Nutcracker esophagus, or hypertensive peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or duration. Several anatomical anomalies can result in deep vein thrombosis dvt. Duplex ultrasound investigation in pelvic congestion. Sonographic evidence of nutcracker phenomenon, an incidental finding in a patient. 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. Nafld is defined by evidence of fatty liver without another factor that could explain the liver fat accumulation, such as excessive alcohol use 21 standard drinksweek for men and 14 for women in the usa. Nutcracker syndrome is a vascular compression disorder, and. Doppler ultrasound measurements of the anteriorposterior ap diameter and peak velocities of the left renal vein may be helpful in diagnosing nutcracker syndrome. Fatty liver nash vascular buddchiari syndrome hepatic. An abdominal ultrasonogram usg showed fatty liver and sand like left renal stones. Nutcracker syndrome nutcracker syndrome occurs when the left renal vein is. Doppler us and ct diagnosis of nutcracker syndrome ncbi. 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. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain.
Doppler sonographic diagnostic criteria were applied, 72 cases 33. I just want to let everyone know that i have vericose veins in my uterus. Nutcracker syndrome associated with celiacomesentric trunk. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong.
Nutcracker syndrome can be difficult to diagnose because its symptoms are similar to many other urological and gynecological disorders. Prevalence of nonalcoholic fatty liver disease and risk factors for advanced fibrosis and mortality in the united states. 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. Nutcracker syndrome associated with celiacomesentric trunk anomaly.
Nutcracker syndrome had been confirmed by conventional venography until recent years. Request pdf nutcracker syndrome left renal vein entrapment syndrome, also known as nutcracker syndrome, involves compression of the left renal vein and can present with. N2 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. Criteria for defining significant central vein stenosis with duplex ultrasound. Diagnosis and surgical treatment of nutcracker syndrome.
Thus, nutcracker syndrome was the most common cause of isolated hematuria in children without urinary tract. The initial imaging test for nutcracker syndrome is ultrasound, which is used in. Nutcracker syndrome ncs refers to the clinical manifestations of ncp. In addition, vascular imaging studies such as ultrasound and contrast. Once other conditions have been ruled out, your vascular surgeon may recommend imaging tests, like an ultrasound, ct scan, or mri to screen for nutcracker syndrome. The usefulness of abdominal ultrasound for the evaluation. Learn vocabulary, terms, and more with flashcards, games, and other study tools.
It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. 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. 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. To assess diagnosis of the nutcracker renal vein entrapment syndrome with doppler ultrasonography us.
New procedure offers speedier recovery for patients with nutcracker syndrome. Aug 04, 2011 i also was told, wo blood in urine not a problem, although my nephro at home disagrees. The sensitivity and specificity of color doppler sonography for revealing the nutcracker syndrome were 78% and 100%, respectively, when color flow in collateral veins was included in the diagnostic criteria. The diagnosis of the nutcracker syndrome was suspected based on clinical examination, doppler scan, duplex ultrasound scan, computed tomography scan, and magnetic resonance imaging. Nutcracker syndrome ncs is the set of clinical manifestations of venostasis induced by left renal vein lrv compression nutcracker phenomenon between the superior mesenteric artery sma and aorta anterior ncs or between the aorta and spine when the lrv is retroaortic posterior ncs syndrome.
Svensson christina, department of medicine and health science. Nutcracker syndrome ncs describes left renal vein compression between the superior mesenteric artery and the aorta. Clinical and radiological features in four adolescents with. An unusual course of segmental renal artery displays a rare. Nutcracker syndrome is caused by the narrowing in the anatomical passage formed by the aorta and to the superior mesenteric artery, causing increased pressure in the territory of the. Examination in long axis using color doppler will reveal the presence of a velocity gradient from the perihilar to mesoaortic region 810. However, when the left rein vein is routinely examined by ultrasound andor duplex, the nutcracker phenomenon is quite frequently seen and. The following report examines two case studies where a diagnosis of ncp was made from clinical history and ultrasound findings. 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.
