The utility of magnetic resonance angiography in children with nutcracker syndrome

Background/aim: The presented study aimed to evaluate the utility of magnetic resonance angiography (MRA) in the pediatric population with nutcracker syndrome. Materials and methods: Patients with suggestive clinical symptoms and laboratory findings and got the diagnosis of nutcracker syndrome with Doppler ultrasonography between January 2011­2019 were included in the study. In addition, children who had renal MRA due to hypertension were evaluated as the control group. MRA images of all patients were examined retrospectively by three radiologists at different levels of experience, and the superior mesenteric artery angle, aorta-mesenteric distance, left renal vein diameter both in the regions of aorta-mesenteric, and renal hilum were recorded. Results: Forty-five patients diagnosed with nutcracker syndrome were included in the study. The mean age of patients was 12 (4­16) and 30 (67%) were female. As the control group, 25 patients with hypertension who had MRA were included and they had a mean age of 12 (1­18) and 19 (76%) were male. The mean superior mesenteric artery angle was 26.5 ° (16­73 ± 12) in the patient group and 57.8 ° (25­139, ± 33) in the control group (p < 0.001); the mean aorta-mesenteric distance was 3.3 mm (1.7­6.5, ± 1.1) in the patient group and 8 mm (3.4­32, ± 5.9) in the control group (p < 0.001). MRA measurements of three radiologists were consistent with each other. Conclusion: MRA imaging can be applied as an alternative diagnostic method for Doppler ultrasonography and multidetector CT examinations by radiologists with different experience levels in pediatric patients with nutcracker syndrome.

Endovascular treatment of Nutcracker syndrome: case report.

Chronic pelvic pain is a debilitating disease that directly impacts on quality of life and generates costs for health services. Nutcracker Syndrome is an important cause of pelvic pain and consists of a set of signs secondary to compression of the left renal vein, most commonly between the superior mesenteric artery and the aorta. Treatment remains controversial and varies depending on the patient's clinical severity. However, endovascular treatment with renal vein stenting has achieved excellent results. We report the case of a 59 year-old female treated by endovascular repair with a self-expanding nitinol stent. Clinical data, details of the procedure, and follow-up results are presented. Technical success was achieved and there patient reported no postoperative complications. Short-term, there was relief from symptoms and follow-up imaging tests showed improvement.

[Microscopic spermatic vein ligation for nutcracker phenomenon complicated with left varicocele].

OBJECTIVE: To investigate the clinical effect of microscopic spermatic vein ligation in the treatment of nutcracker phenomenon (NCP) complicated with left varicocele (VC). METHODS: This retrospective study included 31 cases of NCP complicated with left VC treated in our hospital by subinguinal microscopic ligation of the left spermatic vein (group A, n = 11), open retroperitoneal high ligation of the left spermatic vein (group B, n = 11), or conservative therapy (group C, n = 9). The patients were followed up for 6－24 (15.3 ± 5.4) months. We compared the semen parameters, spermatic vein diameter, left testis volume, and recurrence rate among the three groups of patients before and after treatment. RESULTS: Compared with the baseline, the semen quality parameters were significantly improved in both groups A and B at 6 months after treatment (P<0.05) but reduced in group C (P<0.05); the spermatic vein diameter at rest and that at Valsalva maneuver were markedly decreased in groups A (ï¼»2.53 ± 0.27ï¼½ vs ï¼»1.84 ± 0.22ï¼½ and ï¼»3.53 ± 0.19ï¼½ vs ï¼»2.16 ± 0.25ï¼½ mm, P<0.05) and B (ï¼»2.62 ± 0.33ï¼½ vs ï¼»2.15 ± 0.43ï¼½ and ï¼»3.36 ± 0.25ï¼½ vs ï¼»2.44 ± 0.27ï¼½ mm, P<0.05) but increased in group C (ï¼»2.56 ± 0.28ï¼½ vs ï¼»2.94 ± 0.24ï¼½ and ï¼»3.33 ± 0.21ï¼½ vs ï¼»3.77 ± 0.26ï¼½ mm, P<0.05). No statistically significant differences were found in the left testis volume at 6 months after treatment in group A (ï¼»9.85 ± 1.86ï¼½ vs ï¼»10.27 ± 1.18ï¼½ ml, P>0.05), B (ï¼»9.77 ± 2.03ï¼½ vs ï¼»9.96 ± 1.72ï¼½ ml, P>0.05), or C (ï¼»9.83 ± 1.59ï¼½ vs ï¼»10.48 ± 2.05ï¼½ ml, P>0.05), nor in the recurrence rate between groups A and B (P>0.05). CONCLUSIONS: Hematuria, proteinuria and other mild symptoms of nutcracker phenomenon complicated with left VC can be treated palliatively by microscopic ligation of the spermatic vein, which can relieve the clinical symptoms, improve the semen quality, and protect the testicular function of the patient.

