Beyond Abdominal Pain: Decoding the Mysteries of Nutcracker Syndrome.

Nutcracker syndrome (NCS), a rare but impactful vascular condition, emerges from the compression of the left renal vein by adjacent major arteries, leading to a diverse array of symptoms such as hematuria, flank pain, and renal challenges. Highlighting the case of a 30-year-old male with an atypical presentation of NCS, this report explores the diagnostic complexities arising from its varied presentations and therapeutic options. It emphasizes the critical role of computed tomography (CT) in unveiling the underlying vascular constriction. Through this lens, the case underscores the necessity of considering NCS in the differential diagnosis of abdominal pain, advocating for a prompt and accurate diagnosis to guide effective management strategies, ranging from conservative approaches to surgical intervention. This stresses the importance of heightened awareness and ongoing research for optimizing patient outcomes in the face of this elusive condition.

Nutcracker Syndrome Revealed by Hematuria in a Young Woman: A Case Report and Literature Review.

Nutcracker syndrome is a rare condition that arises from the left renal vein getting compressed between the aorta and the superior mesenteric artery. Despite its clinical significance, this syndrome is often challenging to diagnose and is frequently overlooked. Its key clinical presentations include hematuria and pelvic or back pain. This condition involves elevated pressure on the left renal vein, leading to various signs and symptoms, with hematuria being a common manifestation. Herein, we report a 28-year-old woman with no medical history who presented with hematuria for two months. Abdominal CT revealed compression of the left renal vein between the superior mesenteric artery and abdominal aorta, with dilated left ovarian vein and pelvic varices, consistent with nutcracker syndrome.

A unique posterior nutcracker syndrome combined with Wilkie syndrome: A singular case.

Left renal vein variants are not commonly observed in the general population. Usually, the renal vein runs in front of the aorta before entering the inferior vena cava, while the most common variants include the presence of a circumaortic or retroaortic renal vein. However, when present, left venal rein variants are important to recognize due to their potential clinical and surgical relevance. In this regard, CE-CT is an instrument with high sensitivity and specificity in detecting vascular anomalies and can certainly help diagnose. In this article, we present a unique case of a left venal rein compressed between the left iliac artery and vertebral bodies associated with the presence of a superior mesenteric artery Syndrome, another rare entity that occurs when the duodenum is compressed between the aorta and the superior mesenteric artery.

Case Report; Concomitant Left Nutcracker Syndrome and Right Ureteropelvic Junction Obstruction.

We report rare case of concomitant left nutcracker syndrome and right ureteropelvic junction obstruction (UPJO) on adult female patient diagnosed by CT urogram after she presented with intermittent bilateral flank pain. For this we did Anderson-Hynes pyeloplasty for right ureteropelvic obstruction, it was laparoscopic initially but due to difficulty of stenting it is changed to open. She had smooth post-op course then discharged on 3rd post-op day. Symptoms of nutcracker syndrome are not that much bothersome for the patient so we planned to follow her conservatively.

A new notable compression source of left renal vein entrapment: right renal artery.

PURPOSE: To estimate the incidences of left renal vein (LRV) entrapment by right renal artery (RRA), a phenomenon primarily reported as case reports. METHODS: The cross-sectional study consecutively screened renal vessel CT data of 38 (Renal) patients with nephropathy and 305 (Non-renal) patients with peripheral arterial diseases in a teaching hospital in northeast China between November 2018 and March 2023. The LRV compression by adjacent anatomical structures, including but not limited to RRA and multiple compression-related parameters, were investigated through multiplanar analysis of the CT data. RESULTS: The overall LRV entrapment rates by adjacent structures were 41.93% (12/31) and 24.00% (6/25), the rates of RRA-sourced LRV compression 22.58% (7/31) and 20.00% (5/25), and the rates of compression by superior mesenteric artery (SMA) 16.13% (5/31) and 4.00% (1/25) in the Renal and Non-renal groups, respectively, with no significance. The venous segments distal to the RRA-compressed site had a significantly larger transectional lumen area than those of the non-compressed veins in both groups (3.09 ± 1.29 vs. 1.82 ± 0.23, p < 0.001 and 4.30 ± 2.65 vs. 2.12 ± 0.55, p = 0.006; maximum-to-minimum area ratios in Renal and Non-renal groups, respectively). Nearly 80% of RRAs were found arising anteriorly rightwards instead of passing straight to the right. CONCLUSION: RRA-sourced LRV compression was not rare, and its incidence was higher than that of the compression by SMA in both patient cohorts. RRA could be a more common compression source than SMA concerning LRV entrapment. Further investigations involving different populations, including healthy individuals, are needed.

The Da Vinci Single-Port (SP) Treatment of Nutcracker Syndrome: A Case Report of a Novel Approach.

