Orthostatic proteinuria due to inferior vena cava interruption without nutcracker phenomenon in an old obese female: a case report and literature review.

BACKGROUND: Nutcracker syndrome (NCS) caused by left renal vein (LRV) entrapment, is one of the most common causes of orthostatic proteinuria. In stereotype, orthostatic proteinuria is often accompanied by left renal vein obstruction and is found in young and underweight individuals. Here, we report a rare case with orthostatic proteinuria in an old obese female caused by a rare type of congenital inferior vena cava (IVC) interruption. CASE PRESENTATION: A 65-year-old obese woman, who suffered from fluctuated proteinuria, had been misdiagnosed as chronic glomerulitis for 30 years. Instead of having any sign of NCS, she had a unique type of IVC interruption. Most venous blood from infrarenal IVC and right kidney drained into her LRV, and then through the expanded communicating vessel, drained into the left ascending lumbar vein which extended as hemiazygos vein. To the best of our knowledge, this is one of the first cases reported of orthostatic proteinuria attributed to the subsequent hemodynamic irregularity caused by IVC interruption without nutcracker phenomenon. CONCLUSION: Adult-onset orthostatic proteinuria is relatively rare, hard to be recognized and could be misdiagnosed as chronic glomerulonephritis. The case provided a novel differential diagnostic condition for those who suffered from fluctuated proteinuria of unknown causes.

A unique case of extrarenal calyces and associated vascular variations in an adult female cadaver.

The following urogenital and vascular anomalies were observed in the left kidney of an 81-year-old female cadaver during routine dissection: three extrarenal calyces; an accessory renal artery originating directly from the abdominal aorta; and a circumaortic renal vein. The typical renal anatomical structures were identified, from anterior to posterior, as the renal vein, renal artery, and ureter appearing near the hilum of the left kidney. After closer examination, three extrarenal calyces were observed exiting from the hilum of the left kidney to form the pelvis, then narrowed and became the ureter which descended 21.5 cm to empty into the bladder. The accessory renal artery originated from the lateral aspect of the abdominal aorta 7.3 cm below the aortic origin of the left renal artery. A corresponding accessary renal vein, identified as a circumaortic vein, left the hilum 4.5 cm below the left renal vein and travelled posterior to the abdominal aorta to drain into the inferior vena cava. Extrarenal calyces are rare among urogenital tract variations. They can be associated with embryological abnormalities such as renal ectopia, horseshoe kidney or malrotation as well as clinical manifestations such as pelviureteric junction obstruction and hydronephrosis. Compression of the accessory renal artery can cause decreased blood flow to the inferior pole of the left kidney, thereby causing fibrosis, atrophy, or renal failure. The retro-aortic path of the circumaortic renal vein has been associated with posterior nutcracker phenomenon, haematuria, left renal vein thrombus formation, and renal vein hypertension. This unique combination of a collecting system anomaly and extrarenal vessel variations could have significant implications in abdominal surgery.

An Uncommon Variant of Nutcracker Syndrome Secondary to Left Renal Vein Compression Between the Right Renal Artery and The Proper Hepatic Artery.

Nutcracker syndrome refers to the compression of the left renal vein between the abdominal aorta and the superior mesenteric artery. The subsequent venous congestion of the left kidney, when symptomatic, could be associated with left flank pain, hematuria, varicocele, dyspareunia, dysmenorrhea, and proteinuria. Here we describe a 42-year-old female patient with simultaneous Dunbar syndrome and a rare variant of nutcracker syndrome in which the left renal vein (LRV) compression is secondary to the unusual path of the vein between the right renal artery and the proper hepatic artery. For both the nutcracker syndrome and the Dunbar syndrome, open approach by median mini-laparotomic access for transposition of LRV, and resection of the diaphragmatic pillars and arcuate ligament was attempted. During the intervention, due to anatomical issues, the LRV transposition was converted to endovascular stenting of the LRV, moreover the implanted stent was transfixed with an external non-absorbable suture to avoid migration. At the 12 months follow-up the patient was asymptomatic, and the duplex scan confirmed the patency of the celiac trunk without re-stenosis and a correct position of the LRV stent with no proximal or distal migration.

Triple Retroaortic Renal Vein With Tumor Thrombus.