Transcatheter ovarian vein embolisation without renal vein stenting for pelvic venous congestion and nutcracker anatomy.

The diagnosis of a nutcracker syndrome can be aggravated by overlap of a nutcracker phenomenon with other pathologies. In patients with nutcracker anatomy and predominantly pelvic congestion symptoms, ovarian vein embolization without left renal vein stenting could be considered a first line therapy.

Bilateral Renal Hilar Nutcracker Phenomenon in a Male Cadaver.

Nutcracker phenomenon (NCP) typically refers to the entrapment of the left renal vein (LRV) between the aorta and the superior mesenteric artery. Similar to the classic NCP, the renal vein can also get entrapped between the segmental branches of the renal artery at the renal hilum, which has been referred to as 'renal hilar nutcracker phenomenon (RHNP).' During routine dissection of a male cadaver of 67 years, the renal veins of both sides at the renal hilum were seen between the segmental branches of renal arteries, which we identified as the 'renal hilar nutcracker phenomenon.' The disposition of the rest of the perihilar structures was normal. 'Renal hilar nutcracker phenomenon' can have similar clinical presentation like that of the nutcracker phenomenon. Hence, knowledge of such anatomical variation at the renal hilum is desirable.

Outcomes of conservative management in patients with nutcracker syndrome.

OBJECTIVE: This study aims to evaluate outcomes in nutcracker syndrome patients with tolerable symptoms and treated conservatively without invasive interventions. METHODS: This prospective study included patients treated conservatively. Promoting weight gain, the endpoint of the study was spontaneous resolution of symptoms. RESULTS: Sixteen patients (75% female and mean age 24.4 ± 3.5 years) underwent conservative management. Over a mean follow-up of 27.3 months [13-42, interquartile range (IQR)], the diameter ratio (5.5 [5-6.5, IQR] vs 4.3 [4.1-6], p = NS), the peak velocity ratio (6 [5-7, IQR] vs 4.8 [4.8-5.8], p = NS), beak angle (27° [24-30, IQR] vs 29° [24-32]; p = NS), and aortomesenteric angle (26° [23-29, IQR] vs 28° [24-30]; p = NS) exhibited no statistically significant changes. Complete resolution and improvement of symptoms were 28.5% and 31.4%, respectively, while 68.5% remained unchanged. CONCLUSIONS: This study shows that a conservative approach contributes to the spontaneous improvement or complete resolution in young adult patients with mild symptoms.

Nutcracker phenomenon: An unusual presentation of acute aortic dissection.

We report a case of acute aortic dissection leading to compression of the left renal vein (LRV), thereby resulting in the nutcracker phenomenon. A 49-year-old previously healthy woman presented with intermittent gross hematuria and mild left flank pain of five days' duration. Laboratory examinations were within normal limits, except for the elevated C-reactive protein. Cystoscopy revealed bleeding from the left ureteral orifice. Contrast-enhanced computed tomography demonstrated acute Type B aortic dissection and compression of LRV between the enlarged aorta and superior mesenteric artery with an associated dilatation of the left gonadal vein as a collateral circulation.