Nutcracker syndrome (NCS) is a rare disease affecting the left kidney. Surgical management is the only choice of treatment. Minimal invasive surgeries can be effective and may prevent complications of the major surgery. We present the case of a 33-year-old woman suffering from chronic left flank pain, diagnosed with NCS and treated with extravascular stents. Robotic-assisted extravascular stent insertion was performed using the Da Vinci single-port (SP) (Intuitive Surgical, Inc., Sunnyvale, USA) system. This approach offers the advantages of minimal invasiveness, precise stent placement, and reduced operative time. To our knowledge, this is the first case of using Da Vinci SP for this indication. Further studies are needed to evaluate the long-term outcomes and safety.

Hemodynamics in nutcracker syndrome: implications for diagnosis.

BACKGROUND: Nutcracker syndrome is a disease characterized by complex symptoms, making its diagnosis challenging and often delayed, often resulting in a painful experience for the patients. OBJECTIVE: This study aimed to investigate the pathogenesis of nutcracker syndrome through the perspective of hemodynamics by simulating blood flow with varying compression degrees of the left renal vein. METHODS: 3D patient-specific vascular models of the abdominal aorta, superior mesenteric artery and left renal vein were constructed based on CT images of patients suspected of having nutcracker syndrome. A hemodynamic simulation was then conducted using computational fluid dynamics to identify the correlation between alterations in hemodynamic parameters and varying degrees of compression. RESULTS: The study indicated the presence of an evident gradient in velocity distribution over the left renal vein with relatively high degrees of stenosis (α ≤ 50°), with maximum velocity in the central region of the stenosis. Additionally, when the compression degree of the left renal vein increases, the pressure distribution of the left renal vein presents an increasing number of gradient layers. Furthermore, the wall shear stress shows a correlation with the variation of blood flow velocity, i.e., the increase of wall shear stress correlates with the acceleration of the blood flow velocity. CONCLUSIONS: Using computational fluid dynamics as a non-invasive instrument to obtain the hemodynamic characteristics of nutcracker syndrome is feasible and could provide insights into the pathological mechanisms of the nutcracker syndrome supporting clinicians in diagnosis.

Vascular compression syndromes in the abdomen and pelvis: a concise pictorial review.

Vascular compression syndromes are a diverse group of pathologies that can manifest asymptomatically and incidentally in otherwise healthy individuals or symptomatically with a spectrum of presentations. Due to their relative rarity, these syndromes are often poorly understood and overlooked. Early identification of these syndromes can have a significant impact on subsequent clinical management. This pictorial review provides a concise summary of seven vascular compression syndromes within the abdomen and pelvis including median arcuate ligament (MAL) syndrome, superior mesenteric artery (SMA) syndrome, nutcracker syndrome (NCS), May-Thurner syndrome (MTS), ureteropelvic junction obstruction (UPJO), vascular compression of the ureter, and portal biliopathy. The demographics, pathophysiology, predisposing factors, and expected treatment for each compression syndrome are reviewed. Salient imaging features of each entity are illustrated through imaging examples using multiple modalities including ultrasound, fluoroscopy, CT, and MRI.

Robotic Renal Vein Bypass: A Novel Technique for Treating a Challenging Case of Nutcracker Syndrome.

This case report highlights the successful application of a robotic-assisted surgical approach in managing Nutcracker syndrome. The patient, a 36-year-old female presented with severe symptoms and underwent robotic left renal vein transposition after failing conservative management. The procedure was performed through a minimally invasive approach utilizing the Da Vinci robotic system™ which offers enhanced visualization and precision. However, challenges arose during the renal vein anastomosis due to tension and poor flow through the transposition, requiring two revisions with a bovine pericardial patch. Ultimately, an 8 mm ringed PTFE bypass was anastomosed from the distal left renal vein to the Inferior Vena Cava. Despite these challenges, the patient experienced a successful outcome with complete symptom resolution of this complicated pathology.

Endovascular retrieval of a migrated covered stent from the pulmonary artery.

Stent migration is a rare but significant complication following endovascular procedures. Techniques for managing dislodged stents have included surgical, endovascular, and conservative approaches. This case details a patient who had a covered stent placed within the left renal vein which later migrated to the pulmonary artery causing damage to the tricuspid valve. The migrated stent was successfully removed using a percutaneous endovascular approach utilizing fluoroscopy and transesophageal echocardiogram guidance.

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 syndrome unveiled by severe Typhoid fever: A rare case report.