A case report of a 51-year-old man with left renal tumor and level II vena cava tumor thrombus (thrombus extending >2 cm above the renal vein, but below the hepatic veins) in a rare anatomical variant of renal vein. In nonmetastatic patients, aggressive surgical resection is widely accepted as the standard management option, but some doubts about the best practice in these patients are relevant. Surgical approach on those patients is a challenge for the surgeons, and anatomical variants make the procedure even more difficulty. These patients should be referring to a tertiary center because of the potential perioperative complexity.

Endovascular management of a patient with massive renal arteriovenous fistula: challenges and tricks.

We describe the endovascular management of a patient with a massive renal arteriovenous fistula and a huge venous aneurysmal sac, who presented with features of cardiac failure and fever. The challenges faced and the outcomes are discussed with relevant literature review.

Hypertension Caused by Renal Arteriovenous Fistula with Multiple Renal Artery Aneurysms.

Renal arteriovenous fistula with renal artery aneurysms and dilated renal veins presents as an infrequent lesion. Endovascular therapy has recently been considered the first-line treatment for these conditions. We report a case of a patient with idiopathic renal arteriovenous fistula concomitant with multiple renal artery aneurysms that was successfully treated by the placement of a covered stent.

Coexistence of a rare type of ectopic kidney with atypical renal vasculature.

Knowledge of anatomical anomalies is significant for all specialists in clinical practice and may prevent serious complications following medical procedures. This report presents the rare crossed fused renal ectopia (CFRE) with atypical renal vasculature in cadaver of a 68-year-old man. The ectopic kidney was located on right side with four renal veins, three renal arteries, two ureters, where one of them is double. The embryological background, as well as the potential clinical significance of this morphological variation, is discussed. An interventional radiological and surgical procedure should be appropriately implemented to treat anomalies of vessels and CFRE.

Endovascular treatment of anterior nutcracker syndrome and pelvic varices in a patient with an anterior and a posterior renal vein.

There are few data on endovascular treatment of anterior nutcracker syndrome and pelvic varices in patients with anterior and posterior renal veins. Our objective is to report a case, identify occurrences and compare diagnosis and treatments. A 42-year-old woman presented with flank and pelvic pain and hematuria. She had anterior nutcracker syndrome and pelvic varices with an anterior and a posterior renal vein. A successful complete endovascular approach was done with stent implantation in the anterior renal vein and left gonadal vein embolisation. After 12-month follow-up, the patient remained asymptomatic with good results on CT. Only two case reports of patients with nutcracker syndrome with anterior and posterior renal veins were identified. In both, a self-expanding stent was implanted in the anterior renal vein. In conclusion, endovascular treatment represents a safe and successful option in patients with nutcracker syndrome and pelvic varices with an anterior and a posterior renal vein.

Renal transplantation using vascular conduit reconstruction in deceased kidneys with multiple renal arteries and short renal veins. / Trasplante renal mediante reconstrucción de conducto vascular en riñones de donantes fallecidos con arterias renales múltiples y venas renales cortas.

BACKGROUND: Transplantation of kidneys with vascular anatomical variants remains a challenge. Due to its varying success in regard to graft function after transplantation, these organs have been frequently discarded assuming in advance an unaffordable rate of vascular complications. PATIENTS AND METHODS: We performed three kidney transplants using organs from deceased donors harboring vascular variants (multiple arteries and short veins), including an unsplittable horseshoe kidney. Different grafts harvested from the same donor aorta, common iliac artery, and inferior vena cava, were used to reconstruct the initial vascular configuration by creating single arterial and venous conduits aimed to simplify the vascular anastomoses in the recipient. RESULTS: No post-operative complications were recorded. Warm ischemia times remained comparable to single artery renal allografts. No delayed graft function was noted in any case, and every patient regained normal renal function after transplantation. CONCLUSIONS: Vascular reconstruction using arterial and venous grafts harvested from the same deceased donor may result a helpful tool to simplify vascular anastomoses during transplantation surgery, thus avoiding their discard in advance, minimizing perioperative complications, and enabling normal graft function rates in the long-term follow-up. The successful outcome obtained by using this approach would help to expand the donor criteria for the inclusion of organs containing vascular anatomical variants.

Is a Retroaortic Vein a Risk Factor in Laparoscopic Living Donor Nephrectomy?

