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Superior mesenteric artery (SMA) syndrome causes duodenal obstruction between the SMA and aorta, which culminates into bowel obstruction. Meanwhile, nutcracker syndrome (NCS) involves left renal vein compression between the aorta and SMA, categorized by the compression site. We present a 15-year-old female with no prior medical or surgical history who displayed early signs of the rarely coexisting SMA and nutcracker phenomena, which were managed symptomatically along with nutritional support to reach her optimal body mass index.

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Introduction: Transplant renal vein thrombosis is a serious post-transplant complication. We report a case in which a thrombus was found in the transplant renal vein and rescued the transplanted kidney utilizing interventional radiology. Case presentation: A 56-year-old woman underwent ABO-compatible living donor renal transplantation due to impaired renal function caused by IgA nephropathy. On postoperative Day 13, there was a finding on transplant renal echocardiography that appeared to be an interruption of peripheral renal blood flow in diastole. Contrast-enhanced computed tomography revealed that the vein was occluded due to the hematoma, and thrombosis was observed within, and distal contrast showed regurgitation into the collateral vessels. The stenosis was breached and balloon dilation restored progressive blood flow through interventional radiology. Conclusion: Although open surgical thrombectomy is mainly considered for treatment for transplant renal vein thrombosis, interventional radiology might be the alternative treatment option.

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Renal angiomyolipoma (AML) is a typically benign renal tumor that is divided into 2 classes, the classical variant and the more aggressive epithelioid variant. It is extremely rare for an AML to exhibit aggressive features such as vascular invasion. We present the case of a 36-year-old female who presented with right lower quadrant pain for 9 months and was found to have an AML with tumor extension into the renal vein and the IVC. Diagnosis was confirmed with histopathology and the patient was treated with a total nephrectomy. The epithelioid subtype of AML is a rare variant that should be considered in the differential of a renal mass with vascular invasion.

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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.

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Renal vein thrombosis (RVT) is not an uncommon condition in patients occurring nephrotic syndrome. Renal cyst by bacterial infection is also rare. Only one case for RVT complicated with infected renal cyst is reported in the English literature. A 78-year-old female was admitted for fever and drowsy mentality for 4 days. Contrast-enhanced computed tomography (CECT) of the abdomen showed 3.7 cm sized irregular shaped exophytic cyst well enhanced in left kidney upper pole and the left RVT. The culture of cystic fluid revealed Klebsiella pneumoniae. Our patient was effectively treated with antibiotics for 8 weeks and anticoagulant for 12 weeks. At 12-week follow-up, CECT of the kidney showed decreased cyst and nearly disappeared RVT. The possibility of RVT in patients with renal cyst infection by bacteria warrants consideration.

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Venous compressive disorders are a heterogenous group of vascular syndromes characterized by extrinsic venous compression that can lead to complications of venous hypertension or venous thrombosis. Endovascular damage secondary to deep venous thrombosis (DVT) can result in post-thrombotic syndrome (PTS), a potentially debilitating condition that can be associated with significant morbidity in the pediatric population. Here we discuss 4 venous compressive disorders: iliac vein compression (May-Thurner syndrome [MTS]); subclavian vein compression at the venous thoracic inlet (Paget-Schroetter syndrome); left renal vein compression (nutcracker syndrome); and popliteal vein compression (popliteal entrapment syndrome) with a focus on clinical evaluation and diagnostic methods. Where endovascular therapy is appropriate, specific procedural considerations including procedure indications, equipment, procedural steps, technical challenges, complications, clinical follow-up and expected outcomes are discussed.

