Evaluation of asymptomatic microscopic hematuria in adults. My daughter has nutcracker syndrome testicular disorders. Loin pain and hematuria are common symptoms, presenting in patients of all ages. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. Some people may not have symptoms, while others develop severe and persistent symptoms. In general, for people with mild hematuria or with mild and tolerable symptoms, conservative management is recommended. The nutcracker syndrome has been associated with macroscopic hematuria, and since no other cause of hematuria such as renal or urothelial malignancy was found, we propose that it is the cause of the hematuria. Etiology and evaluation of hematuria in adults uptodate. Nutcracker syndrome refers to the entrapment of the left renal vein in the mesoaortic angle with elevation of left renal vein pressure. Gross haematuria in patients with nutcracker syndrome nefrologia. Nutcracker syndrome has many symptoms for both adults and children, although some especially children dont have any symptoms. Jun 12, 2016 prior to consultation with me she had had an mr venogram showing compression of her left renal vein by the superior mesenteric artery nutcracker phenomena. A rare and potentially under diagnosed cause of haematuria.
Jan 24, 2019 in this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome. 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 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. In women, the hypertension in the left gonadal vein can also cause increased pain during menses. Hematuria that is not explained by an obvious underlying condition eg, cystitis, ureteral stone is fairly common. 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. 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. It is characterized by left renal vein entrapment between the abdominal. Nutcracker syndrome ncs, also known as left renal vein lrv entrapment syndrome is a condition that leads to stenosis of the aortomesenteric region of the lrv, with dilatation of the distal portion of the vessel. Nutcracker syndrome refers to the impingement of the left renal vein lrv located between the abdominal aorta and superior mesenteric artery 4, 5.
Nutcracker syndrome can cause pressure to build up inside your renal veins. 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. Symptoms of nutcracker syndromes may include intermittent left flank pain associated with hematuria, proteinuria, and sometimes with symptoms of pelvic congestion. Nutcracker syndrome caused by compression of the left renal vein between the aorta and superior mesenteric artery is a nonglomerular cause of left. Nutcracker syndrome in children presenting with recurrent. Since some of these causes are potentially serious and may be treatable if identified early, it is important to establish a. The ncs or left venous hypertension is a rare clinical manifestation often caused by the compression of left renal vein lrv between abdominal.
The nutcracker syndrome occurs due to the compression of the left renal vein between the aorta and the superior mesenteric artery. 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 and loin pain hematuria syndrome youtube. Nutcracker phenomenon refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood outflow often accompanied by distention of the distal portion of the vein. Posterior ncs occurs when the persistent posterior branch of fetal periaortic vascular ring gets compressed between aorta and vertebral body. A 32year old woman visited our clinic complaining of gross hematuria. 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. Nutcracker syndrome an overview sciencedirect topics. On the other hand, there is an appreciable risk of malignancy in older patients eg, over age 35 years with hematuria, even if transient. Patients with lphs have persistent and reoccurring loin pain on one or both sides of their body, and blood in their urine. Following are the common symptoms that have been studied in the nutcracker syndrome. For many patients with loin pain hematuria syndrome, having kidney graft surgery is a better option.
Extrinsic compression of the left renal vein lrv between the superior mesenteric artery sma and the aorta ao is known as nutcracker syndrome. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Posts about nutcracker syndrome written by docpark. Nutcracker syndrome definition of nutcracker syndrome by. Nov 06, 2014 term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. Some people may not have symptoms, while others develop severe and. Nutcracker syndrome ncs is an uncommon cause of hematuria. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. We finally went to mayo clinic and she was diagnosed there. Loin pain hematuria syndrome lphs is a rare disorder that may be caused by primary loin pain hematuria syndrome and or nutcracker syndrome, kidney stones and various other kidney diseases. The nutcracker syndrome ncs is the clinical equivalent of. We use cookies to offer you a better experience, personalize content, tailor advertising, provide social media features, and better understand the use of our services.
Two children who presented to us with recurrent gross hematuria were evaluated. Several treatment options have been described to relieve associated symptoms. Renal vein compression syndromes are rare causes of hematuria and can be divided into anterior and posterior nutcracker syndrome. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. Patients may present with hematuria, proteinuria, collateral vessel formation and.
The posterior nutcracker syndrome is very rare and is considered when the left renal vein is compressed between the aorta and vertebral column. Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. It refers to the compression of the left renal vein between the aorta and superior mesenteric artery. Jun 22, 2017 the best treatment for each person with renal nutcracker syndrome ncs is a controversial topic. Nutcracker syndrome symptoms, treatment, causes, surgery. Hematuria should be from the left ureteric orifice only 4. The most common clinical manifestations of nutcracker syndrome are left flank pain, pelvic pain, hematuria and gonadal varices. Nutcracker syndrome and radiographic evaluation of loin. In many such patients, particularly young adult patients, the hematuria is transient and of no consequence. Renal parenchymal disease and abnormalities in the urinary tract were ruled out. Young woman with nutcracker syndrome without main clinic.
