The patient has also nutcracker syndrome, which is the compression of the left renal vein between the aorta and the superior mesenteric artery at its origin. It should be included in the differential diagnosis of unexplained hematuria. It should be considered in the differential diagnosis of chronic fatigue syndrome associated with abdominal pain. The nutcracker syndrome ns is caused by the compression of the left renal vein lrv, due to a reduced angle between the superior mesenteric artery sma and the aorta. Maythurner syndrome is one underlying cause of a common condition. Therefore, it seems to be wise not to forget the differential diagnosis if a dilated left renal vein is seen in a patient with haematuria or orthostatic. It is associated with the formation of the left renal vein from the aortic collar during the 6th8th week of gestation and abnormal angulation of the sma from the aorta. To confirm a diagnosis of this disease, patients will need to have imaging and laboratory studies in order to solidify the diagnosis. The posterior nutcracker syndrome is very rare and is considered when the left renal vein is compressed between the aorta and vertebral column. 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 syndrome presents with hematuria blood in the urine and flank a region of the lower back beneath the ribs and above the ilium pain which can result from a number of causes. We then performed a series of diagnostic left t9 paravertebral blocks to determine her. 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. Nutcracker syndrome develops when lrv is compressed between the aorta and sma.
Nutcracker syndrome ncs is a rare vascular compression disorder that involves compression of the left renal vein most commonly between the aorta and the superior mesenteric artery sma, although variations exist. Maythurner syndrome, also known as iliac vein compression syndrome or cocketts syndrome, affects two blood vessels that go to your legs. Of these, nutcracker syndrome was diagnosed in 40 percent after a renal ultrasound was done. Superior mesenteric artery syndrome accompanying with. 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. Differential diagnosis of hematuria from the left kidney should include the nutcracker syndrome.
In medicine, the nutcracker syndrome also known as nutcracker phenomenon, renal vein entrapment syndrome, or mesoaortic compression of the left renal vein is a compression of the left renal vein between the abdominal aorta aa and superior mesenteric artery sma. In patients with new daily persistent headache and orthostatic symptoms one may consider a differential diagnosis of nutcracker syndrome, especially in patients with comorbid hypermobility syndromes, hematuria or gastric symptoms. Renal nutcracker syndrome symptoms, diagnosis, treatments and. In addition to an appropriate clinical history, ct findings indicating decreased aortomesenteric angle and a shortened aortomesenteric distance can suggest the diagnosis for. The diagnosis and treatment of nutcracker syndrome are discussed. With anterior nutcracker syndrome, the left renal vein is compressed between the aorta and the superior mesenteric artery. Diagnosing nutcracker syndrome and pelvic congestion syndrome. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on nutcracker syndrome.
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. There are established criteria for the diagnosis of the entrapment of the left renal vein by twodimensional ultrasonography and. When an os trigonum is present, this accessory ossicle together with surrounding soft tissues can become wedged between the tibia, talus and calcaneus. Diagnosis and successful surgical management of posterior. Though lrv transposition and now lrv stenting are commonly employed. Purpose the purpose of this case study is to highlight the symptoms of the nutcracker syndrome ncs, the methods of clinical investigations and the importance of differential diagnosis. An inclusion of this entity in the differential diagnosis of patients with unknown cause of hematuria and flank pain can achieve efficient management of this condition.
Nutcracker syndrome symptoms and diagnosis nutcracker syndrome symptoms. The ct findings are consistent with nutcracker syndrome. Although under diagnosed, there are several treatment options available ranging from simple surveillance to surgical resolution. Wilkie syndrome and nutcracker syndrome are rare compression syndromes. However, an incidental finding of nutcracker phenomenon syndrome was made. Nutcracker syndrome can be hard for doctors to diagnose, but once its diagnosed, the outlook is often good. Nutcracker syndrome gary sharp 1, and derek glenn 2 1 trauma department at st george hospital, kogarah, sydney, nsw 2217, australia. Oct 31, 2012 nutcracker syndrome differential diagnosis. Although it can be invasive and uncomfortable for the patient, renal venography combined with measurement of the pressure gradient between the left renal vein and the ivc is the gold standard for demonstrating renal vein hypertension.