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PURPOSE: Left renal vein compression syndrome (LRVCS) remains a challenging diagnosis. This study aimed to correlate imaging and hemodynamic findings with clinical outcomes for patients with LRVCS. MATERIALS AND METHODS: A retrospective review of 66 renal venography procedures with or without intravascular ultrasound (IVUS) was performed from 2017 to 2023 at a single institution. Patients with prior LRVCS treatment or other indications were excluded (n = 11). Primary outcome measure was correlation of catheter-based endovascular (CBE) findings with clinical outcomes (n = 55). Secondary outcome measures included correlation of CBE findings and LRV (i.e., beak) angle > 32°, beak sign, aortomesenteric angle (AMA < 41°), and hilar-to-aortomesenteric ratio (HTAMR ≥ 4.9) on cross sectional imaging. Descriptive statistics, chi-square testing, and ROC analyses were used. RESULTS: Of the 55 patients, 52 (94.5%) were females (median age 31, range 14-72) and 56.4% (n = 31) had a diagnosis of LRVCS on CBE evaluation. A renocaval pressure gradient of ≥ 3 mmHg, presence of collaterals, and > 50% area stenosis on IVUS were significantly associated with CBE diagnosis of LRVCS (p < 0.001). Surgical treatment (renal autotransplantation or LRV transposition) was recommended to all patients with CBE diagnosis of LRVCS (n = 31). 81.2% (18/22) of patients who underwent surgery reported symptom resolution or improvement. When the cross sectional imaging measurements were compared with CBE evaluation, AMA was the most sensitive (100%), HTAMR and beak sign were highly specific (93.3%), and beak angle was the most predictive (77.4% sensitivity; 86.7% specificity). CONCLUSION: CBE diagnosis of LRVCS was highly predictive of surgical candidacy and post-surgical symptom resolution. The presence of collaterals, > 50% area stenosis on IVUS, or a renocaval pressure gradient ≥ 3 mmHg had a significant association with a CBE diagnosis of LRVCS.

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BACKGROUND: The diverse drainage patterns of the left renal vein (LRV), often with asymptomatic congenital anomalies, present considerable challenges in renal and retroperitoneal surgical contexts. The potential for significant bleeding and subsequent renal compromise upon vascular injury highlights the need for increased surgical awareness. OBJECTIVE: This study investigates the LRV's variable anatomical drainage patterns and morphometry. It also evaluates the embryological factors contributing to these variations and discusses their surgical implications and technical considerations. METHODS: Anatomical dissections were conducted on 21 adult human cadavers within the Department of Anatomy. Concurrently, a retrospective analysis was conducted on 15 patients who underwent various retroperitoneal surgical interventions in the Urology Department. Demographic variables and intraoperative findings were recorded and analyzed. RESULTS: Dissection analysis predominantly identified preaortic LRVs in 18 cadavers. Notable anatomical variations included a circumaortic left renal vein (CLRV), a delayed preaortic confluence of extrahilar duo LRVs, and an extrahilar tetramerous confluence with a retroiliac topography. The majority of LRVs usually end in the inferior vena cava. However, an extrahilar tetramerous variant had an unusual drainage pathway. Out of 15 cases, three (20%) had a retroaortic left renal vein (RLRV). One patient with a nonfunctioning kidney had type 1 RLRV, and another patient with pelvic ureteric junction obstruction had type 4 retroiliac left renal vein (RILRV). In both of these patients, symptoms were relieved after surgery. In a young patient with left varicocele and microscopic hematuria who had type 2 RLRV, symptoms resolved spontaneously after a few months. CONCLUSION: A thorough understanding of the variable anatomical drainage patterns of the LRV is crucial for surgeons. Accurate preoperative identification can provide valuable insights, potentially leading to improved surgical outcomes in renal procedures.

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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.

