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