INTRODUCTION: In living donor transplantation choosing the right donor and donor side for laparoscopic donor nephrectomy is a challenging task in clinical practice. Knowledge about anomalies in renal blood supply are crucial to evaluate the feasibility of the operative procedure. Few data so far exist whether the existence of a retroaortic left renal vein has an impact on living kidney transplantation outcome for donor and recipient. MATERIALS AND METHODS: We retrospectively analyzed 221 patients who underwent laparoscopic living donor nephrectomy between 2011 and 2017 for existence of a retroaortic left renal vein. Clinical characteristics and operative outcomes for donors and recipients were analyzed. RESULTS: 221 patients underwent donor nephrectomy between 2011 and 2017; 11 patients (4.98%) showed the feature of a retroaortic left renal vein, and in 8 patients (72.7%) out of those 11 the left kidney was chosen for transplantation. Mean preoperative serum creatinine was 0.77 (0.49-0.98) mg/dL and 1.28 (0.97-1.64) mg/dL at discharge. In recipients mean serum creatinine preoperatively, after 1 week, 1 month,1 year, 2 and 3 years of follow-up was 10.36 (6.09-20.77) mg/dL, 1.71 (0.67-2.72), 1.33 (0.70-1.89), 1.31 (0.95-2.13), 1.31 (0.98-2.13) and 1.33 (1.03-1.84), respectively. Neither donors nor recipients suffered from any operative complications. CONCLUSIONS: Laparoscopic living donor nephrectomy of a left kidney with retroaortic renal vein is safe for the donor, without limitation in the outcome for the recipient.

A Case of Nutcracker Syndrome Combined with Wilkie Syndrome with Unusual Clinical Presentation.

BACKGROUND Nutcracker syndrome and Wilkie's syndrome are rare vascular diseases due to the abnormal course of the superior mesenteric artery originating from the abdominal aorta with reduced angle (<22°) and consequent compression of the left renal vein (nutcracker) and duodenum (Wilkie). Here, we report the case of a patient with a rare combination of these 2 syndromes and with unusual clinical manifestation of post-prandial pain. CASE REPORT We describe the case of a young male patient with rapid weight loss, coupled with post-prandial abdominal pain, with sub-acute onset, not associated with other symptoms. The ultrasound examination found an aorto-mesenteric angle of 18° and compression of the left renal vein and left varicocele. A CT study was performed to exclude oncological diseases and/or other pathologies responsible for the pain and weight loss, which confirmed the ultrasound findings and showed compression of the third part of the duodenum. The patient underwent endovascular treatment, with stent placement in the left renal vein, which resolved the vascular compression and of the duodenum, with regression of symptoms. CONCLUSIONS The ultrasound scan promptly highlighted the reduction of the aorto-mesenteric angle and the signs of venous congestion of the left renal vein. Based on this experience, in patients with weight loss and post-prandial pain, in our opinion, diagnostic investigations should also be extended to the study of the aorto-mesenteric angle to confirm or exclude any vascular and/or duodenal compression.

Case Report of a Pelvic Crossed Fused Renal Ectopic Kidney.

Herein we present a case of a horseshoe kidney with crossed fused renal ectopia. Both of these pathologies are congenital anomalies; however, to date, there are few cases that present with both. In this case, discovered during routine dissection, the fused kidney was mostly left-sided and very low in the pelvis. No renal artery arose from the right wall of the abdominal aorta, and the right renal vein drained into the lower part of the inferior vena cava (IVC) where the right and left common iliac veins joined. It is essential for clinicians and surgeons to understand these types of congenital anomalies, as they could impact patient care.

Robotic assisted extravascular stent placement for nutcracker phenomenon of the left renal vein: a case series.

Nutcracker phenomenon of the left renal vein is a rare anatomic anomaly that can present with chronic flank/pelvic pain, pelvic congestion, and hematuria. Conventional treatment options (superior mesenteric artery transposition, endovascular stent placement, auto-transplantation) involve substantial risk, morbidity, or the need for chronic anti-coagulation. We now report our institution's robotic experience with extravascular left renal vein stent placement. A retrospective, single surgeon series from December 2016 to May 2019 was reviewed. After positioning and port placement (three robotic ports, one assistant), the left renal vein was exposed and dissected free circumferentially down to the inferior vena cava insertion. The distance between the renal vein ostium and adrenal vein was measured and a 1 cm-diameter ringed polytetrafluoroethylene vascular stent of this length placed. The stent edges were secured to itself with 3-0 polyglactin sutures. Demographics, surgical, and functional outcomes were collected. Six patients with mean age of 45 ± 6 years and body mass index of 20.3 ± 3.3 g underwent the procedure. Mean operative time was 143 ± 20 min. Estimated blood loss was minimal. Mean graft length utilized was 2.25 ± 0.3 cm. Median day of discharge was 1.5 days (range 1-3). No high-grade complications occurred. All patients received immediate pain relief and 50% also saw other symptomatic improvements. Robotic assisted extravascular left renal vein stent placement appears safe and effective in a small cohort with short follow-up. Further long-term follow-up for pain relief and graft-related complications are needed.

