Nutcracker syndrome in pediatrics: initial findings and long-term follow-up results.

BACKGROUND: Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein, we present our single-center experience with a large number of patients who have long-term follow-up results. METHODS: All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were obtained retrospectively. RESULTS: A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10-14) years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria (16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination. All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%) within 35.8 ± 25.8 months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3 months. The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation. CONCLUSIONS: In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents. Our results support conservative management in children as the first-line treatment approach.

Left renal vein entrapment in postural proteinuria: the diagnostic utility of the aortomesenteric angle.

Nutcracker syndrome related to the left kidney vein compression is a cause of orthostatic proteinuria during childhood. Some studies have shown that the ratios between maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions must be more than 4 in order to make a Nutcracker syndrome diagnosis. Our aim was to investigate whether the use of a decrease in aortomesenteric angle between upright and supine positions in the presence of isolated orthostatic proteinuria can be a criterion for the diagnosis of Nutcracker syndrome. Relevant patient information, which included demographic data, clinical examination findings, laboratory data, urinary system ultrasound, and kidney color flow Doppler ultrasound results, were prospectively collected. Thirty-nine pediatric patients with orthostatic proteinuria were included in the study. Left kidney vein compression findings were demonstrated in 31 patients. The ratio of maximum velocities of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions was above 4 in only 7 of our patients. Ratio of aortomesenteric angle between upright and supine positions was significantly decreased for patients with left kidney vein compression findings.  Conclusion: The use of a decrease in the ratio of aortomesenteric angle between upright and supine positions in the presence of orthostatic proteinuria, instead of the ratios for maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments, can be more helpful for the diagnosis of Nutcracker syndrome in the differential diagnosis of orthostatic proteinuria. What is Known: • Proteinuria may be a sign of an impending kidney disease • Nutcracker syndrome is a cause of orthostatic proteinuria. What is New: • Ratio of aortomesenteric angle between upright and supine positions > 0.6 can be used for Nutcracker syndrome diagnosis.

Nutcracker syndrome mimicking new daily persistent headache: A case report.

INTRODUCTION: Compression of the duodenum and left renal vein between the aorta and superior mesenteric artery usually leads to symptoms of proximal bowel obstruction or hematuria and, more rarely, nonspecific mild headaches. CASE: A young woman presented with new daily persistent headache refractory to numerous pharmacological treatments, onabotulinumtoxinA, nerve blocks, and occipital nerve stimulation. Following several years of daily severe headache, worsening abdominal pain and intolerance for food intake led to the discovery of aortomesenteric compression. Surgical treatment gave prompt improvement in gastric symptoms but also essentially resolved the headache. CONCLUSION: This is the first description of new daily persistent headache in association with aortomesenteric compression as well as marked improvement of headache following aortomesenteric decompression. 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.

Posterior nutcracker syndrome - a systematic review.

Posterior nutcracker syndrome (PNCS) is the entrapment of the left renal vein between the aorta and the vertebral column. Although uncommon, it is still an important diagnosis due to the high morbidity associated with the risk of secondary anaemia from haematuria, from long-term left renal vein hypertension, vascular thrombosis, and even blood clots in the urinary system. A literature search of PubMed and EMBASE databases was performed and 27 publications containing 27 cases were included for the final analysis. The following frequency of clinical signs and symptoms was noted: twenty-five patients had haematuria, 13 patients had flank pain, and two had hypertension. Overall, male-female distribution was balanced and there were more adult than paediatric (age < 18 years) patients. All symptoms of patients with conservative treatment were either well-controlled or under spontaneous resolution. Conservative management instead of surgical treatment should be preferred in most cases. Taken together, despite the low incidence of PNCS, its recognition and management are highly important. This systematic study explores the evidence base for conservative and medical options.

Laparoscopic Left Renal Vein Transposition for Nutcracker Syndrome.