There are various underlying pathologic causes, including renal or ureteric calculi or tumors, intrinsic kidney disease including glomerulonephritis, and loin pain hematuria syndrome. Left renal vein entrapment syndrome, also known as nutcracker syndrome, involves compression of the left renal vein and can present with abdominopelvic pain. Ct scan of abdomen showed left renal vein duplication. Nutcracker syndrome complicated by left renal vein thrombosis. Ultrasound imaging can often demonstrate the compressed renal vein and allow for measurement of the vein diameter and stenosis secondary to the compression fig. Individuals with ncs may also experience pelvic congestion causing various pelvic symptoms.
The ct findings are consistent with nutcracker syndrome. An ultrasound scan at the level of the umbilical region showed that blood flow in the left ivc was weak. Left renal vein entrapment syndrome, also known as nutcracker syndrome, involves compression of the left renal vein and can present with abdominopelvic pain, hematuria, and symptoms of pelvic congestion syndrome. 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. Which specialists are essential to meet, what tests are needed and other useful information for the diagnosis of nutcracker syndrome. 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. Posterior nutcracker syndrome with left renal vein duplication. Diagnosis of the nutcracker syndrome with color doppler. The presence of increased flow within collateral vessels is further evidence of renal. For the diagnosis of ncp, several diagnostic tools are applied, but definite diagnostic criteria have not yet been established. She had surgery, which is left renal vein transposition, where. Since some of these causes are potentially serious and may be treatable if identified early, it is important to establish a.
Current trends in the diagnosis and management of renal. In certain situations, the syndrome can result from a retroaortic or circumaortic left renal vein nutcracker syndrome should not be confused with superior mesenteric artery syndrome wilkie syndrome also a superior mesenteric artery compression disorder, where the sma compresses the third part of the duodenum the two conditions, however, may be associated. Common signs and symptoms when the condition shows no. A rare condition caused by arterial compression of the left renal vein between the superior mesenteric artery and the aorta. Jul 14, 2017 nutcracker syndrome is one of the many unfortunate consequences of our bipedal lifestyle. I had an ultrasound to check the flow in the kidney and they decided that indeed the ct scan and the ultrasound showed impingement in that area.
Imaging findings and clinical features of abdominal. Jun 14, 2014 the potential role of ultrasound by the clinician is increasing. Following are the common symptoms that have been studied in the nutcracker syndrome. The estimated prevalence of nonalcoholic fatty liver disease nafld is approximately 34%. Although uncommon, it is an important diagnosis due to the important morbidity associated with it, including the risk of chronic kidney disease from longterm left renal vein lrv hypertension and the risk of lrv thrombosis.
Nutcracker syndrome is a vascular compression disorder, and refers. 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. In this article, we aimed to illustrate imaging findings that are characteristic of. Feb 28, 2018 nutcracker syndrome and loin pain hematuria syndrome duration. Flow may be reduced in gastroduodenal artery and common hepatic artery. My four year old just had an abdominal ultrasound and the results were that she has nutcracker syndrome. An update on current diagnostic criteria and management guidelines. These include phase contrast microscopy, abdominal ultrasound and intravenous urography, coagulation profiles, tests for urinary bilharziasis, cystoscopic localisation of the haematuria, selective urine cytology. Common method of screening diagnostic of nafld remained abdominal ultrasound, despite his. The purpose of this study was to evaluate late results of lrv transposition and identify risk factors affecting outcomes. Nutcracker phenomenon and nutcracker syndrome mayo clinic. In most cases, compression of the left renal vein is between the abdominal aorta the main artery in the abdomen and the superior mesenteric artery, which brings blood.
