Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. This vascular compression may lead to hypertension of the left renal vein and a possible emergence of collateral veins4. The experts of uw health s loin pain hematuria syndrome clinic provide diagnosis and treatment for patients with loin pain hematuria syndrome. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. 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 ncp nutcracker syndrome ncs in figure 1. Delayed diagnosis and treatment of the underlying pathology might predispose.
Symptoms are often aggravated by physical activity and commonly include hematuria, pain or gonadal vein syndrome, varicocele, orthostatic proteinuria, and. In general, for people with mild hematuria or with mild and tolerable symptoms, conservative management is recommended. Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Symptoms of nutcracker syndromes may include intermittent left flank pain associated with hematuria, proteinuria, and sometimes with symptoms of pelvic congestion. Since some of these causes are potentially serious and may be treatable if identified early, it is important to establish a. Common signs and symptoms when the condition shows no. Nutcracker phenomenon and nutcracker syndrome ncbi. The nutcracker syndrome presents with left sided abdominal and flank pain of varying severity, usually in concert with micro or macroscopic hematuria of uncertain origin. I will also be going into detail about my next steps in my journey since i was. Nutcracker phenomenon and nutcracker syndrome mayo clinic.
We finally went to mayo clinic and she was diagnosed there. Degree of left renal vein compression predicts nutcracker. It embraces an extended nonpathognomonic spectrum of symptoms that imply a. Renal nutcracker syndrome genetic and rare diseases. The symptoms of the syndrome include hematuria, which is due to rupture of thinwalled varices into the collecting system, left flank pain and other fatigue symptoms. My kidney function is below normal, but with continuous oversight and a biopsy, it shows i am doing well. The symptoms vary from asymptomatic hematuria to severe pelvic congestion.
It is a rare cause of hematuria, which results from the rupture of congested renal veins into the collecting system. Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to confirm. The prevalence of nutcracker syndrome is unknown 1 and while not an inherited condition, cases in siblings have been reported 3. 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. Effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. No consensus diagnostic criteria exist to guide endovascular treatment. Nutcracker syndrome is an easily missed cause of hematuria in children. Nutcracker syndrome is a clinical diagnosis of a complex of symptoms resembling those seen with nutcracker phenomenon. Hematuria, pelvic or back pain and left varicocele are the most commonly symptoms. Patients with lphs have persistent and reoccurring loin pain on one or both sides of their body, and blood in their urine. The nutcracker syndrome ncs is a rare cause of haematuria.
We aimed to evaluate the specificity of lrv compression to ns symptoms. Cae e urg naie roe 21 oue 1 ue 5 the diagnosis of nutcracker syndrome is difficult to establish with routine diagnostic methods 11. Apr 02, 2018 loin pain hematuria syndrome lphs is diagnosed when all other nonglomerular causes of the symptoms have been ruled out known as a diagnosis of exclusion. This symptom can get triggered due to rigorous exercise regimes. This generally consists of regular surveillance and observation. In addition to ruling out other possible causes, the episodes loin pain and blood in the urine must persist or be. The management of ncs depends upon the clinical presentation and the severity of the lrv hypertension. 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. Endovascular stenting was followed by complete resolution of symptoms. Loin pain and hematuria are common symptoms, presenting in patients of all ages.
Nutcracker syndrome associated with severe anemia and mild. A case of persistent hematuria and pain in the presence of a leftsided inferior vena cava obi ekwenna1, michael a. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Nutcracker syndrome ncs is caused by compression of left renal vein lrv, usually between the aorta and the superior mesenteric artery sma. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. Spontaneous resolution of hematuria in children with nutcracker syndrome has been reported following an increase in body mass index. I have been diagnosed with sarcoidosis which led to a heart transplant two years ago.