Dec 21, 2009 in summary, this case shows that nutcracker syndrome is a potential cause for common symptoms such as loin pain and hematuria, but that a high index of clinical suspicion is required to make the diagnosis. It should be considered when patients present with pain in the left flank and intermittent macroscopic hematuria or pelvic congestion syndrome. Kirby sebro 1, lester goetz 1, satyendra persaud 2 1 division of surgical sciences, university of the west indies, st augustine, trinidad and tobago. Duplex scanning and ct angiography show compression of the left renal vein. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. Another clinical study has shown that nutcracker syndrome is a frequent finding in varicoceleaffected patients and possibly, nutcracker syndrome should be routinely excluded as a possible cause of varicocele and pelvic congestion. Symptoms vary widely and are often exacerbated by physical activity. Nutcracker syndrome consists of haematuria originating from the left collecting system secondary to compression of the left renal vein, between the superior. The diagnosis of the syndrome is often difficult and under diagnosed. Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. Left renal vein transposition for nutcracker syndrome. Nutcracker syndrome is an uncommonly diagnosed cause of loin pain and hematuria. The hematuria resolved and had not recurred with reinitiating warfarin therapy. Nutcracker phenomenon and nutcracker syndrome are sometimes used interchangeably in literatures.
The prevalence of nutcracker syndrome is unknown 1 and while not an inherited condition, cases in siblings have been reported 3. 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. The exam was carried out to look for a cause of macroscopic hematuria. Nutcracker syndrome is an easily missed cause of hematuria in children. These include hematuria, chronic pelvic pain, varicoceles, orthostatic proteinuria and orthostatic intolerance and fatigue 4, 5.
Anatomical configuration of left renal vein entrapment syndrome conforms to the shape of a nutcracker overlaid imaging diagnosis. A case of the nutcracker syndrome developed after delivery. 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. 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 nutcracker syndrome results from compression of the left renal vein between the aorta and the superior mesenteric artery. Management and diagnosis of nutcracker syndromea case. 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. 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. Cae e urg naie roe 21 oue 1 ue 5 the diagnosis of nutcracker syndrome is difficult to establish with routine diagnostic methods 11. This vascular compression may lead to hypertension of the left renal vein and a possible emergence of collateral veins4.
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 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, both microscopic and gross, occurs as a result of rupture of thinwalled varices into the collecting system. Most patients present with both manifestations, but some present with loin pain or hematuria alone. With anterior nutcracker syndrome, the left renal vein is compressed between the aorta and the superior mesenteric artery.
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. Children with iga nephropathy, familial benign hematuria, or alport syndrome can have macroscopic hematuria at the time of, or 1 or 2 days following, an upper respiratory tract infectiona phenomenon. 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. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. We report a 6yearold girl with episodic macroscopic hematuria who was diagnosed as ncs with doppler ultrasound and 3d computerized tomography angiography. The management of ncs depends upon the clinical presentation and the severity of. She started seeing a vascular doctor in april 2017, who diagnosed her with nutcracker syndrome, a disorder that can lead to loin pain hematuria syndrome lphs. The left renal vein normally runs between the superior mesenteric artery and aorta, resembling a nut between the jaws of a nutcracker. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks.
It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Treating loin pain hematuria syndrome with kidney graft. The management of ncs depends upon the clinical presentation and the severity of the lrv hypertension. 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. Although most cases are asymptomatic, this syndrome may result to varicocele, ovarian vein syndrome, hematuria, flank or abdominal pain, lrv hypertension, and pelviuteral varices 3. Hence, patients with unknown causes of unilateral hematuria and flank pain should undergo further studies such as ultrasonography, ct, mri, mra, angiography, or venography to clarify this possibility. Ct angiography revealed a compressed left renal vein with dilatation and hence a diagnosis of nutcracker syndrome was made. 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. Loin pain hematuria syndrome clinic patient stories uw. Renal nutcracker syndrome genetic and rare diseases. 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.
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. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to confirm. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Nutcracker syndrome is more common in women in their. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. Spontaneous resolution of hematuria in children with nutcracker syndrome has been reported following an increase in body mass index.
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. I will also be going into detail about my next steps in my journey since i was. The main clinical manifestations are hematuria and pelvic or back pain. Lphs causes excruciating loin pain on one or both sides of the body and blood in the urine.
Nutcracker syndrome and radiographic evaluation of loin pain. Loin pain hematuria syndrome clinic uw health madison, wi. When the left renal vein is compressed between the aorta and the superior mesenteric artery it causes anterior nutcracker syndrome. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological.
A case report of a young girl with recurrent hematuria. Left renal venous hypertension from extrinsic left renal vein compression should be considered in the differential diagnosis of hematuria, especially. Left renal vein hypertension, also called nutcracker phenomenon or nutcracker syndrome, is a rare vascular abnormality responsible for gross hematuria. Evaluation of asymptomatic microscopic hematuria in adults this is a corrected version of the article that appeared in print. There are various underlying pathologic causes, including renal or ureteric calculi or tumors, intrinsic kidney disease including glomerulonephritis, and loin pain hematuria syndrome. Signs and symptoms of eisenmenger syndrome and treatment options. The most common symptoms of nutcracker syndrome can include. Multidetector ct of vascular compression syndromes in the.
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, also called renal nutcracker syndrome is a rare vascular condition. We report a case of nutcracker syndrome that developed after delivery. Jul 14, 2017 posts about nutcracker syndrome written by docpark. Nephrectomy removing one or both of your kidneys kidney autotransplant. Hematuria is the most commonly reported symptom and is attributed to rupture of thinwalled varices, due to elevated venous. In conclusion, nutcracker syndrome is a rare cause of hematuria and is easily missed by routine diagnostic methods. There are only two treatments for loin pain hematuria syndrome. In this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome. A 32yearold woman visited our clinic complaining of gross hematuria. The phenomenon results in left renal venous hypertension, which leads to left renal vein and left gonadal vein varices and unilateral hematuria.
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