The patient stated that he did not feel an ache in his scrotum and that his blood pressure was normal. Fatty liver fl is commonly associated with metabolic syndrome diabetes. So we are currently waiting to get a second opinion. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Ultrasound showing diffuse increased echogenicity of the liver. This article describes the typical imaging findings and clinical features that are associated with four abdominal vascular compression syndromes. Oct 19, 2006 in the present study, we could not identify the cause of hematuria in 149 69% of the 216 patients when renal doppler ultrasound was not performed, but 40 percent was found to have nutcracker syndrome by renal doppler ultrasound. The diagnosis of steatosis is made when fat in the liver exceeds 510 % by weight.
In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. Its main abdominal branches are the celiac trunk, superior mesenteric artery, bilateral renal arteries and inferior mesenteric artery. The vascular surgeon spoke with me and said that since i have no blood in my urine, i dont have an exciting case of this nutcracker syndrome. Nutcracker syndrome is a variation of nutcracker phenomenon or renal vein entrapment syndrome, in which the arteries near the kidney compress the left renal kidney vein. Maythurner syndrome also known as iliac vein compression syndrome is one of them. 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. Superior mesenteric artery sma syndrome is a gastrovascular disorder in which the third and. Association between noninvasive fibrosis markers and mortality among adults with nonalcoholic fatty liver disease in the united. We report a case of nutcracker syndrome diagnosed with 3dimensional computed tomography angiography 3d cta. One patient presented with orthostatic proteinuria and 1 with pelvic congestion syndrome.
Effect of renal doppler ultrasound on the detection of. Terms in this set 69 nutcracker syndrome is the result of extrinsic compression of what. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. Clinician decision depends radiologist to diagnose and staging this disease. Radiology imaging, medical imaging, superior mesenteric artery syndrome. Renal nutcracker syndrome genetic and rare diseases.
It has been referred to by a variety of other names, including cast syndrome, wilkie syndrome, arteriomesenteric duodenal obstruction, and chronic duodenal ileus. Area of focal fatty sparing in a fatty liver fatty liver, ultrasound, anatomy. Nonalcoholic fatty liver disease on the web most recent articles. Nutcracker syndrome and radiographic evaluation of loin. Strongly associated with metabolic syndrome, the incidence of nafld will continue to rise with the projected progression of the obesity epidemic1,2. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. We have went to a urologist and he brushed us off as though her pain was strictly constipation.
Ncs refers to a syndrome caused by significant compression of lrv between the abdominal aorta and sma, resulting in hypertension of the lrv and hematuria due to rupture of the thinwalled veins close to the calyceal fornices 10,11. Shin ji, park jm, lee js, kim mj 2007 effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria. However, a narrow sma angle alone is not enough to make a diagnosis, because. Occasionally there may be tiredness or pain in the upper right side of the abdomen. 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.
A case report of a young girl with recurrent hematuria. Diagnosis of the nutcracker syndrome with color doppler sonography. My daughter has nutcracker syndrome testicular disorders. The nutcracker syndrome, once considered to be a rare entity, has recently been shown to be diagnosed increasingly among patients with hematuria or proteinuria. Several treatment options have been described to relieve associated symptoms. Hematuria was the most common presenting symptom 21 of 23 patients. Nowadays, with the development of imaging techniques, color doppler sonogram and 3d cta are replacing venography for the diagnosis of nutcracker. Aims this paper aims to provide education and ultrasound protocol for clinicians and sonographers who encounter patients with the symptoms of ncs during their course of practice. The syndrome is characterized by compression of the third portion of the duodenum due to narrowing of the space. The nutcracker syndrome can exist in either nondistended or distended left renal veins.
Left renal vein transposition for nutcracker syndrome. We have analyzed the most five used ultrasound criteria and liver lobes diameters from 2 patients with nafld. 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. Loin pain and hematuria are common symptoms, presenting in patients of all ages. Nutcracker syndrome diagnosed with 3dimensional computed. Because of the variability of symptoms and absence of consensus on diagnostic criteria, the exact prevalence of ncp is unknown but may be slightly higher in females. However, the exact prevalence rate of nutcracker syndrome cannot be formulated due to variability in symptoms and lack of standardized criteria for diagnosing nutcracker syndrome 3, 4.