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BACKGROUND: Short harvested right renal veins (RV) are quite common in living donor kidney transplantation (KT). This technical difficulty might interfere implanting and increase warm ischemic time. Several techniques to overcome this problem have been applied, including iliac vein transposition, inverted transplant, synthetic graft, saphenous vein… Application of accompanying gonadal vein (GV), which is easily approachable and less time-consuming, has been recently published. This study aims to evaluate its effectiveness and safety. METHODS: Retrospective study on KT using the gonadal vein to lengthen the short right renal vein at Viet Duc University Hospital from April 2019 to April 2022. The following data were gathered: baseline characteristics, vascular imaging in CT scan/after nephrectomy and after reconstruction (mm), reconstruction and surgical time, hospitalization days. The outcomes were determined by kidney function after transplantation (plasma creatinine, creatinine clearance) and related complications. RESULTS: Twenty-five cases with procured right kidney with short RV from the living donor which were reconstructed and lengthened by the accompanying GV were collected. The additional length of RV was 15.9 ± 2.4 mm. Average cold ischemic time, venoplasty time, warm ischemic time were 60.4 ± 8.2, 21.2 ± 5.3, and 38.1 ± 5.6 min, respectively. The average hospital stay was 15.3 ± 3.2 days. Average follow-up time was 31 ± 5.2 months, creatinine clearance was around 60 ml/min after 1 year, no vascular or urologic complications was observed. CONCLUSION: Accompanying GV from a living donor to lengthen short right RV in KT is a feasible, safe, and effective technique.

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Background: Variations in renal veins are quite common, and most people do not experience issues due to them. However, these variations are important for healthcare professionals, especially in surgical procedures and imaging studies, as precise knowledge of vascular anatomy is essential to avoid complications during medical interventions. The purpose of this study was to expose the frequency of anatomical variations in the renal vein (RV) and detail their relationship with the retroperitoneal and renal regions. Methods: A systematic search was conducted in the Medline, Scopus, Web of Science, Google Scholar, CINAHL, and LILACS databases from their inception until January 2024. Two authors independently carried out the search, study selection, and data extraction and assessed methodological quality using a quality assurance tool for anatomical studies (AQUA). Ultimately, consolidated prevalence was estimated using a random effects model. Results: In total, 91 studies meeting the eligibility criteria were identified. This study included 91 investigations with a total of 46,664 subjects; the meta-analysis encompassed 64 studies. The overall prevalence of multiple renal veins was 5%, with a confidence interval (CI) of 4% to 5%. The prevalence of the renal vein trajectory was 5%, with a CI of 4% to 5%. The prevalence of renal vein branching was 3%, with a CI of 0% to 6%. Lastly, the prevalence of unusual renal vein origin was 2%, with a CI of 1% to 4%. Conclusions: The analysis of these variants is crucial for both surgical clinical management and the treatment of patients with renal transplant and hemodialysis.

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Phospholipase A2 receptor (PLA2R)-associated membranous nephropathy is an important cause of nephrotic syndrome that can lead to a variety of systemic manifestations. Chylous ascites and bilateral renal vein thrombosis are rare manifestations in adult nephrotic syndrome, and there have been no reported cases demonstrating both chylous ascites and bilateral renal vein thrombosis in patients with PLA2R-associated membranous nephropathy. Here, we report the first case of PLA2R-associated membranous nephropathy complicated by renal vein thrombosis and chylous ascites successfully treated with anticoagulation and rituximab. A 65-year-old African American male presented with abdominal pain for four days, hematochezia for one day, and lower extremity edema for one year. Blood pressure was 158/73 mmHg and other vital signs were normal. Physical examination revealed abdominal distention, periumbilical tenderness, and bilateral lower extremity edema. Laboratory analysis showed high serum creatinine, hypoalbuminemia, hyperlipidemia, and proteinuria on 24-hour urine chemistry, all consistent with nephrotic syndrome. Abdominal computed tomography scan demonstrated nonocclusive bilateral renal vein thrombosis with ascites. Paracentesis revealed chylous ascites. Continuous heparin infusion was started for thrombosis. Esophagoduodenoscopy and colonoscopy did not reveal a source of bleeding. Serum anti-PLA2R was found positive, suggesting membranous nephropathy. Rituximab, along with warfarin switched from heparin, successfully controlled disease activity. Chylous ascites in nephrotic syndrome is thought to be associated with bowel edema. In our case, we hypothesize that renal vein thrombosis caused lymphatic fluid leakage by increasing lymphatic pressure. The case illustrates the importance of considering membranous nephropathy as a cause of chylous ascites and renal vein thrombosis. Development of lymphatic imaging techniques is warranted to clarify the pathophysiology.

