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.

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.

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.

Síndrome de cascanueces y agenesia renal derecha. Una rara asociación

Introducción: El síndrome de cascanueces se considera una anomalía infrecuente y poco pensada en la práctica médica; su incidencia no está bien establecida debido a su sintomatología variada. La agenesia renal unilateral se estima entre 1/2500 y 1/4000 nacidos vivos. Objetivos: Describir una paciente de 18 años con sospecha prenatal de agenesia renal derecha confirmada por imágenes después del nacimiento, que ingresó por proteinuria. Presentación del caso: Paciente de 18 años con agenesia renal derecha conocida, en la que un examen de orina detectó proteinuria desde los 13 años. En su estudio se clasificó como proteinuria ortostática y evolutivamente refirió dolor lumbar izquierdo ligero y transitorio. En el ultrasonido renal, realizado para valorar crecimiento del riñón único, se detectó dilatación de la vena renal izquierda. Se repitió el estudio ecográfico para precisar ángulo aorto-mesentérico y dilatación de vena renal izquierda, y se confirmó el síndrome de cascanueces. Conclusiones: La asociación entre agenesia renal derecha y síndrome de cascanueces, resulta extremadamente rara, y, cuando se presenta con proteinuria ortostática y dolor lumbar ocasional, debe seguirse en forma expectante, pero no se necesita tratamiento quirúrgico en la mayoría de los casos.

Introduction: Nutcracker syndrome is considered an infrequent and poorly thought out anomaly in medical practice; its incidence is not well established due to its varied symptomatology. Unilateral renal agenesis is estimated to be between 1/2500 and 1/4000 live births. Objectives: To describe an 18-year-old female patient with suspected pre-natal imaging-confirmed right renal agenesis after birth, who was admitted due to proteinuria. Case presentation: An 18-year-old female patient with known right renal agenesis, in whom a urine test detected proteinuria from the age of 13. In the study it was classified as orthostatic proteinuria and evolutionarily she referred mild and transient left low back pain. Renal ultrasound, performed to assess single kidney growth, showed dilation of the left renal vein. The ultrasound study was repeated to specify aorto-mesenteric angle and left renal vein dilation, and nutcracker syndrome was confirmed. Conclusions: The association between right renal agenesis and nutcracker syndrome is extremely rare, and, when it presents with orthostatic proteinuria and occasional low back pain, it should be followed expectantly, but surgical treatment is not needed in most cases.

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.

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.

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.

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.

Spontaneous rupture of the ovarian vein in association with nutcracker syndrome: a case report.

BACKGROUND: Nutcracker syndrome is a condition in which the left renal vein is pinched between the abdominal aorta and the superior mesenteric artery, resulting in an increase in renal vein pressure and certain symptoms. We report a very rare case of retroperitoneal hematoma caused by the rupture of varicose veins of the left ovary. CASE PRESENTATION: A 77-year-old Japanese woman, para 7, experienced sudden left lower abdominal pain. She had no history of trauma or treatment complications. Computed tomography revealed a left retroperitoneal hematoma, but her abdominal pain subsided quickly; thus, urgent treatment was not required. We then scheduled her for an assessment regarding the cause of her bleeding. However, 6 days after the pain onset, abdominal pain symptoms recurred, confirming hematoma regrowth. Magnetic resonance imaging and three-dimensional computed tomography revealed an abnormal vascular network from the left side of the uterus to the left adnexa. Subsequent angiography revealed that the retroperitoneal bleeding originated from rupture of the distended left ovarian vein, which caused blood reflux from the left renal vein to the left ovarian vein. Although angiography confirmed a passage between the left renal vein and inferior vena cava, computed tomography showed obvious stenosis in the left renal vein. In accordance with these findings, we diagnosed the cause of the distention and rupture of the left ovarian vein as nutcracker syndrome. She underwent embolization of the left ovarian vein as hemostasis treatment, and had a good course thereafter. CONCLUSIONS: This is the first report of a spontaneous rupture of the left ovarian vein caused by nutcracker syndrome. Nutcracker syndrome is not yet well known to clinicians and should be considered as part of the differential diagnosis when an abnormal vascular network in the pelvis is found.

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.

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.

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.

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.