Some people may not have symptoms, while others develop severe and. Feb 23, 20 nutcracker syndrome is a vascular compression disorder, and refers to the compression of the left renal vein between the superior mesenteric artery sma and aorta. Ultrasonography, computed tomography, and magnetic resonance imaging revealed entrapment of the lrv. Fitoz s, ekim m, ozcakar zb, elhan ah, yalcinkaya f, et al. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis. Cgsurg106 venous angioplasty with or without stent.
Fatty liver disease fld, also known as hepatic steatosis, is a condition where excess fat. Nutcracker syndrome ncs, also known as left renal vein lrv entrapment syndrome, was first defined anatomically by grant in 1937 as follows. Nutcracker syndrome ncs is a syndrome caused by compression of the left. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal doppler ultrasound as a screening examination has a substantial effect on the detection of nutcracker syndrome. Ncs typically causes intermittent gross hematuria but may be accompanied by microscopic hematuria, proteinuria, or left flank pain, without.
Current trends in the diagnosis and management of renal nutcracker syndrome. Effect of renal doppler ultrasound on the detection of nutcracker syndrome in children with hematuria 19 october 2006 european journal of pediatrics, vol. Unusual coexistence of double inferior vena cava with. We explain the underlying pathophysiology that results in these clinical syndromes so that the patient subset who will benefit from treatment can be identified. Nutcracker syndrome got its name because the compression of the renal vein is like a nutcracker cracking a nut. Doppler us findings in 16 patients with the nutcracker syndrome and in 18 healthy control subjects were compared. Multidetector ct of vascular compression syndromes in the. Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Complications may include cirrhosis, liver cancer, and esophageal varices there are two types of fatty liver disease nonalcoholic fatty liver disease nafld. The aorta is the largest artery in the human body, it begins at heart and branches out to supply oxygenated blood to all the viscera and extremities. Nutcracker esophagus is one of several motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. See more ideas about superior mesenteric artery, pelvic congestion syndrome and superior mesenteric artery syndrome.
Nutcracker syndrome is a rare disease entity that is caused by entrapment of the left renal vein between the aorta and superior. Diagnosis and treatment of the nutcracker syndrome. Fatty liver disease fld, also known as hepatic steatosis, is a condition where excess fat builds up in the liver. In the 72 children with nutcracker syndrome, the pv2pv1 ratios were 6.
Symptoms include blood in the urine along with flank and abdominal pain. All 23 patients met the criteria for establishing the diagnosis of the nutcracker syndrome. Nutcracker syndrome was suspected based on the combination of clinical examination, ultrasound, ct imaging, and mri imaging. 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. In this article, we refer to this heterogeneous group of. Articles from korean journal of radiology are provided here. Anticoagulation was started and three weeks later, cta showed complete disappearance of the renal. Nutcracker syndrome ncs is a rare condition characterized by entrapment of the left renal vein. A group of symptoms that occur often as a result of cancer when the superior vena cava. Nov 24, 20 isolated renal vein thrombosis is a rare entity. Common method of screening diagnostic of nafld remained abdominal ultrasound, despite his modest sensitivity and specificity. Superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction.
The widely used diagnostic method of ncp is renal venography. In 52 children with microscopic haematuria, onethird fulfilled the ultrasonographic criteria for nutcracker phenomenon. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks. The nutcracker syndrome is said to be an underdiagnosed cause of haematuria. Sonographic evidence of nutcracker phenomenon, an incidental finding in a patient referred to investigate cholecystitis. Bedside ultrasound in the diagnosis of nonalcoholic fatty. The small intestines hang like baggy sausages off the branched stems of the superior mesenteric artery sma, and in some individuals, the sma compresses the left renal vein against the aorta. Nutcracker syndrome is an easily missed cause of hematuria in children. Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric. The most common ultrasound criteria used for evaluating pcs in the literature are.