Nutcracker phenomenon secondary to superior mesenteric artery syndrome.

Superior mesenteric artery (SMA) syndrome, compression of the duodenum due to a decreased angle between the aorta and SMA, has a wide range of clinical presentations making it difficult to diagnose. Compression of the left renal vein is known as Nutcracker syndrome. We present the case of a 26-year-old male with a delayed diagnosis of SMA syndrome and Nutcracker phenomenon due to the patient's history of chronic pancreatitis. As a result of his obstruction and aspiration pneumonia, he was diagnosed with septic shock. The patient was treated for septic shock and maintained on an enteric diet with improvement in the body mass index and complete resolution of SMA syndrome symptoms.

CT Evaluation of the Findings of Nutcracker Syndrome in Patients with Bladder Cancer after Radical Cystectomy and Ileal Neobladder Formation: A Correlation with Hematuria.

Purpose: Patients with bladder cancer may show hematuria after radical cystectomy with ileal neobladder formation, causing anxiety regarding tumor recurrence. Here, we aim to show that the nutcracker syndrome (NCS) can be a cause of hematuria post-operation, and is a common, rather than a rare syndrome. Materials and Methods: A retrospective review of contrast-enhanced abdominopelvic CT (CE-APCT) and urine analysis (UA) findings of 255 patients with bladder cancer who underwent radical cystectomy and ileal neobladder formation between 2011 and 2016 was performed. In the CE-APCT review, the left renal vein flow patterns were evaluated to determine the presence of NCS findings. In the UA review, patients were classified according to the percentage of UA tests with positive hematuria among the total number of UA tests. Results: CT findings of NCS were present in 31.9% of the 135 patients. In the positive hematuria group, there were 26% more patients with NCS findings than those without. Conclusion: NCS findings are prevalent even for bladder cancer patients after surgery, and there is a strong correlation between NCS findings and hematuria. Furthermore, the prevalence of NCS findings is much higher than urinary tract recurrence after the surgery.

A Rare Case of Nutcracker Phenomenon Associated With Midgut Malrotation and Congenital Solitary Kidney.

Medical conditions such as the nutcracker phenomenon, midgut malrotation, and congenital solitary kidney are rare. Hereby, we present a 21-year-old South Asian male referred to the nephrology clinic for hypertension and increased serum creatinine and was diagnosed with all three conditions. We briefly reviewed the literature on this subject. We present this case report to highlight the complex interplay of multiple renal and gastrointestinal anomalies and to emphasize the importance of a multidisciplinary approach in patient care to optimize outcomes and quality of life.

Using the Resected Kidney for Transplantation After Nephrectomy for Nutcracker Syndrome.

Nutcracker syndrome (NCS) is the clinical manifestation of unilateral renal venous hypertension. It develops secondary to the nutcracker phenomenon caused by compression of the left renal vein between the superior mesenteric artery and the aorta. We present the case of a 43-year-old female with a history of left flank pain, pelvic congestion, and hematuria secondary to NCS. The patient frequently required high-dose non-steroidal anti-inflammatory medications with minimal relief. She initiated a kidney donor evaluation after electing to undergo a nephrectomy for the possible long-term resolution of NCS symptoms. If diagnosed early, NCS does not generate pathology within the kidney. This finding allows an individual with medically refractory NCS to avoid the morbidity of a complex surgical procedure by instead donating their kidney. Attention to this treatment modality could provide individuals with NCS resolution of symptoms while providing someone with end-stage renal disease with a life-saving organ.

Nutcracker phenomenon with a daily persistent headache as the primary symptom: Case series and a proposed pathogenesis model based on a novel MRI technique to evaluate for spinal epidural venous congestion.