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. 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 intestine is a long, winding tube that connects the stomach to the anus. This was observed primarily in children, 10, 33 34 and reports among adults followed. Other etiologic hypotheses of nutcracker syndrome include posterior renal ptosis with resultant stretching of the left renal vein over the aorta, and abnormal branching of the superior mesenteric artery from the aorta. Ct scan and mri revealed a retroaortic left renal vein. Symptoms include blood in the urine along with flank and abdominal pain. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared. Undetected and untreated ncs may lead to the development of lrv thrombosis. Symptoms of nutcracker syndromes may include intermittent left flank pain associated with hematuria, proteinuria, and sometimes with symptoms of pelvic congestion.
For many patients, nutcracker syndrome is incidentally discovered on. Diagnosis of nutcracker syndrome was suspected by doppler ultrasonography and then confirmed by magnetic resonance angiography performed with administration of bloodpool paramagnetic contrast medium. A differential diagnosis of haematuria following a motor. Maythurner syndrome mts is an anatomically and pathologically variable condition leading to venous outflow obstruction as a result of extrinsic venous compression in the iliocaval venous territory. Symptoms usually dont become obvious until the third or fourth decade. The nutcracker syndrome left renal vein entrapment, demonstrated by ultrasonic imaging and doppler flow scanning renal manifestations of sickle cell disease view in chinese hematuria has been attributed to the greater length of the left renal vein and the nutcracker phenomenon, in which compression of the left renal vein between. Nutcracker syndrome ncs is a rare condition characterized by entrapment of the left renal vein. The term syndrome refers to a group of signs and symptoms primarily occurring together, characteristic of a specific condition. Differential diagnosis for renal vein thrombosis includes renal malignancy, nephrotic syndrome, hypovolaemia, extrinsic compression of renal vein, hypercoagulable conditions of the blood, oral contraceptive use and myeloproliferative disorders.
Renal vein thrombosis is one the more serious complications. Case report a differential diagnosis of haematuria. After investigation no trauma related injuries were identified. This anatomic finding has been shown to be present in more than 20% of the population. Left renal phlebography in a patient with the nutcracker syndrome showing. I just want to let everyone know that i have vericose veins in my uterus. Other common presenting symptoms include proteinuria, gonadal vein syndrome, and varicocele due to development of collateral vessels. It could make you more likely to have a dvt deep vein. Nutcracker syndrome symptoms, treatment, causes, surgery. The clinical symptoms associated with this condition are nonspecific in most of the cases, therefore.
However, an incidental finding of nutcracker phenomenonsyndrome was made. Some people may not have symptoms, while others develop severe and. The diagnosis and treatment of wilkie syndrome is controversial. When symptoms starts showing up, treatments should be started immediately. Nutcracker syndrome garysharp 1 andderekglenn 2 trauma department at st george hospital, kogarah, sydney, nsw, australia st george hospital, kogarah, sydney, nsw, australia correspondence should be addressed to gary sharp. Nutcracker syndrome has many symptoms for both adults and children, although some especially children dont have any symptoms. Nutcracker syndrome ncs was first described by grant, who likened the impingement of the left renal vein lrv by the superior mesenteric artery sma against the aorta to that of a nut within the jaws of a nutcracker. Due to the varieties in symptoms, the diagnosis of. With partial venous obstruction, the condition can be asymptomatic, but progression with symptoms related to chronic venous hypertension or. A rare condition where the left renal vein becomes compressed between the abdominal aorta and the superior mesenteric artery. The popular name nutcracker syndrome derives from the fact that, in the sagittal view, the sma and aa with some. To assess diagnosis of the nutcracker renal vein entrapment syndrome with doppler ultrasonography us. The os trigonum syndrome refers to pain posterior of the ankle and reduced plantarflexion caused by the nutcrackerphenomenon. 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.
The first clinical case of ncs was acknowledged by elsadr and mina in 1950 and its management was first documented in 1974. 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 syndrome doppler ultrasound diagnosis eurorad. Posterior nutcracker syndrome was considered the most likely diagnosis. The differential diagnosis of the disease involves telling its symptoms apart from those of.
