary tract.
From the department of nephrology, 1 department of radiology, chang gung memorial hospital, taipei. Aortomesenteric angle, hematuria, left renal vein, nutcracker syndrome background. The prevalence of nutcracker syndrome in children with hematuria is. 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. 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. Dilated left gonadal vein and pelvic varices indicate left renal vein lrv ouflow obstruction by the superior mesenteric artery sma. A child with recurrent gross hematuria caused by the nutcracker syndrome. The term nutcracker syndrome is reserved for patients with both clinical and radiological nutcracker features 2.
To assess the role of lrv ultrasonography in the diagnosis of the nutcracker syndrome in children. St josephs childrens hospital, paterson, nj, usa see all articles by this author. We have termed this unusual anatomic finding and its associated symptoms as the inverted nutcracker syndrome, and describe its successful management with nephrectomy and autotransplantation. Rare causes of hematuria associated with various vascular. Several treatment options have been described to relieve associated symptoms. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Most patients present with both manifestations, but some present with loin pain or hematuria alone. The patients condition improved with intravenous infusion of hemostat and better nutrition. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to. Multidetector ct of vascular compression syndromes in the. Shin ji, park jm, lee js, kim mj 2007 effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria.
The hematuria resolved and had not recurred with reinitiating warfarin therapy. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. The phenomenon is attributable to the idiopathic decrease in the angle between the aorta and the superior mesenteric artery with consequent compression of the left renal vein. Diagnosis and surgical treatment of nutcracker syndrome. The left renal vein normally runs between the superior mesenteric artery and aorta, resembling a nut between the jaws of a nutcracker. All consecutive 221 children with isolated hematuria gross and microscopic who visited our nephrology unit between 1 january 2002 and 31 december 2004 were routinely offered a renal doppler ultrasound to detect cases of nutcracker syndrome in addition to a complete blood cell count, electrolytes, biochemistry, coagulation profile, serology for hepatitis b, antinuclear antibody. Fitoz s, ekim m, ozcakar zb, elhan ah, yalcinkaya f, et al. With anterior nutcracker syndrome, the left renal vein is compressed between the. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension.
Renal parenchymal disease and abnormalities in the urinary tract were ruled out. Nutcracker syndrome ncs results most commonly from the compression of the left renal vein by the aorta and vertebral column. The nutcracker syndrome domenico russo, md, roberto minutolo, phd, vittorio iaccarino, md, micheleandreucci, phd, alfredo capuano, md, and francescoa. My daughter has nutcracker syndrome testicular disorders. Nutcracker syndrome an overview sciencedirect topics. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations.
The name derives from the fact that, in the sagittal plane and or transverse plane, the sma and aa with some imagination appear to be a. I would like to find anyone that has been diagnosed with the nutcracker syndrome. One report attributed the gross hematuria to an increase in renal plasma flow during pregnancy. A child with recurrent gross hematuria caused by the. The management of ncs depends upon the clinical presentation and the severity of the lrv hypertension. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and aa with some imagination appear to be a nutcracker crushing a nut the renal vein. Etiology and evaluation of hematuria in adults uptodate. This patient had gross hematuria secondary to posterior nutcracker syndrome characterized by compression of the anomalous retroaortic left renal vein between the aorta and the spine. Case report of gross hematuria in the nutcracker syndrome resolved by renocaval reimplantation. One patient presented with orthostatic proteinuria and 1 with pelvic congestion syndrome. Nutcracker syndrome and radiographic evaluation of loin. In this case, the gross hematuria disappeared after delivery by cesarean section, and the diagnosis of nutcracker syndrome was made. A 32year old woman visited our clinic complaining of gross hematuria. A high index of suspicion and proper imaging is needed to reach.