OBJECTIVE: To determine if a specific population of patients with a daily persistent headache from onset have underlying nutcracker physiology and to propose a pathogenesis model for their headaches utilizing a novel MRI protocol. BACKGROUND: A single case report of a daily persistent headache associated with nutcracker syndrome was recently published. As the left renal vein has a connection to the spinal lumbar veins and secondarily to the spinal epidural venous plexus, one could hypothesize that renal vein compression could lead to persistent headache by altering spinal and cerebral venous pressure with secondary alterations in CSF pressure. The authors have published on a series of patients with a unique subtype of daily persistent headache from onset that appears to be caused by an abnormal reset of CSF pressure to an elevated state. The goal of the present study was to look for the presence of nutcracker physiology in this unique patient subgroup and to propose a pathogenesis model utilizing a novel MRI protocol to evaluate for retrograde lumbar vein flow and regional spinal epidural venous plexus congestion. MATERIALS AND METHODS: Case series of patients with a daily persistent headache from onset, head pressure, and whose headaches worsened in the Trendelenburg position. Patients were imaged with a 3 T MRI in the supine position from the lower diaphragm to the top of the pelvis with a dynamic angiogram centered over the left L2 lumbar vein. RESULTS: 12 patients were studied of which 8 were positive for left renal vein compression, lumbar vein dilation and early spinal epidural venous plexus enhancement. All were women. Mean age of headache onset was 39 years. Six of the 8 patients had a lumbar puncture, and all had a normal opening pressure. All improved with CSF volume removal although pain resolution lasted from hours to 6 months. The patient's headaches were marked by holocranial pressure and the majority displayed migrainous associated symptoms although none had a prior headache history. They did not complain of typical symptoms or signs of nutcracker syndrome. CONCLUSION: We suggest that patients with a daily persistent headache from onset who worsen in the Trendelenburg position may have underlying nutcracker physiology. From our imaging findings, it can be hypothesized that left renal vein compression leads to retrograde flow through the valveless lumbar vein which then leads to spinal epidural venous congestion and subsequently causes congestion of the cerebral venous system leading to an elevation of CSF pressure and to a daily headache. What appears to be unique about these patients is that a daily headache is their only manifestation of nutcracker physiology.

Prevalence of Incidentally Detected Vascular Compressions in Abdominal Computed Tomography.

Background: Abdominopelvic vascular structures are exposed to be compressed by adjacent organs or might cause compression of the adjacent hollow viscera. Most of these conditions are asymptomatic and they are detected on imaging incidentally. However, when they are symptomatic, they can lead to a variety of uncommon syndromes in the abdomen and pelvis. Aim of the study was to assess the prevalence of incidental abdominopelvic vascular compressions on computed tomography. Method: A retrospective cross-sectional study was conducted. All the CT was performed using 64 slice machine. All computed tomography scan of the abdomen between January and April 2019 were evaluated. Data were collected by evaluating abdominal Computed Tomographic scans from Picture archiving and communication system (PACS). Statistical analysis was performed by using SPSS version 25.0 software. Results: Out of 623 multi detector abdominopelvic computed tomography (MDCT) performed between January 2019 and April 2019; a total of 513 (N = 513) patients were included in the study. This study group comprised of 277 (54 %) females and 236 male (46%) patients. Mean age was 38 ± 20 (mean ± SD). We identified 35(6.8%) participants with imaging features of Superior mesenteric artery (SMA) compressions and a 34(6.6%) with imaging features of nutcracker phenomenon. The celiac artery was compressed by median arcuate ligament (MAL) in 22(4.3%) of them. Conclusion: Incidentally detected intraabdominal vascular compressions are common to asymptomatic patients. This result emphasizes that, vascular compression syndromes diagnosis should not be made on imaging alone.

Nutcracker Phenomenon: A Rare Incidental Finding.

