A rare, syndromic renal disease characterized by the entrapment of left renal vein. Nutcracker syndrome and loin pain hematuria syndrome youtube. Treatment decision should be based on the severity of symptoms and their expected reversibility with regard to patients age and the stage of the syndrome 27. Loin pain and hematuria are common symptoms, presenting in patients of all ages. Hematuria should be from the left ureteric orifice only 4. Rare causes of hematuria associated with various vascular. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. 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. Nov 09, 2016 nutcracker syndrome treatment options. The available options are subdivided into four groups, namely surveillance, open surgical procedures, intra or extra vascular stents, and lastly intrapelvic chemical cauterisation 1.
Nutcracker syndrome and radiographic evaluation of loin pain. 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. The major causes of flank pain and hematuria, such as nephrolithiasis and blood clot, were not present. It is characterized by severe, often incapacitating loin flank pain that may be intermittent or constant, unilateral or bilateral, and accompanied by either microscopic or. The main presenting symptom is hematuria, with or without left flank pain. Ncs usually present in women and children, ranging from asymptomatic to variable symptoms including recurrent macroscopic hematuria with isomorphic urine red blood cells, left flank pain, pelvic congestion, orthostatic intolerance, and unilateral proteinuria without renal impairment 68, which may aggravate by exercise.
Etiology and evaluation of hematuria in adults uptodate. Nutcracker syndrome is an easily missed cause of hematuria in children. Nutcracker syndrome symptoms, treatment, causes, surgery, radiology. My daughter has nutcracker syndrome testicular disorders. 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. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks. 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. This symptom can get triggered due to rigorous exercise regimes.
Nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. 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. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. The abdominal pain may improve or worsen depending on positioning. Following are the common symptoms that have been studied in the nutcracker syndrome. Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut. Diagnosis and surgical treatment of nutcracker syndrome. Among the different diagnostic algorithms that are commonly used in outpatient nephrology consultations, nutcracker syndromephenomenon is a very rare cause.
Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound dus, computed tomography ct, magnetic resonance imaging mri, and venography. 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. The best treatment for each person with renal nutcracker syndrome ncs is a controversial topic. Red or dark colored urine, also known as macroscopic hematuria, suggest nutcracker syndrome along with many other conditions. Nutcracker syndrome, which refers to symptoms associated with the above phenomenon, usually presents with acute onset hematuria and abdominal pain. Posterior nutcracker syndrome with left renal vein duplication. A case report of a young girl with recurrent hematuria. Once symptoms occur its called nutcracker syndrome. She had surgery, which is left renal vein transposition, where. Introduction loin pain and hematuria are common symp. The classic symptoms of this syndrome include left flank pain with gross or microscopic hematuria. Dec 21, 2009 loin pain and hematuria are common symptoms, presenting in patients of all ages. 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.
Patients with lphs have persistent and reoccurring loin pain on one or both sides of their body, and blood in their urine. Loin pain hematuria syndrome lphs is a rare but very painful disorder involving the kidneys or urinary tract. With a diagnosis of nutcracker syndrome, the patient received conservative treatment. The signs and symptoms of ncs are all derived from the outflow obstruction of the left renal vein. 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. Treatment of nutcracker syndrome has been evolving in the last four decades ranging from observation to nephrectomy, depending on the severity of symptoms 3. Nutcracker syndrome an overview sciencedirect topics. The prevalence of nutcracker syndrome is unknown 1 and while not an inherited condition, cases in siblings have been reported 3. Most patients present with both manifestations, but some present with loin pain or hematuria alone. 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. Renal nutcracker syndrome genetic and rare diseases. Posterior nutcracker syndrome with left renal vein.
In this article, we refer to this heterogeneous group of. The most common symptoms of nutcracker syndrome can include. This phenomenon with its associated symptoms of unilateral hematuria and left flank pain is known as the nutcracker syndrome. Symptoms vary widely and are often exacerbated by physical activity. Loin pain hematuria syndrome clinic uw health madison, wi. Common signs and symptoms when the condition shows no.