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, pelvic congestion and varicose veins in the lower abdomen can cause pain and heaviness in the genital area. Nutcracker syndrome is defined as compression of the left renal vein between the superior mesenteric artery and the aorta and is associated with urinary abnormalities, fig 8a. 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. Nutcracker syndrome, a rare cause of haematuria, is caused by a compression of the lrv between the aa and the sma. Nutcracker syndrome and radiographic evaluation of loin. Naidu 2 and rahmi oklu 2, id 1 department of general surgery, mayo clinic, phoenix, az 85054, usa. The syndrome is characterized by severe intermittent or persistent flank pain. Journal of clinical medicine article degree of left renal vein compression predicts nutcracker syndrome patrick t.
Nutcracker syndrome and loin pain hematuria syndrome youtube. 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. 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. 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. 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. 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. When the left renal vein is compressed between the aorta and the superior mesenteric artery it causes anterior nutcracker syndrome. The hematuria resolved and had not recurred with reinitiating warfarin therapy. Loin pain hematuria syndrome clinic uw health madison, wi. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy.
A rare and potentially under diagnosed cause of haematuria. 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. Symptoms vary widely and are often exacerbated by physical activity. 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. Hematuria is likely due to varices in the collecting system secondary to renal venous hypertension. Nutcracker syndrome, also called renal nutcracker syndrome is a rare vascular condition.
Nov 22, 2017 these include hematuria, chronic pelvic pain, varicoceles, orthostatic proteinuria and orthostatic intolerance and fatigue 4,5. Most patients present with both manifestations, but some present with loin pain or hematuria alone. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. The most common symptoms of nutcracker syndrome can include. Loin pain hematuria syndrome lphs is diagnosed when all other nonglomerular causes of the symptoms have been ruled out known as a diagnosis of exclusion. This can lead to obstruction of flow into the inferior vena cava and secondary left renal venous hypertension. 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.
Doppler ultrasound, computed tomography ct and selective left renal vein phlebography are useful to. In this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome. Glomeruli are the blood cleaning units of the kidneys and are believed to be the source of the bleeding in lphs. Left renal vein transposition for nutcracker syndrome mayo. The posterior nutcracker syndrome is very rare and is considered when the left renal vein is compressed between the aorta and vertebral column.
Posterior nutcracker syndrome with left renal vein. Nutcracker syndrome symptoms, treatment, causes, surgery. Posterior nutcracker syndrome with left renal vein duplication. These include hematuria, chronic pelvic pain, varicoceles, orthostatic proteinuria and orthostatic intolerance and fatigue 4,5. The nutcracker syndrome domenico russo, md, roberto minutolo, phd, vittorio iaccarino, md, micheleandreucci, phd, alfredo capuano, md, and francescoa. It has been postulated that the mechanism that produces the hematuria is an increase in left renal vein pressure, which may cause minute rupture of thinwalled veins. The best treatment for each person with renal nutcracker syndrome ncs is a controversial topic.
There are various underlying pathologic causes, including renal or ureteric calculi or tumors, intrinsic kidney disease including glomerulonephritis, and loin pain hematuria syndrome. Kidney swelling can result, causing the part of the kidney called the renal pelvis to bulge distend, leading to possible kidney scarring and impaired kidney function. 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 got its name because the compression of the renal vein is like a nutcracker cracking a nut. Nutcracker syndrome ncs refers to the clinical manifestations of ncp. Nutcracker syndrome an overview sciencedirect topics. Hydronephrosis occurs when a kidney has an excess of fluid due to a backup of urine, often caused by an obstruction in the upper part of the urinary tract. 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. Variable combinations of symptoms including left flank pain, abdominal pain, hematuria macroscopic or microscopic and proteinuria have been. Jan 24, 2019 in this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome.