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Renal vein thrombosis (RVT) is a relatively uncommon condition that is most frequently observed in individuals with nephrotic syndrome. While rare, pyelonephritis (PN) may serve as a predisposing factor for secondary RVT. In such cases, one should consider the possibility of RVT when patients fail to respond to appropriate antibiotic treatment. Typically, these patients require additional anticoagulation therapy for a duration of 3 to 6 months, with a generally favorable prognosis. In this report, we present the case of a 74-year-old female who developed RVT due to Klebsiella pneumoniae PN. Additionally, we reviewed 11 cases of PN complicated by RVT, which were documented in the PubMed database over a span of 40 years, emphasizing key elements in diagnostic and therapeutic approaches. Lastly, we elaborated upon the role of thrombo-inflammation, especially in the context of sepsis.

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Membranous nephropathy (MN) is a significant cause of nephrotic syndrome in non-diabetic adults. It can be primary, attributed to autoantibodies targeting podocyte antigens, or secondary to various disorders. Although rare, nerve epidermal growth factor-like 1 (NELL-1)-associated MN presents diagnostic and management challenges. Thrombotic complications such as renal vein thrombosis (RVT) are recognized but less reported, especially in NELL-1-positive MN. We report a 43-year-old male with NELL-1-positive MN complicated by acute kidney injury (AKI) due to bilateral RVT, treated successfully with thrombolysis. Histopathological analysis confirmed MN with specific immunohistochemical staining for NELL-1. Treatment included immunosuppressive therapy and tailored anticoagulation. This case emphasizes recognizing thrombotic complications in MN, particularly in NELL-1-positive cases. Further research is needed to explore serum anti-NELL-1 antibodies as biomarkers and optimal anticoagulation strategies in MN patients at risk of thrombotic events to improve outcomes and guide personalized management.

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PURPOSE: Assess whether a Retroaortic left renal vein (RLRV) affects vertebral osteophyte formation in the lumbar spine, compared to normal anatomy left renal vein. METHODS: We conducted a retrospective case-control study. Computed tomography (CT) scans of individuals with a RLRV (study group) were compared to age- and gender-matched normal anatomy CT scans (control group). L1 to L4 vertebral levels were appreciated for: left renal vein level, osteophyte presence and the aorta-vertebral distance (AVD) at the left renal vein level. Univariate analyses were conducted using Chi-square test and Fisher's test for categorical variables, and Student's t-test for continuous variables. Logistic regression was used for multivariate analyses. RESULTS: A total of 240 patients were included in the study - equally distributed between the study and control groups. Normal anatomy left renal veins traversed the spine only at the L1 and L2 levels. RLRVs traversed the spine in all L1-L4 levels, mostly at the L3 and L2. Osteophyte prevalence at the level of left renal vein was significantly higher in the study group, compared with the control group [OR 2.54, P = 0.01]. Mean AVD was greater in the study group [9.2 mm ±3.6 mm Vs. 3.5 mm ± 2.6 mm, P < 0.001]. Increased AVD was found to be associated with a higher chance of osteophyte presence at the level of the left renal vein [OR 1.282, P = 0.025]. CONCLUSIONS: Osteophytes are more prevalent at the level of the RLRV variant compared to the normal anatomy. Furthermore, the RLRV is characterized by a lower lumbar level compared to the normal anatomy. CLINICAL RELEVANCE STATEMENT: This anatomic variation could assist in further understanding of osteophyte formation.