The left renal vein (LRV) reimplantation into the distal inferior vena cava is considered to be the gold standard of care for symptomatic nutcracker syndrome (NCS). The vast majority of these surgical procedures are performed by open surgery. Experiences with minimally invasive laparoscopic surgery in this field are very limited. We present a case of a 17-year-old boy with NCS in whom the transposition of the LRV was done laparoscopically. The patient suffered from left flank pain, painful left-sided varicocele, microscopic hematuria, proteinuria, and oligoasthenospermia. There were no intraoperative complications, and the postoperative course was uneventful. At 12-month follow-up, hematuria, left flank pain, and left testicular pain resolved. Duplex ultrasonography revealed patent LRV. Laparoscopic LRV transposition appears to be safe, feasible, and has favorable postoperative course.

The utility of bioimpedance analysis for monitoring the children with nutcracker syndrome.

UNLABELLED: We aimed to evaluate anthropometric and bioimpedance analysis (BIA) indices of children with nutcracker syndrome (NCS). Eighteen patients and 20 age-gender matched controls were enrolled. Weight, height, body mass index (BMI), mid-arm circumference (MAC), waist circumference (WC), waist/hip ratio measurements, and BIA results were assessed. Laboratory parameters and Doppler sonographic findings were recorded. The weight, BMI, MAC, WC, body fat z scores, and waist/hip ratio were significantly lower in patients than in controls (p < 0.05). Serum creatinine (Scr), albumin, HDL cholesterol, and urine protein (Up) were significantly increased in NCS patients compared to controls (p < 0.05). On multivariate analysis, body fat z score was independently related to Up and the degree of superior mesenteric artery (SMA) angle (beta = -0.965, p = 0.018 and beta = 0.841, p = 0.04, respectively). CONCLUSION: NCS might be considered in slim proteinuric patients with unproven etiology. Serial anthropometric measurements and BIA analysis would estimate the severity of entrapment in LRV. Long-term follow-up of Scr might be required in NCS. WHAT IS KNOWN: • Nutcracker syndrome (NCS) is very rare and characterized by the compression of LRV between aorta and SMA. What is new: • Patients with NCS have a slimmer body feature with lower anthropometric and BIA indices. • Fat percentage indicates the degree of LRV entrapment and amount of proteinuria in NCS.

Laparoscopic inferior mesenteric to gonadal vein end-to-side bypass: a new attempt for nutcracker syndrome treatment.

The nutcracker syndrome (NCS) is because of the compression of the left renal vein when it passes between the aorta and the superior mesenteric artery. The treatment of NCS is controversial, and conservative, endovascular stent implantation, open surgical, and laparoscopic treatments have been previously described. In this study, we present a new method of conducting end-to-side anastomosis between the inferior mesenteric and left gonadal vein. The proposed method was proven feasible for treating NCS.

Late stent migration into the right ventricle in a patient with nutcracker syndrome.

BACKGROUND: Stent migration into the right ventricle is rare in patients treated with endovenous stenting, but can have potentially serious complications including endocarditis, cardiac arrhythmias, and heart failure. METHODS: We present a case of stent migration into the right ventricle 5 months after stent placement in a patient with nutcracker syndrome. RESULTS: Echocardiography revealed a stent caught within the subvalvular chordal structures, with significant tricuspid regurgitation. Subsequent severe damage to the tricuspid apparatus necessitated prosthetic valve replacement, as tricuspid valvuloplasty failed after stent removal. CONCLUSIONS: Because stent migration is a potential complication in left renal vein stenting that can occur up to 5 months after intervention therapy, follow-up using ultrasonography is necessary. In addition, knowing the precise location of the stent, which is important for subsequent treatment, is essential when transabdominal ultrasonography reveals the absence of the stent in the left renal vein.

Gross hematuria caused by nutcracker syndrome and segmental pseudoaneursym.

Nutcracker syndrome is a rare disorder characterized by extrinsic compression of left renal vein by the superior mesenteric artery and aorta. Prevalence of the disease is unknown, but presents most commonly with gross hematuria and flank pain. Diagnosis requires a high index of suspicion and treatment consists of a wide range of vascular surgical options with a more recent focus using an endovascular approach. We present a case of a 29-year-old female with continuous gross hematuria and flank pain from a segmental artery pseudoaneursym secondary to nutcracker and pelvic congestion syndrome.