Since some of these causes are potentially serious and may be treatable if identified early, it is important to establish a. Symptoms can include hematuria micro and macroscopic, orthostatic proteinuria, left flank pain, abdominal pain, left lowerextremity varicose veins, left. We present a rare case report of a posterior nutcracker syndrome diagnosed in a young lady with longstanding symptoms that required surgical intervention. Increased quantities of blood are eliminated through the urine. Posterior ncs occurs when the persistent posterior branch of fetal periaortic vascular ring gets compressed between aorta and vertebral body. In order to make an accurate diagnosis, the most important thing for a physician to be aware of the symptoms that can be present when a patient first shows manifestations of nutcracker syndrome. However, the nutcracker syndrome may also be associated with an array of pelvic and lower extremity symptoms in the absence of hematuria. Dus is the initial choice after clinical suspicion based on symptoms. The reason why this happens is a medical mystery but it is believed that abnormal organogenesis during fetal life is the. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery.
The term nutcracker syndrome is reserved for patients with both clinical and radiological nutcracker features 2. Ct angiography revealed a compressed left renal vein with dilatation and hence a diagnosis of nutcracker syndrome was made. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Nutcracker syndrome symptoms, treatment, causes, surgery. Nov 22, 2017 the nutcracker syndrome, first reported in 1950, is said to occur when the nutcracker phenomenon is accompanied by clinical symptoms including chronic abdominal pain and haematuria. However, recurrent gross hematuria with anemia, severe flank pain, renal functional impairment. 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. Mild and tolerable symptoms can be followed conservatively. The nutcracker syndrome, first reported in 1950, is said to occur when the nutcracker phenomenon is accompanied by clinical symptoms including chronic abdominal pain and haematuria. This disease can occur anytime during a persons life but the highest incidence of this disease can be found in adults in their 20s30s. The loin pain hematuria syndrome lphs was first described in 1967 in a report of three young women 20 to 28 years of age who had recurrent episodes of severe unilateral or bilateral loin flank pain that were accompanied by gross or microscopic hematuria. Pelvic congestion symptoms such as pelvic pain and dyuspareunia are also noticed.
It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. Term of nutcracker syndrome ncs is used for patients with clinical symptoms associated with nutcracker anatomy. The frequency and severity of the syndrome vary from asymptomatic microhematuria to severe pelvic congestion. It occasionally presents as a varicocele in boys or abnormal menstruation in pubertal girls as a result of the development of venous varicosities of the gonadal vein. Nutcracker syndrome, also called renal nutcracker syndrome is a rare vascular condition. 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 was suspected based on the combination of clinical examination, ultrasound, ct imaging, and mri imaging. The signs and symptoms of ncs are all derived from the outflow. The loin painhematuria syndrome lphs was first described in 1967 in a report of three young women 20 to 28 years of age who had recurrent episodes of severe unilateral or bilateral loin flank pain that were accompanied by gross or microscopic hematuria.
Delayed diagnosis and treatment of the underlying pathology might predispose to irreversible consequences and chronic kidney disease. 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. Ab renal vein compression syndromes are rare causes of hematuria and can be divided into anterior and posterior nutcracker syndrome. Since its description in 1972, the nutcracker syndrome or aortomesenteric left. Accurate education loin pain hematuria syndrome lphs.
I will also be going into detail about my next steps in my journey since i was. Normally considered a benign, temporary condition, hematuria may also indicate of a more serious condition if there are persistent symptoms that worsen. Renal parenchymal disease and abnormalities in the urinary tract were ruled out. The nutcracker syndrome ncs is a rare cause of haematuria. We finally went to mayo clinic and she was diagnosed there. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. There are various underlying pathologic causes, including renal or ureteric calculi or tumors, intrinsic kidney disease including glomerulonephritis, and loin pain hematuria syndrome. One patient presented with orthostatic proteinuria and 1 with pelvic congestion syndrome. Nutcracker syndrome and radiographic evaluation of loin. My main symptoms are left flank pain and unexplained hematuria.