Caracterización del síndrome de cascanueces / Characterization of Nutcracker Syndrome

Introducción: El síndrome de cascanueces es una anomalía vascular poco buscada y diagnosticada, por su baja incidencia y por la dificultad de sospecharla ante manifestaciones clínicas frecuentes. Objetivo: Examinar las características clínicas e imagenológicas de los pacientes diagnosticados con el síndrome de cascanueces. Método: Estudio descriptivo retrospectivo y prospectivo en pacientes atendidos en el Hospital Pediátrico Universitario William Soler entre enero de 2013 - diciembre de 2017. Cuando se sospechó el síndrome se realizó ultrasonido buscando la dilatación de la vena renal izquierda y el ángulo de salida de la arteria mesentérica superior. En 7 de los pacientes se realizó angio-TAC. Se revisaron variables epidemiológicas, clínicas, de laboratorio e imagenológicas. Presentación de los casos: Del total de 9 pacientes en que se diagnosticó la anomalía vascular, 6 (66,7 por ciento) pertenecen al sexo masculino. La edad media al diagnóstico fue 11,4 años (rango: 7-15). Se diagnosticó cascanueces anterior en 7 (77,7 por ciento), posterior en 1 (11,1 por ciento) y síndrome de Wilkie con fenómeno cascanueces asociado en 1 (11,1 por ciento). En el 100 por ciento de los casos hubo dolor abdominal o lumbar, asociado a hematuria en 4, orinas espumosas en 4 y varicocele izquierdo en 2 de los 6 varones (33,3 por ciento). Conclusiones: El síndrome de cascanueces debe buscarse en los pacientes que presentan dolor abdominal, lumbar o en flanco izquierdo en forma recurrente, episodios de hematuria recurrente o proteinuria ortostática. En el varón con varicocele izquierdo debe descartarse esta posibilidad. El fenómeno cascanueces, puede diagnosticarse cuando se investiga al paciente por causa no relacionada(AU)

Introduction: Nutcracker syndrome (NCS) is a vascular anomaly which is rarely searched and diagnosed due to its low incidence and because of the difficulty to suspect on it among other frequent clinical manifestations. Objective: To examine clinical and imaging characteristics of patients diagnosed with NCS. Methods: Descriptive, retrospective and prospective study in patients admitted in William Soler University Pediatric Hospital in the period from January, 2013 to December, 2017. When it was suspected the presence of the syndrome, it was made an ultrasound looking for dilation of the left renal vein and the output angle of the superior mesenteric artery. In 7 patients it was made an angio-TAC. The epidemiologic, clinical, laboratory and imaging variables were reviewed. Presentation of cases: From the total of 9 patients diagnosed with a vascular anomaly; 6 (66,7 percent) were males. The mean age was 11,4 years (range 7-15 years). It was diagnosed front NCS in 7 patients (77.7 percent), back NCS in 1 patient (11.1 percent) and Wilkie syndrome with associated NCS in 1 patient (11.1 percent). 100 percent of the cases presented abdominal or lumbar pain related to hematuria (4 patients), foaming urine (4 patients) and left varicocele in 2 of the male patients (33.3 percent). Conclusions: Nutcracker syndrome should be searched in patients presenting frequent abdominal, lumbar or left side pain, recurrent hematuria or orthostatic proteinuria episodes. In males with left varicocele should be discounted this possibility. Nutcracker phenomenon can be also diagnosed when the patient is being investigated due to not related causes(AU)

Prevalence of left renal vein compression (nutcracker phenomenon) signs on computed tomography angiography of healthy individuals.

OBJECTIVE: We evaluated the frequency of significant left renal vein (LRV) compression, also called the nutcracker phenomenon (NCP), in a normal asymptomatic population. METHODS: The present retrospective descriptive anatomic study analyzed the data from high-definition renal computed tomography (CT) angiography of living kidney donors. A total of 324 CT examinations were evaluated for signs of LRV compression, including the beak sign, aortomesenteric angle <41°, LRV diameter ratio ≥4.9, and beak angle ≥32°. The presence of pelvic varicose veins and the left gonadal vein in the proximal and mid-portion (considered dilated if >0.5 cm) were also evaluated. Anthropometric and laboratory (urine erythrocyte count) data were collected from the medical records. Statistical inference was calculated using Fisher's exact test and Student's t test. RESULTS: The mean aortomesenteric angle was 53.1° in women and 58.7° in men (P = .044). The beak sign and beak angle were present in 15.3% and 9.8%, respectively, and both had a greater prevalence in the women (P = .01). An aortomesenteric angle <41° was identified in 30.5%, with a greater prevalence in women (P < .01). The diameter ratio was positive in 0.7% of the cases, with no difference between the sexes. A left gonadal vein >0.5 cm was more prevalent in women in both the proximal and the mid-portions (P < .01). Although analysis stratified by positive criteria (3 or 4) showed no difference between the sexes, a positive correlation was found with younger age (P < .01). The limitations included the absence of a nutcracker syndrome (NCS) population; the lack of a renocaval pressure gradient, because of the need for intervention; the absence of other types of imaging studies, such as duplex ultrasound scan; and the absence of female parity data. CONCLUSIONS: The NCP and NCS CT criteria were present with a high frequency in healthy individuals. Women and younger individuals showed a greater prevalence of compression findings in the aortomesenteric axis. Revision of the current NCP and NCS criteria with a distinct categorization between sex, age, and body mass index is recommended to better evaluate LRV compressive events.