Retroperitoneal laparoscopic reimplantation of the left renal vein for nutcracker syndrome.

OBJECTIVE: To describe the feasibility of retroperitoneal laparoscopic reimplantation of the left renal vein (LRV) for nutcracker syndrome (NCS). PATIENTS AND METHODS: Two patients with NCS underwent the surgery. Both patients complained of gross hematuria and flank discomfort that could not be relieved by resting. They were placed in a supine position and 5 ports were placed in the right abdominal wall. The procedures were performed with a retroperitoneal approach. The LRV was transected and then reimplanted into the distal inferior vena cava. RESULTS: The procedures were performed successfully without any major complications. The total operation time was 105 and 120 min, respectively. Hematuria and flank discomfort were resolved after the surgery. Ultrasonography revealed a patent lumen without compression. CONCLUSIONS: Retroperitoneal laparoscopic reimplantation of the LRV appears to be a feasible procedure with satisfactory short-term outcomes.

Macroscopic hematuria caused by congenital portosystemic shunt and concomitant nutcracker syndrome.

Nutcracker syndrome (NCS) is an uncommon vascular abnormality that causes a variety of symptoms that range from asymptomatic microscopic hematuria to severe pelvic congestion. Congenital portosystemic shunt (CPSS) is an extremely rare anomaly that causes serious complications. Many cases of NCS and CPSS that have presented separately have been reported, but no cases of concomitant NCS and CPSS have been reported. We present a case of intermittent macroscopic hematuria in a patient with both NCS and CPSS. We diagnosed NCS on pressure gradient between the left renal vein (LRV) and the inferior vena cava. The presence of CPSS, which emerged from the LRV and connected to the extrahepatic portal vein, was confirmed on computed tomography. The interaction between NCS and CPSS resulted in mild intermittent macroscopic hematuria only, rather than the more common symptoms that occur when NCS or CPSS present separately.

Diagnosis and successful surgical management of posterior nutcracker syndrome in a patient with loin pain hematuria.

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. A 22-year-old white female with a 4-year history of left flank pain and hematuria underwent an extensive workup with normal renal ultrasound and cystourethroscopies. CT scan and MRI revealed a retro-aortic left renal vein. Posterior nutcracker syndrome was considered the most likely diagnosis. The patient underwent a left laparoscopic nephrectomy with auto-transplantation in the right iliac fossa. She developed azotemia shortly after, which resolved and since then has become asymptomatic.

Recurrent hematuria caused by nutcracker syndrome.

A 26-year-old woman presented to the emergency department complaining of left flank pain, and proteinuria and hematuria were detected during urinalysis. A renal ultrasound did not reveal any disorder, and after performing a computed tomography angiography scan, compression of the left renal vein between the superior mesenteric artery and the aorta was seen. This compression is known as Nutcracker syndrome. From among the different treatment options available, it was decided, with patient consensus, to use open surgical management, performing a transposition of the left renal vein to a more distal level in the inferior vena cava. The immediate postoperative care progressed without complications and the symptoms resolved; after 1 year of surveillance, the patient continues to be asymptomatic. Nutcracker syndrome is a rare phenomenon, with few cases described. There are different therapeutic options for the treatment of Nutcracker syndrome, such as open surgery, endovascular treatment, or conservative treatment; because of the low prevalence of this syndrome, there are no sufficiently large series at present or with the necessary long-term surveillance to decide on the most suitable treatment. Distal transposition of the left renal vein in the inferior cava vein has proved to offer good long-term results, and this option offers a higher chance of resolution without the need for as many postsurgery controls as would be required with endovascular treatment.

Infrarenal abdominal aorta aneurysm: a rare cause of anterior nutcracker syndrome with associated pelvic congestion.