Nov 01, 20 nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. We believe that this is on the basis of renal vein pressures, and those with robust. In this article, we refer to this heterogeneous group of. Nutcracker syndrome is more common in women in their third or fourth. Ct angiography revealed a compressed left renal vein with dilatation and hence a diagnosis of nutcracker syndrome was made. My main symptoms are left flank pain and unexplained hematuria. Gross haematuria in patients with nutcracker syndrome nefrologia. Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria.
The nutcracker syndrome, caused by compression of the left renal vein between the superior mesenteric artery and the aorta, usually manifests with hematuria, flank pain and proteinuria. Nutcracker syndrome consists of haematuria originating from the left collecting system secondary to compression of the left renal vein, between the superior. Nutcracker syndrome refers to compression of the lrv between the aorta and superior mesenteric artery that results in lrv hypertension and development of collateral veins. Recurrent gross hematuria suggestive of iga nephropathy, familial benign hematuria, nutcracker syndrome, or alport syndrome exerciseinduced hematuria is a transient hematuria that appears immediately after severe exercise such as longdistance running and usually disappears within 48 hours. Nutcracker syndrome in an 84yearold woman with hematuria.
Hematuria was the most common presenting symptom 21 of 23 patients. Renal vein thrombosis is one the more serious complications. We report a case of nutcracker syndrome that developed after delivery. Left renal vein hypertension, also called nutcracker phenomenon or nutcracker syndrome, is a rare vascular abnormality responsible for gross hematuria. However, there are some rare cause of gross hematuria including nutcracker syndrome ncs with occasionally delayed and misdiagnosed, because of different manifestations and absence of consensus on diagnostic criteria 1,2. This compression causes venous hypertension, which results in the formation of renal venous collaterals and subsequent rupture of the septum between the veins and the collecting. Loin pain hematuria syndrome lphs is a rare disorder that may be caused by primary loin pain hematuria syndrome andor nutcracker syndrome, kidney stones and various other kidney diseases. The ct findings are consistent with nutcracker syndrome. 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. I need the name of a doctor at either the mayo clinic andor hopkins that i can treat with, at least to obtain a definitive. 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. Hematuria should be from the left ureteric orifice only 4. A child with recurrent gross hematuria caused by the nutcracker syndrome roberto jodorkovsky, md and edward milman, md clinical pediatrics 2011 51. A case of the nutcracker syndrome developed after delivery.
Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. We finally went to mayo clinic and she was diagnosed there. 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. Jul 29, 2016 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. Nutcracker syndrome is defined as a set of signs and symptoms secondary to compression of the left renal vein lrv in the acute anatomic angle between the aorta. Nutcracker syndrome is an easily missed cause of hematuria in children.
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 syndrome and radiographic evaluation of loin pain. Nutcracker syndrome, which refers to symptoms associated with the above phenomenon, usually presents with acute onset hematuria and abdominal pain, but more rarely, may present as chronic fatigue 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. Gross hematuria is an important concern in pediatric nephrology. All consecutive 221 children with isolated hematuria gross and. I will also be going into detail about my next steps in my journey since i was. It refers to the compression of the left renal vein between the aorta and superior mesenteric artery. It may be obvious from simply looking at the urine gross hematuria, or incidentally found on a urinalysis and or a urine.
Banzic i, fatic n, pejkic s, davidovic l, sladojevic m, koncar i. Savino, phd c left renal vein hypertension, also called nutcracker phenomenonor nutcracker syndrome,is a rare vascular. Although ncs had described in many studies, her prevalence still not known, usual clinicals signs are gross hematuria and flank pain. With anterior nutcracker syndrome, the left renal vein is compressed between the aorta and the superior mesenteric artery. Patients with lphs have persistent and reoccurring loin pain on one or both sides of their body, and blood in their urine. Its common clinical presentation is intermittent microscopic hematuria, repeated attack of gross hematuria as well as left loin pain as in the presentation of this case. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. The reason why this happens is a medical mystery but it is believed that abnormal organogenesis during fetal life is the. The entrapment of the left renal vein is not easily. A 17yearold male was evaluated due to recurrent macroscopic hematuria. Effect of renal doppler ultrasound on the detection of nutcracker. Superimposition of nutcracker syndrome in a hematuric child. Secondary to this anatomic anomaly, the patient experienced a manyyear history of flank pain and intermittent gross hematuria.