Nutcracker phenomenon is a rare cause of haematuria resulting from nontraumatic compression of the. Superior mesenteric artery syndrome is caused when the third part of the duodenum is trapped or compressed between the two arteries the aorta and the superior mesenteric artery. Nutcracker syndrome refers to the compression of the left renal vein. In this article, we refer to this heterogeneous group of. Shin ji, lee js 2005 nutcracker phenomenon or nutcracker syndrome.
Differential diagnosis for renal vein thrombosis includes renal malignancy, nephrotic. Nutcracker syndrome wikimili, the free encyclopedia. Patient with both wilkie syndrome and nutcracker syndrome. A differential diagnosis of haematuria following a motor vehicle. The syndrome of loin pain hematuria in the absence of stones is poorly understood but must be considered in the differential diagnosis for patients with clinical manifestations resembling nephrolithiasis.
Other symptoms of the nutcracker syndrome may include varicose veins in the legs, severe menstrual cramping in women, lack of energy, painful urination and vulvae varicose veins. 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. 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. Differential diagnosis for renal vein thrombosis includes renal malignancy, nephrotic syndrome, hypovolaemia, extrinsic. Diagnostic tests include urine analysis, ultrasound scan, color doppler. Left renal venous hypertension from extrinsic left renal vein compression should be considered in the differential diagnosis of hematuria, especially in cases with involvement of the left kidney. Normally, the angle between the sma and aa is approximately 450. Intravascular ultrasound imaging confirmed the diagnosis. Nutcracker syndrome mimicking new daily persistent. 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. Nutcracker syndrome is a rare condition, which if left untreated may cause damage to the left kidney. Renal nutcracker syndrome symptoms, diagnosis, treatments. For many patients, nutcracker syndrome is incidentally discovered on imaging done for another cause. Some less common symptoms include varicoceles, orthostatic proteinuria and dilated gonadal veins.
The nutcracker syndrome in a venous or even broader scope, vascular compression syndromes is based upon decreased space between the superior mesenteric artery and the aorta in the area of the left renal vein. Controversy exists about obtaining a reliable diagnosis of nutcracker syndrome. Pain management specialists should include nutcracker syndrome in the differential when the symptoms indicate, and not assume. Kurklinsky ak, rooke tw 2010 nutcracker phenomenon and nutcracker syndrome. The most common symptoms of nutcracker syndrome can include. However, it is rarely considered in the differential diagnosis of leg edema, dvt, and chronic venous disease, particularly in patients with other risk factors. A 22yearold white female with a 4year history of left flank pain and hematuria underwent an extensive workup with normal renal ultrasound and cystourethroscopies. However, it is rarely considered in the differential diagnosis of leg edema, dvt, and chronic venous disease, particularly in. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and. Left renal phlebography in a patient with the nutcracker syndrome showing a delay in drainage of dye from the renal vein.
Nutcracker syndrome should be part of the differential consideration in. This anatomical finding has been known to anatomists for over 70 years 1 and is called the nutcracker phenomenon 2. A moderate degree of protenuria was incidentally found in a 16 year old male during some check up tests. 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. Individuals with ncs may also experience pelvic congestion causing various pelvic symptoms. Diagnosis of the nutcracker syndrome depends on a pattern of pathological findings. Diagnosis should be suspected upon presentation with leftsided loin pain and hematuria. I have had pain since i was a child, i am now 29 and after years of searching, going from doc to doc, i finally found out that i had something called nutcracker syndrome which is a clinically manifest variant of nutcracker phenomenon, renal vein entrapment syndrome, or mesoaortic compression of the left renal vein. A differential diagnosis of haematuria following a motor vehicle collision. The initial management should include gastric andduodenal decompression, treatment of fluid and electrolyte imbalance as well as reversal of weight loss using enteral and parenteral feeding. Mri is a noninvasive diagnostic image modality that should be used before phlebography and pressure measurements in the left renal vein are performed.
691 159 684 70 317 1249 1418 696 1113 433 987 1569 1201 1559 1467 705 432 924 526 1176 422 1026 678 432 112 1029 167 159 977 890 735 1108 862