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. Abdominal or flank pain that may radiate to the thigh and buttock. Varicocele in men almost always occurring on the left side. Pelvic congestion in women, or varicose veins in the lower abdomen that may cause pain and heaviness in the pelvis or genital area. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. If the left gonad gets emptied through the left renal vein it can too lead to testicular pain in men and quadrant pain in the lower.
Nutcracker syndrome servier phlebolymphologyservier. Nutcracker syndrome can be treated, and treatments depend on your age, anatomy, and symptoms. Gross haematuria in patients with nutcracker syndrome nefrologia. Multidetector ct of vascular compression syndromes in the. 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. It embraces an extended nonpathognomonic spectrum of symptoms that imply a difficult diagnosis. Loin pain hematuria syndrome genetic and rare diseases. Glomeruli are the blood cleaning units of the kidneys and are believed to be the source of the bleeding in lphs. Abhishek shah, b and dianbo cao b a department of internal medicine, chinajapan union hospital of jilin university, changchun, jilin province 021, china. In women, pelvic congestion and varicose veins in the lower abdomen can cause pain and heaviness in the genital area. I have found very little information on this syndrome. The compression causes renal vein hypertension, leading to hematuria which can lead to anemia and abdominal pain classically left flank or pelvic pain. Jun 22, 2017 the best treatment for each person with renal nutcracker syndrome ncs is a controversial topic.
In addition to ruling out other possible causes, the episodes loin pain and blood in the urine must persist or be. The major causes of flank pain and hematuria, such as nephrolithiasis and blood clot. Evaluation of asymptomatic microscopic hematuria in adults. Nutcracker syndrome is a rare cause of recurrent gross hematuria in. Two children who presented to us with recurrent gross hematuria were evaluated. Treatment for hematuria syndrome is usually centered on alleviating the secondary condition causing the symptoms. Jan 24, 2019 in this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome. I would like to find anyone that has been diagnosed with the nutcracker syndrome. When the condition shows no symptoms, its usually known as nutcracker phenomenon. In the presence of infection, such as a urinary tract infection uti, an antibiotic is generally given for several days, during which. Apr 20, 2020 hematuria syndrome is a condition in which there is blood in the urine. 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. Nutcracker syndrome encompasses classical symptoms of hematuria and flank pain resulting from the compression of the left renal vein between the aorta and the superior mesenteric artery. In some cases, nutcracker syndrome may not need treatment, especially for patients who are.
Symptoms blood in the urine hematuria which can occasionally cause anemia requiring blood transfusions. 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. In general, for people with mild hematuria or with mild and tolerable symptoms, conservative management is recommended. It refers impingement left renal vein lrv located between abdominal aorta and. Aug 25, 2016 however, the nutcracker syndrome may also be associated with an array of pelvic and lower extremity symptoms in the absence of hematuria. Ab renal vein compression syndromes are rare causes of hematuria and can be divided into. Presence of variable nonspecific symptoms and nonsignificant. Spontaneous resolution of hematuria in children with nutcracker syndrome has been reported following an increase in body mass index. Nutcracker syndrome in children presenting with recurrent. In patients with unexplained leftsided hematuria, flank pain or nonspecific abdominal pain, careful interrogation of diagnostic abdominal imaging should be performed to exclude the possibility of external.
The selection of the imaging modality is a stepwise process. Background nutcracker syndrome is an entity resulting from left renal vein compression by the aorta and the superior mesenteric artery, which leads to symptoms of hematuria or left flank pain. 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. Any significant increase in the degree of microscopic hematuria more than 50 red blood cells per highpower field, an episode of gross hematuria or the new onset of irritative voiding symptoms. In this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome. Common signs and symptoms when the condition shows no symptoms, its usually known.
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