Typhoid fever, caused by Salmonella Typhi, is a severe bacterial infection prevalent in developing countries, and can result in life-threatening complications if untreated. Nutcracker Syndrome is a rare vascular disorder involving compression of the left renal vein between the aorta and the superior mesenteric artery. It can lead to various symptoms and poses diagnostic and management challenges. We present a case study of a patient diagnosed with typhoid fever in a Unit of Critical Emergency Care. Coincidentally, the evaluation through CT-scan revealed the presence of Nutcracker Syndrome. This report underscores the incidental discovery of Nutcracker Syndrome during the assessment of a patient with typhoid fever in a critical emergency care setting.

Patient-Reported Outcomes for Robot-Assisted Laparoscopic Extravascular Renal Vein Stent Placements for Nutcracker Syndrome.

Introduction: Nutcracker phenomenon is the compression of the left renal vein between the superior mesenteric artery (SMA) and the abdominal aorta. Nutcracker syndrome refers to the presence of nutcracker phenomenon with symptoms. Between 2016 and 2022, we performed 18 robot-assisted laparoscopic extravascular renal vein stent (RALERVS) placements. We sought to assess patient-reported outcomes of RALERVS placement by a single institution. Methods: We performed a single-center retrospective review of 18 patients with a minimum of 3 months follow-up. Symptoms were assessed utilizing a questionnaire conducted with a 5-point Likert scale at a minimum of 3 months postoperatively. Five on the Likert scale was severe while 1 was none. Primary study outcomes compared pre- and postoperative patient-reported symptom scores. Results: Twelve out of 18 patients responded to the survey. The average length of time from date of operation to completion of survey was 2.6 years. Average age of the cohort was 36 years with a mean BMI of 19.4 kg/m2. There was only one man. Mean operative time was 137 minutes and mean estimated blood loss was 12 mL. Mean preoperative SMA angle was 19° and mean postoperative SMA angle was 36°. Patients reported that flank pain, abdominal pain, nausea, headaches, back pain, pelvic pain, and early satiety improved (p < 0.05). Ten of 12 respondents would recommend RALERVS for nutcracker syndrome. Conclusion: RALERVS demonstrates an effective treatment for nutcracker syndrome. Patients reported improved symptoms at 3 months postoperatively across multiple domains. Further studies need to be conducted to assess long-term durability of the extravascular renal vein graft.

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.

Morphological Changes of the Ovarian Vein in Pelvic Venous Disorders.

OBJECTIVE: The management of pelvic venous disorders (PeVD) remains controversial. Open surgical and endovascular methods are currently used for treatment, but there are few data in the literature on the morphology and histology of the ectatic ovarian vein (OV). This study aimed to explore the histomorphological changes in a dilated OV in patients with PeVD and compare it with a normal OV obtained post-mortem and a normal great saphenous vein (GSV). METHODS: Histology of the OV was studied in 16 patients who underwent surgery for PeVD, 10 control cadavers from whom fragments of the OV without visible gross changes were taken at autopsy, and nine control patients in whom the GSV was resected to be used for coronary artery bypass. RESULTS: The OV wall in patients with PeVD consisted of three layers: intima, media, and adventitia. The OV looked very similar to the GSV wall because of a clearly developed layer of smooth muscle fibres. The thickness of the normal OV was significantly different to the OV wall in PeVD (475.3 µm, IQR 370.7, 607.6 vs. 776.3 µm, IQR 668.9, 879.6, p < .001) and did not differ significantly from the thickness of a normal GSV wall (784.3 µm, IQR 722.2, 898.2). The intima-media complex of the OV was significantly thinner than the GSV in PeVD (118.9 µm, IQR 75.6, 159.6 vs. 415 µm, IQR 399.5, 520.0, р < .001); however, the adventitia of the OV was significantly thicker than in normal OV and GSV (599.6 µm, IQR 444.3, 749.7 vs. 373.5 µm, IQR 323.8, 482.0 vs. 308.4 µm, IQR 275.9, 338.2, p < .001). CONCLUSION: Dilatation of the OV in patients with PeVD was accompanied by a significant increase in the overall thickness of the vein wall, which brings it closer in structure to the GSV. This implies that the OV may be used safely for transposition into the inferior vena cava or iliac vein.

Effect of left renal vein compression stenosis on functional status of left kidney in patients with pelvic venous disorders.