We present a rare case of anterior nutcracker syndrome caused by an abdominal aorta aneurysm (AAA). A 61-year-old woman was admitted to our institution for computed tomography angiography. It revealed an AAA 51 mm in diameter that was lifting off of the left renal vein toward the superior mesenteric artery, causing anterior nutcracker syndrome with consequent left renal vein compression and left ovarian vein congestion. Aneurysm resection was performed, followed by left ovarian vein ligation and left adnexectomy to prevent vein conglomerate rupture. This is the first case that describes anterior nutcracker syndrome caused by AAA, which was successfully treated by aneurysm resection.

Transposition of the left renal vein for the treatment of nutcracker syndrome in children: a short-term experience.

BACKGROUND: Nutcracker syndrome is caused by compression of the left renal vein between the superior mesenteric artery and the aorta. Invasive surgical intervention for this pathologic entity is controversial, particularly in the pediatric population. We aim to describe our early clinical and operative experience with such patients. METHODS: We report 3 cases of pediatric patients undergoing successful left renal vein transposition for the treatment of nutcracker syndrome. RESULTS: All 3 patients were female (age 9-17 years) and presented with a mean of 11.7 months of abdominal or left flank pain requiring chronic narcotic analgesia. Initial clinical presentations were associated with either hematuria or proteinuria. Diagnosis of nutcracker syndrome was supported in each case by an elevated renocaval pressure gradient and/or axial imaging demonstrating mesoaortic compression of the left renal vein. All patients underwent open surgical repair, which included left renal vein transposition, liberation of the ligament of Treitz and associated adhesions, as well as excision of periaortic nodal tissue (mean hospital length of stay 5.7 days). After mean follow-up of 13 months, all patients report complete resolution of symptoms and hematuria/proteinuria. CONCLUSIONS: Transposition of the left renal vein is a safe and effective treatment for nutcracker syndrome in appropriately selected pediatric patients. Further experience and long-term follow-up are warranted to better evaluate the sustained efficacy of this procedure in this unique patient population.

Nutcracker syndrome in a young female with solitary functional left ovary: a surgical challenge.

Nutcracker syndrome (NCS), a rare clinical entity, when refractory to medical management warrants surgical intervention. In the following discussion, we present a case of NCS which was managed successfully by left renal vein transposition using a decompression shunt.

Effectiveness of supine/standing urinalysis for differential diagnosis of left renal vein entrapment syndrome combined with or without glomerulopathy.

AIM: To assess the effectiveness of supine/standing urinalysis for differential diagnosis of left renal vein entrapment syndrome (LRVES) combined with or without glomerulopathy. METHODS: The enrolled patients with abnormal urinalysis and LRVES demonstrated by Doppler sonography were guided to perform a supine/standing urinalysis. RESULTS: Fifty-two patients were enrolled. Most of them were adolescents (aged 14-29 years, 73.1%) and with low body mass index (BMI, mean BMI, 19.8 ± 2.4 kg/m(2)). Seventeen cases (32.7%) manifested orthostatic urine abnormalities (OUA, proteinuria and/or haematuria show negative in supine while positive after 15 min standing), two patients who had undergone renal biopsies both showed no evidence of kidney lesions, another two patients were changed from abnormal to normal urinalysis after weight gain. The remaining 35 cases (67.3%) manifested non-orthostatic urine abnormalities (NOUA, proteinuria and/or haematuria show positive both in supine and standing), 15 patients had undergone renal biopsies and showed different degrees of glomerulopathy. After prednisone/immunosuppression therapy, four patients with glomerulonephritis were changed from the NOUA to the OUA classification. Statistics analyses showed that serum total protein and albumin levels were significantly lower (P = 0.028, 0.007, respectively) and urinary protein was significantly higher (P = 0.007) in the NOUA group than in the OUA group. CONCLUSION: After the indication of LRVES by ultrasound, patients with OUA likely have only LRVES, while patients with NOUA likely also have glomerulopathy. Supine/standing urinalysis combined with Doppler sonography can be helpful for differential diagnosis of LRVES combined with or without glomerulopathy.