Nutcracker syndrome symptoms, treatment, causes, surgery. Left renal vein transposition for nutcracker syndrome. I have found very little information on this syndrome. The best treatment for each person with renal nutcracker syndrome ncs is a controversial topic. 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. In general, for people with mild hematuria or with mild and tolerable symptoms, conservative management is recommended. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to confirm. Nutcracker syndrome in children presenting with recurrent. Nutcracker syndrome in children with gross haematuria. In the absence of clinical symptoms, renal vein compression is referred to as nutcracker phenomenon or nutcracker anatomy, which can be a more common situation. 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. Renal nutcracker syndrome genetic and rare diseases.
Nutcracker syndrome is an uncommonly diagnosed cause of loin pain and hematuria. Jun 22, 2017 the best treatment for each person with renal nutcracker syndrome ncs is a controversial topic. Nutcracker syndrome was suspected based on the combination of clinical examination, ultrasound, ct imaging, and mri imaging. Imaging teaching case nutcracker syndrome and radiographic evaluation of loin pain and hematuria amir bhanji, md, mrcp,1 paul malcolm, frcr,2 and mahzuz karim, phd, frcp1 index words.
Among 51 patients presenting with pelvic congestion, nutcracker syndrome was diagnosed in 9. Superimposition of nutcracker syndrome in a hematuric. Nutcracker syndrome, hematuria nutcracker syndrome refers to the compression of the left renal vein between the aorta and superior mesenteric artery1. Jul 14, 2017 the presence of hematuria, proteinuria, and pain albeit atypically right sided made it nutcracker syndrome. This generally consists of regular surveillance and observation. Nutcracker syndrome is caused by compression of the left renal vein between. A case report of a young girl with recurrent hematuria. Nutcracker syndrome is characterized by gross haematuria caused by left renal vein lrv entrapment. Patients with mild hematuria characterized by microscopic hematuria or short periods of painless gross presence of blood in the urine may be followed up closely without treatment. Loin pain hematuria syndrome lphs is the combination of debilitating unilateral or bilateral flank pain and microscopic or macroscopic amounts of blood in the urine that is otherwise unexplained.
Hematuria, both microscopic and gross, occurs as a result of rupture of thinwalled varices into the collecting system. Posterior ncs occurs when the persistent posterior branch of fetal periaortic vascular ring gets compressed between aorta and vertebral body. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. Normally, the angle between the sma and aa is approximately 450. Request pdf nutcracker syndrome associated with severe anemia and mild proteinuria a 70yearold man was referred to our hospital with the chief complaint of gross hematuria. These include hematuria, chronic pelvic pain, varicoceles, orthostatic proteinuria and orthostatic intolerance and fatigue 4,5. Loin pain hematuria syndrome clinic uw health madison, wi.
Posterior nutcracker syndrome with left renal vein. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. Two children who presented to us with recurrent gross hematuria were evaluated. The prevalence of nutcracker syndrome is unknown 1 and while not an inherited condition, cases in siblings have been reported 3. My daughter has nutcracker syndrome gross hematuria and sickle cells trait diagnosis and management of patients with inherited arrhythmia syndromes what is antiphospholipid syndrome and when it occurs. Nutcracker syndrome is a rare cause of recurrent gross hematuria in children. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. Delayed diagnosis and treatment of the underlying pathology might predispose. Case report of gross hematuria in the nutcracker syndrome.
1014 258 103 141 997 961 1124 1571 479 346 1549 729 57 1151 379 1599 396 549 718 38 1288 381 256 1606 1537 1329 1375 25 386 1517 1268 1264 1331 868 767 1499 1105 53 541 1140 1053 546