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BACKGROUND: Renal function after left renal vein (LRV) ligation following en bloc resection of segmental inferior vena cava (IVC) and right kidney is understudied. We assessed the impact of LRV ligation on postoperative renal function following en bloc resection of segmental IVC and right kidney. METHODS: We retrospectively reviewed 28 patients who underwent LRV ligation during en bloc resection of segmental IVC and right kidney. Patient demographics, tumor characteristics, intraoperative factors, complications, length of hospital and intensive care unit (ICU) stay, and patient survival were collected. Pre- and postoperative renal function was retrospectively analyzed. RESULTS: Twenty patients underwent robot-assisted surgery and eight patients underwent open surgery. The median operative time was 162 min and estimated blood loss was 350 mL. Ten patients had normal renal function and 12 patients had an initial increase in creatinine but improved gradually. Six patients developed acute renal failure; five patients gradually recovered in 5-32 days after temporary hemodialysis. Renal replacement therapy significantly correlated with maximal anterior-posterior diameter of the LRV (p = 0.001). Complications were observed in 11 cases, four of which were Clavien-Dindo grades I-II. Thirteen patients were alive with no recurrence, nine patients were alive with metastasis, and six cases died during the follow-up period. CONCLUSIONS: LRV ligation following en bloc resection of segmental IVC and right kidney is feasible, with no significant long-term impact on renal function. The maximum anterior-posterior diameter of the LRV is a reliable method for predicting renal replacement therapy in the absence of collateral circulation.

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Renin-secreting tumours are rare causes of secondary hypertension and hypokalaemia. They are usually surgically curable, hence proper diagnostic work-up and tumour localisation is essential. In this paper, we present three Swedish patients recently diagnosed with renin secreting tumours, two with reninomas and one with an extrarenal renin-producing tumour, to illustrate diagnostic challenges. We also discuss the biochemical work-up, the pros and cons of different imaging techniques (computer tomography [CT], magnetic resonance imaging and [18F]fluorodeoxyglucose-positron emission tomography-CT), as well as how renal vein sampling (RVC) may contribute to localisation of the tumour.

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Renal vein thrombosis (RVT) is a common complication of nephrotic syndrome and renal malignancy. However, its association with oral contraceptive use has rarely been reported. We report a case of a 29-year-old female with a history of oral contraceptive use, presenting with acute flank pain. On further investigation, she was found to have unilateral RVT. Oral contraception was discontinued, and she was started on therapeutic anticoagulation, initially with low-molecular-weight heparin, and then switched to apixaban. Her symptoms improved, and she is currently doing well. This case signifies the importance of proper history-taking and how oral contraception should be considered a significant risk factor for venous thromboembolism.

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Key Clinical Message: Because of the complex embryonic origin of the abdominal venous structures, IVC and azygous systems can show numerous and even previously unreported anatomical variations and anomalies. Also, evaluating major vascular structures should not be dismissed in non-contrast-enhanced CT as it can provide valuable information about these structures. Abstract: Double IVC is a rare occurrence of IVC anatomical variations and congenital anomalies. Herein, we discuss a case of a very rare type of double IVC that has not been reported in the literature before. A non-contrast-enhanced CT study was performed for a 34-year-old patient who visited our ER to evaluate for urolithiasis, during which two IVCs were noted. Each renal vein joined the ipsilateral IVC at a perpendicular angle. Unusually, the right IVC was formed from the confluence of both left and right common iliac veins (CIV), and the left IVC-Instead of crossing the midline at the renal veins level and reuniting the right IVC-cranially contributed to the azygos vein formation and caudally joined the left CIV. Also, there were some small communicating veins between the two IVCs and the left gonadal vein was slightly dilated before suggesting a reflux from the left renal vein (LRV). A complimentary doppler ultrasound exam confirmed the diagnosis and revealed a left-side varicocele. Although rare cases of hemiazygos continuation and interiliac connections of left-side IVC in the cases of double-IVC have been reported previously, a complete confluence of CIVs is rare. The main differential diagnosis is retro-aortic left renal vein (RLRV) type IV which seems to have an oblique course. Radiologists and surgeons should expect previously unreported variations in the vena cava system. Furthermore, reviewing the main abdominal vasculature should not be dismissed in non-contrast CT exams.