Surgical intervention for nutcracker syndrome is the preferred approach in children. The most common clinical manifestations of nutcracker syndrome are left flank pain, pelvic pain, hematuria and gonadal varices. 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. Jan 15, 2020 hydronephrosis occurs when a kidney has an excess of fluid due to a backup of urine, often caused by an obstruction in the upper part of the urinary tract. In women, pelvic congestion and varicose veins in the lower abdomen can. 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. Loin pain hematuria syndrome clinical kidney journal. 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. Savino, phd c left renal vein hypertension, also called nutcracker phenomenonor nutcracker syndrome,is a rare vascular. Common signs and symptoms when the condition shows no symptoms, its usually known. The term nutcracker syndrome is reserved for patients with both clinical and radiological nutcracker features 2.
Renal vein compression syndromes are rare causes of hematuria and can be divided into anterior and posterior nutcracker syndrome. With a diagnosis of nutcracker syndrome, the patient received conservative treatment. Hematuria should be from the left ureteric orifice only 4. Nutcracker syndrome ns refers to symptomatic compression of the left renal vein lrv between the abdominal aorta and superior mesenteric artery with potential symptoms including hematuria, proteinuria, left. Despite potential serious consequences, diagnosing ncs is often challenging, circuitous and commonly delayed. Gorin1, miguel castellan1, victor casillas2, and gaetano ciancio1,3, 1department of urology, 2department of radiology, 3department of surgery. Hangge 1,2, nikhil gupta 2,3, aditya khurana 2,4, keith b. Left renal vein transposition for nutcracker syndrome. My daughter has nutcracker syndrome testicular disorders.
Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Loin pain hematuria syndrome genetic and rare diseases. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. Nutcracker syndrome, which refers to symptoms associated with the above phenomenon, usually presents with acute onset hematuria and abdominal pain. Jun 22, 2017 the best treatment for each person with renal nutcracker syndrome ncs is a controversial topic. Gross hematuria is an important concern in pediatric nephrology. Nutcracker syndrome, hematuria nutcracker syndrome refers to the compression of the left renal vein between the aorta and superior mesenteric artery1. In this syndrome, there is increased pressure on the left renal vein, generating several signs and symptoms, and hematuria is one of present. Posterior ncs occurs when the persistent posterior branch of fetal periaortic vascular ring gets compressed between aorta and vertebral body. 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 painhematuria syndrome lphs is a poorly defined disorder characterized by recurrent or persistent loin flank pain and hematuria that appears to represent glomerular bleeding. Nutcracker syndrome ns refers to symptomatic compression of the left renal vein lrv between the abdominal aorta and superior mesenteric artery with potential symptoms including hematuria, proteinuria, left flank pain, and renal venous hypertension. Nutcracker syndrome, hematuria nutcracker syndrome refers to the compression of the left renal vein between the. Red or dark colored urine, also known as macroscopic hematuria, suggest nutcracker syndrome along with many other conditions. Hematuria, both microscopic and gross, occurs as a result of rupture of thinwalled varices into the collecting system.
Young woman with nutcracker syndrome without main clinic. Micro and macroscopic hematuria caused by renal vein entrapment. Loin pain hematuria syndrome lphs, first described in 1967, is a rare pain syndrome, which is not well understood. She had surgery, which is left renal vein transposition, where. Rare causes of hematuria associated with various vascular. In conclusion, nutcracker syndrome is a rare cause of hematuria and is easily missed by routine diagnostic methods. Nutcracker syndrome is more common in young women, 3 with a maximum incidence peak between 2030 years of age, 3 a range that is in accordance with the case described here. With anterior nutcracker syndrome, the left renal vein is compressed between the. 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. Etiology and evaluation of hematuria in adults uptodate. 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. Among 51 patients presenting with pelvic congestion, nutcracker syndrome was diagnosed in 9.
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. At the upmc heart and vascular institute, our surgeons in the division of vascular surgery are experts in treating nutcracker syndrome. A case of hematuria and flank pain renal and urology news. Posterior nutcracker syndrome in a child journal of.
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. Aug 04, 2011 i also was told, wo blood in urine not a problem, although my nephro at home disagrees. The frequency and severity of the syndrome vary from asymptomatic microhematuria to severe pelvic congestion. A case report of a young girl with recurrent hematuria. 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.
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