The nutcracker phenomenon, or left renal vein (LRV) entrapment syndrome, occurs when there is compression of the LRV, mostly between the abdominal aorta and the superior mesenteric artery. Patients with nutcracker syndrome (NCS) may present with various symptoms, with the most common being hematuria, left flank pain, varicocele in males, proteinuria, and anemia. Our 22-year-old male patient presented with abdominal pain without hematuria. Insidiously, we made the diagnosis of NCS with this unusual presentation. Some studies have proposed a relationship between rapid weight loss in a short period of time and the appearance of NCS. We recommend that healthcare providers suspect NCS in patients who present with abrupt severe abdominal discomfort, particularly in situations that coincide with rapid weight loss for an unknown reason.

Correlation between "Nutcracker phenomenon" and venous hypertension of the lower extremities: Three case reports.

We report three cases of left renal vein (LRV) compression resulting in deep venous insufficiency of the lower extremities, among which two patients showed clinical symptoms in both lower extremities. All patients were treated successfully with LRV stenting. The pathophysiology suggested that the secondary gonadal vein reflux owing to LRV compression drained into the iliac veins, resulting in a volume overload and venous hypertension in the iliocaval region. Computed tomography angiography was useful in diagnosing this condition, allowing the evaluation of LRV compression, identification of early opacification of the gonadal vein reflux, and exclusion of other pathologies resulting in deep venous insufficiency of the lower extremities.

Nutcracker Phenomenon with Menorrhagia in a Woman with Marfan Syndrome.

Nutcracker phenomenon (NCP) refers to left renal vein compression at the superior mesenteric artery origin involving hematuria and dysuria due to the compression of the renal venous return and pelvic congestion syndrome caused by the compression of the gonadal venous return. A leptosomatic woman (body mass index, 19 kg/m2) presented with NCP and Marfan syndrome accompanied by severe menorrhagia. Vascular ultrasonography revealed reversed flow in the left ovarian vein. Preoperative computed tomography revealed a sharp aortomesenteric angle and short aortomesenteric distance. After controlling her menstrual period via oral contraception, she underwent valve-sparing surgery for aortic root dilation, which spontaneously subsided the menorrhagia.

From Nutcracker Phenomenon to Nutcracker Syndrome: A Pictorial Review.

Left renal vein (LRV) entrapment, also known as nutcracker phenomenon if it is asymptomatic, is characterized by abnormality of outflow from the LRV into the inferior vena cava (IVC) due to extrinsic LRV compression, often accompanied by demonstrable lateral (hilar) dilatation and medial (mesoaortic) stenosis. Nutcracker syndrome, on the other hand, includes a well-defined set of symptoms, and the severity of these clinical manifestations is related to the severity of anatomic and hemodynamic findings. With the aim of providing practical guidance for nephrologists and radiologists, we performed a review of the literature through the PubMed database, and we commented on the definition, the main clinical features, and imaging pattern of this syndrome; we also researched the main therapeutic approaches validated in the literature. Finally, from the electronic database of our institute, we have selected some characteristic cases and we have commented on the imaging pattern of this disease.

Splenic vein enlargement, a rare cause of nutcracker syndrome.

Abnormal enlargement of the splenic vein is one of the etiologies of nutcracker syndrome that should be considered when examining the causes of this syndrome. Because knowing rare etiologies can help correct diagnose and reduce mortality.

A Rare Cause of Abdominal and Flank Pain in Children: Nutcracker Syndrome.

The nutcracker phenomenon is characterized by compression of the left renal vein typically between the abdominal aorta and superior mesenteric artery. It is an uncommon and often undiagnosed condition that has the potential to cause a range of symptoms including hematuria and abdominal or flank pain. The term nutcracker syndrome refers to the clinical manifestations of the nutcracker phenomenon. Diagnosis can be made with Doppler ultrasound, computed tomography angiography, magnetic resonance angiography, or venography. Management can range from conservative treatment in the pediatric population due to high spontaneous remission rate to surgical and endovascular interventions. We discuss the case of a previously healthy young female who presented with abdominal pain. Diagnosis of nutcracker syndrome was made based on imaging. The patient was managed conservatively. This case highlights the importance of considering nutcracker syndrome in the differential diagnosis when evaluating patients with abdominal and flank pain.