Nutcracker syndrome (NCS) is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. The development of NCS is associated with the formation of the left renal vein (LRV) from the aortic collar during the sixth to eighth week of gestation and abnormal angulation of the superior mesenteric artery from the aorta. Collateralization of venous circulation is the most significant effect of NCS. It includes mainly the left gonadal vein and the communicating lumbar vein. Undiagnosed NCS may affect retroperitoneal surgery and other radiological and vascular procedures. The clinical symptoms of NCS may generally be described as renal presentation when symptoms like haematuria, left flank pain, and proteinuria occur, but urologic presentation is also possible. Radiological methods of confirming NCS include Doppler ultrasonography as a primary test, retrograde venography, which can measure the renocaval pressure gradient, computed tomography angiography, which is faster and less traumatic, intravascular ultrasound, and magnetic resonance angiography. Treatment can be conservative or surgical, depending on the severity of symptoms and degree of LRV occlusion. Nutcracker syndrome is worth considering especially in differential diagnosis of haematuria of unknown origin.

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What Each Clinical Anatomist Has to Know about Left Renal Vein Entrapment Syndrome (Nutcracker Syndrome): A Review of the Most Important Findings.

Introduction: Superior mesenteric artery syndrome is an uncommon cause of duodenal obstruction, and its manifestations are generally associated with compression on the third part of the duodenum between the abdominal aorta and superior mesenteric artery. Case Presentation: In this report, a patient is described presenting with epigastric pain and weight loss due to superior mesenteric artery syndrome. 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. Conclusions: In addition to an appropriate clinical history, CT findings indicating decreased aortomesenteric angle and a shortened aortomesenteric distance can suggest the diagnosis for both the superior mesenteric artery syndrome and accompanying nutcracker syndrome.

Superior Mesenteric Artery Syndrome Accompanying With Nutcracker Syndrome: A Case Report

Hematuria secondary to renal vein entrapment is mentioned only passing in textbooks and reviews. We performed a search of the National Library of Medicine database for peer-reviewed publications using the terms “renal vein” or “nutcracker” and “hematuria”. We identified 187 published reports/studies that covered 736 patients, of whom 288 had microscopic hematuria and 448 had macroscopic hematuria. The patient cohort comprised 159 patients aged ≤17 years. Abdominal pain was absent in  approximately 65 % of all patients, and a clinically relevant left-sided varicocele was observed in 29 % of the male patients. A normal pre-aortic left renal vein and an anomalous anatomy were noted in 680 and 56 patients, respectively. The body mass index (BMI) was lower in patients with renal vein entrapment than in the controls, with a regression of hematuria correlating with an increase in BMI. A surgical procedure was attempted in 34 % of the patients, of which the most common were endovascular stenting and transposition of the renal vein distally into the vena cava. In cases of unexplained hematuria with or without abdominal pain, clinicians should consider the diagnosis of renal vein congestion, especially in males with varicocele. Ultrasonic Doppler flow scanning is the recommended initial diagnostic modality in these patients. Expectation management is advised in the great majority of cases.

Micro- and macroscopic hematuria caused by renal vein entrapment: systematic review of the literature

Nutcracker syndrome (NCS) is a rare pathology, caused by compression of the left renal vein (LRV) between the abdominal aorta (AA) and the superior mesenteric artery (SMA), due to reduction of the angle between AA and SMA. This leads to LRV varices, left gonadal vein varices and therefore, the pelvic congestion syndrome. For this reason, coexistence of NCS and pelvic congestion syndrome has been described. It manifests by hematuria, proteinuria, and nonspecific pelvic pain secondary to pelvic congestion, dyspareunia and persistent genital arousal. We report a 27-year-old woman who experienced hematuria and left flank pain. The diagnosis of NCS accompanied by pelvic congestion syndrome was missed initially, but later on the diagnosis was made by color Doppler ultrasound, abdominal computed tomography (CT) and CT angiography that were later performed. She refused interventional and surgical treatments, and was lost to follow up.

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Nutcracker Syndrome Accompanying Pelvic Congestion Syndrome; Color Doppler Sonography and Multislice CT Findings: A Case Report

Bartter syndrome is a hereditary disorder that has been characterized by the association of hypokalemia, alkalosis, and the hypertrophy of the juxtaglomerular complex with secondary hyperaldosteronism and normal blood pressure. By contrast, the genetic causes of Bartter syndrome primarily affect molecular structures directly involved in the sodium reabsorption at the level of the Henle loop. The ensuing urinary sodium wasting and chronic sodium depletion are responsible for the contraction of the extracellular volume, the activation of the renin-aldosterone axis, the secretion of prostaglandins, and the biological adaptations of downstream tubular segments, meaning the distal convoluted tubule and the collecting duct. These secondary biological adaptations lead to hypokalemia and alkalosis, illustrating a close integration of the solutes regulation in the tubular structures.

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Primary molecular disorders and secondary biological adaptations in bartter syndrome.

Nutcracker syndrome (NCS) is an uncommon cause of hematuria. It refers to the compression of the left renal vein between the aorta and superior mesenteric artery. It can cause both microscopic and gross hematuria. Hematuria may be associated with left flank pain. The diagnosis of NCS is often delayed. Most patients may have symptoms for many years and non-diagnostic evaluations before accurate diagnosis can be established. It should be included in the differential diagnosis of unexplained hematuria. The diagnosis and treatment of nutcracker syndrome are discussed. Conclusion: NCS is a rare but treatable clinical condition. 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.

The nutcracker syndrome: an underrecognized cause of hematuria

Failure to thrive in an infant has multiple etiologies and at times, is only manifestation of underlying serious disease. Bartter syndrome is a rare disease that manifests as failure to thrive. It can be diagnosed by a careful history, physical examination, and abnormal electrolyte pattern. It can be alleviated by appropriate management, whereas failure to recognize early can be life threatening. A case of severe failure to thrive in an infant due to neonatal form of Bartter syndrome, its manifestations, management, and outcome is presented in this article.

Severe Failure to Thrive in Infant

When alveolar hemorrhage occurs in association with glomerulonephritis, one of the leading causes is Goodpasture syndrome. We report a case of an adolescent who presented to the emergency department in respiratory distress. Subsequent evaluation was consistent with the diagnosis of Goodpasture syndrome.

Gerard Prosper

Papers

The nutcracker syndrome: an underrecognized cause of hematuria

Severe Failure to Thrive in Infant

An asthmatic adolescent with hematuria and hemoptysis.