The nutcracker syndrome (NCS) is defined by compression of the left renal vein (LVR) and may present with a wide variety of symptoms. Due to its rarity in pediatric patients, incidence, diagnostics and performed therapy regimen are widely undefined. To this date, there are only case reports and small collectives of pediatric patients described but comprehensive research is lacking.

A systematic literature research on pediatric NCS was carried out on Medline and Scopus databases according to PRISMA principles using predefined search terms and inclusion criteria. The PROSPERO registered review (CRD42021237415) identified patients' characteristics regarding age, sex, clinical symptoms, applied diagnostic methods and treatment options.

In total 47 articles were included. Overall, 423 children (218 boys and 205 girls) with diagnosed NCS were included in the analysis. Mean age was 12.0 (boys 12.9, girls 12.0) years. Hematuria was most common presentation (55.5%), followed by proteinuria (49.9%). Classical flank pain was only detected in 19.1% of patients. Sonographic evaluation was the most commonly used diagnostic tool (99%). Invasive diagnostic studies were performed in 97 children. 86.8% patients were treated conservatively and 94.9% showed complete resolution (42.8%) or at least improvement (52.2%) of symptoms. Type of operative treatment comprised of open surgery with transposition of LRV, endovascular stenting and laparoscopy.

Overall, data quality regarding NCS in children is poor. However, conservative approach in pediatric patients is recommended and should be regarded first treatment option. Diagnostic and treatment should follow a defined algorithm when NCS is suspected. Sufficient observation and follow-up must be assured in all patients to get significant results in this heterogenous syndrome.

V-IV.

At present, researchers have obtained fruitful results in the study of nutcracker syndrome (NCS), but there is still a lack of systematic research on the overall status of this disease. This article aims to describe the past and current status of research into NCS, and predict future research trends and popular research topics.

Using bibliometric and visualization methods, 552 articles related to NCS collected from the Scopus database from 1974 to 2021 were analyzed from multiple perspectives.

Overall, the amount of literature related to NCS is on the rise every year, and the number of citations is the turning point in 2006. The United States has the largest number of publications and has the most extensive cooperation with other countries. The main contents of the co-authored study focused on the symptoms, surgical procedures, and concomitant diseases of NCS. Keywords such as peak velocity, ultrasonography, orthostatic proteinuria, etc appeared earlier, whereas diagnosis, chronic pelvic pain, endovascular stents, etc appeared later.

The literature utilization rate of NCS is relatively insufficient. The pathogenesis and pathological mechanisms need to be further studied, and the diagnostic criteria and surgical methods will continue to be favored by clinicians.

Nutcracker syndrome (NS) refers to compression of the left renal vein between the aorta and the superior mesenteric artery which results in left renal venous hypertension. The typical clinical presenting feature is hematuria. In this report we describe the case of patient with a single kidney who developed severe proteinuria due to NS. She was successfully treated with left renal vein transposition. This case clearly shows the relation between NS and severe proteinuria based on normal biopsy findings and the complete disappearance of proteinuria following surgery.

The cause of orthostatic proteinuria is not clear but may often relate to obstruction of the left renal vein in the fork between the aorta and the superior mesenteric artery (=renal nutcracker). However, reports dealing with proteinuria only marginally refer to this possible cause of orthostatic proteinuria. We analysed the corresponding literature.

Five reports addressed the frequency of renal nutcracker in 229 subjects with orthostatic proteinuria. Their age ranged between 5.2 and 17 years (female-to-male ratio: 0.96:1.00). Imaging studies demonstrated renal nutcracker in 156 (68%) subjects. Renal nutcracker was also demonstrated in 9 anecdotal reports for a total of 53 subjects with postural proteinuria. Very recently, 13 Italian subjects with orthostatic proteinuria associated with renal nutcracker were reassessed 6 years after the initial diagnosis: in nine subjects, both orthostatic proteinuria and renal nutcracker had disappeared; in three, both orthostatic proteinuria and renal nutcracker had persisted; and in one, orthostatic proteinuria had persisted unassociated with renal nutcracker.

These data provide substantial support for renal nutcracker as a common cause of orthostatic proteinuria.

The use of multidetector computed tomography (MDCT) in routine abdominal explorations has increased the detection of the nutcracker phenomenon, defined as left renal vein (LRV) compression by adjacent anatomic structures. The embryology and anatomy of the nutcracker phenomenon are relevant as a background for the nutcracker syndrome, a rare cause of hematuria as well as other symptoms. MDCT examples of collateral renal vein circulation (gonadal, ureteric, azygous, lumbar, capsular) and aortomesenteric (anterior) and retroaortic (posterior) nutcracker phenomena in patients with no urologic complaint are shown as well as studies performed on patients with gross hematuria of uncertain origin. Incidental observation of collateral veins draining the LRV in abdominal MDCT explorations of asymptomatic patients may be a sign of a compensating nutcracker phenomenon. Imbalance between LRV compression and development of collateral circulation may lead to symptomatic nutcracker syndrome.

Nutcracker syndrome is caused by compression of left renal vein between the aorta and the superior mesenteric artery. This phenomenon results in left renal venous hypertension, left gonadal vein varices and unilateral hematuria. We report a typical case of nutcracker syndrome and we review the literature in an effort to explain this pathology.