OBJECTIVE: We evaluated the effect of left renal vein (LRV) compression stenosis on the functional state of the left kidney in patients with pelvic venous disorders (PeVDs). METHODS: We examined 162 female patients with PeVD and diagnosed LVR compression stenosis using duplex ultrasound (DUS) in 40. Patients with clinical manifestations of PeVD (n = 26) had symptoms and signs of pelvic venous congestion but without pain in the left flank of the abdomen, typical for nutcracker syndrome (NS). The remaining patients were asymptomatic (n = 14). The parameters measured with DUS included the angle of the superior mesenteric artery (SMA) with the aorta, the LRV diameter (Dhilum/Dstenosis) ratio, and LRV velocity (Vstenosis/Vhilum) ratio. All the patients underwent laboratory testing (complete blood count, urinalysis, and biochemical blood testing) and dynamic renal scintigraphy to assess the secretory and evacuation functions of the renal tubular system. RESULTS: The laboratory tests revealed no abnormalities, including no hematuria or proteinuria, in either group. The Dhilum/Dstenosis and Vstenosis/Vhilum ratios varied from 2.8 to 5.2 and from 2.9 to 8.3, respectively, and did not differ between the symptomatic and asymptomatic patients. All 40 patients with LRV compression stenosis were diagnosed with left gonadal vein reflux with a mean duration of 4.7 ± 0.6 seconds and 2.2 ± 0.6 seconds in the symptomatic and asymptomatic patients, respectively (P = .005). Eight patients had signs of NS on DUS, including five in the symptomatic group (SMA angle, 34.8° ± 2.7°; Dhilum/Dstenosis ratio, 5.2 ± 0.2; and Vstenosis/Vhilum ratio, 5.7 ± 0.4) and three in the asymptomatic group (SMA angle, 35° ± 2.8°; Dhilum/Dstenosis ratio, 5; and Vstenosis/Vhilum ratio, 5 ± 0.5). The groups did not differ significantly in the DUS parameters. Scintigraphy did not reveal any cases of secretory or evacuation dysfunction of the left kidney, including in the patients with DUS signs of NS. The maximum uptake time, elimination half-life, and effective renal plasma flow were within the normal ranges. CONCLUSIONS: LRV compression stenosis without hematuria has no significant effects on the functional state of the left kidney, irrespective of the disease severity. In patients with PeVDs, dynamic renal scintigraphy provides an objective assessment of left kidney function.

Nutcracker syndrome: a case-based review.

The nutcracker phenomenon, also known as left renal vein entrapment, occurs when there is extrinsic compression of the left renal vein, most often between the abdominal aorta and the superior mesenteric artery. Nutcracker syndrome refers to the constellation of clinical symptoms that may arise from the nutcracker phenomenon, typically inclusive of haematuria, flank/pelvic pain, orthostatic proteinuria and (in male patients) varicocele. We provide a short review of the nutcracker syndrome including various diagnostic and therapeutic modalities. We utilise our own experience with a patient as a case study and highlight the modern management option of endovascular stenting.

Superior Mesenteric Artery Syndrome and Nutcracker Syndrome as the Presentation of Crohn's Disease in a Young Patient: A Case Report and Review of Literature.

Superior mesenteric artery syndrome and nutcracker syndrome are rare vascular complications most often seen after marked weight loss caused by compression of the duodenum and left renal vein between the superior mesenteric artery and the aorta, respectively. The coexistence of superior mesenteric artery syndrome and nutcracker syndrome has been rarely reported. Herein, we present the case of a 16-year-old male with intermittent periumbilical abdominal pain, bilious vomiting, and weight loss who was found to have both of these vascular complications of significant weight loss as the initial presentation of Crohn's disease. This report provides insight into the diagnosis and treatment of these syndromes while highlighting the importance for practitioners to keep vascular complications on their differential diagnosis of vomiting and abdominal pain in patients with Crohn's disease.

A secondary daily persistent headache from onset with underlying nutcracker physiology and spinal epidural venous congestion: case series with lumbar vein embolization as a therapeutic approach.

The authors have published on a unique subset of patients whose headaches worsened in the Trendelenburg position and who on time-resolved MR angiography demonstrated left renal vein compression (nutcracker physiology) with retrograde left second lumbar vein (L2LV) flow and regional spinal epidural venous plexus (EVP) congestion. We hypothesized that the spinal EVP congestion subsequently causes a secondary congestion of the cerebral venous system, which then leads to an elevation of CSF pressure above that individuals CSF pressure set point. This results in a daily headache from onset. Thus, eliminating the spinal EVP could conceivably improve or eliminate the manifested headache syndrome. We now present a case series of four patients with long-term follow-up utilizing lumbar vein coil embolization as a new therapeutic approach. In each patient, the MR angiography findings were verified by catheter-based venography. Treatment consisted of endovascular embolization of the second lumbar vein. Four patients have had coil embolization of which three are 1 year or longer from their procedure while one is 10 months posttreatment. All patients were women. Duration of daily headache prior to embolization ranged from 4 to 8 years. Post-embolization: Three patients are either headache free or 90-95% improved with substantial pain free time. There were no procedure-related complications. Our results suggest that embolization of L2LV in a specific patient population with nutcracker physiology may substantially improve head pain issues. This is a minimally invasive outpatient technique with no apparent side effects.