RENAL VEIN THROMBOSIS DUE TO POSTERIOR NUTCRACKER SYNDROME.

Thrombosis of the left renal vein (LRV) is a rare occurrence usually associated with hypercoagulable disorders. It may also be caused by extrinsic compression due to anatomical variations. Such variations, known as nutcracker phenomenon (NP), are usually completely asymptomatic, and they have been described in three variants: a narrowed aortomesenteric angle (AMA) with entrapment of the LRV; a second, rarer variant with an anomalous retro aortic LRV compressed between the abdominal aorta (AA) and the spine (posterior nutcracker syndrome, PNCS); and a third variant with a duplicated LRV with a pre- and retroaortic course, compressed both anteriorly and posteriorly. The development of symptoms secondary to NP is mainly due to renal congestion, often difficult to identify, and includes flank pain, ipsilateral varicocele, hematuria, and orthostatic proteinuria, among others. The most severe complication of NCS is LRV thrombosis with or without associated predisposing prothrombotic factors. The present case associates a PNCS to LRV thrombosis with a floating thrombus in the inferior vena cava and pulmonary embolism.

Laparoscopic high anterior resection for rectal cancer with rare variations of the left renal vessels: A case report.

We present a case of rectal cancer with rare variations of the left renal vessels. A man in his 60s underwent endoscopic mucosal resection for an Ip-type lesion in the upper rectum. Histologically, the lesion was a well-differentiated adenocarcinoma that had invaded the deep submucosal layer. Therefore, additional resection of the rectum with regional lymph node dissection was recommended. Preoperative CT revealed rare variations of the left renal vessels. There were two left renal arteries and veins; the caudal left renal artery and vein were located between the inferior mesenteric artery and the abdominal aorta. During the operation, these renal vessels were confirmed, and laparoscopic high anterior resection was performed safely without any injury to these renal vessels. To avoid the risk of unexpected intraoperative injuries, it is important to preoperatively check whether there are any variations in the renal vessels, even before colorectal surgery.

Concomitant multiple anomalies of renal vessels and collecting system.

Although anomalies of renal vessels and collecting system are relatively frequent, their concomitant occurrence is a rare event. During dissection of a 75-year-old male formalin-embalmed cadaver, we found multiple variations in the renal vessels and renal collecting system. Both kidneys were normal in size and anteriorly malrotated, with duplex collecting system and duplex ureter. One ureter drained the upper part of the kidney and the second ureter drained the lower part of the kidney. Superior and inferior collecting systems were separated by renal parenchyma. The right kidney had two renal arteries, the first renal artery (main renal artery) originating from the abdominal aorta, passing behind the inferior vena cava (IVC) and entering the kidney through the superior and inferior renal hilum. The second artery was the inferior polar artery. In addition, the right kidney had two renal veins as well. Three renal tributaries emerged from the upper and lower portion of the right renal hilum, and they joined to form the main renal vein which drained into the IVC. The lower renal vein was the inferior polar vein. The left kidney had four renal arteries (two hilar arteries and two polar arteries). The main left renal vein emerged from both superior and inferior left renal hilum, passed in front of the abdominal aorta and drained into the IVC. The left kidney also had the inferior polar vein which was divided behind the aorta (retro aortic vein) into two venous trunks. These venous trunks drained separately into posteromedial aspect of the IVC. Finally, the right testicular vein was formed by two tributaries and drained into the IVC, whereas the two left testicular veins drained separately into the left main renal vein.

A Case of Right Renal Arteriovenous Malformation Mimicking the Symptoms of Urinary Tract Infection in the Emergency Department.

BACKGROUND: Renal arteriovenous malformation (AVM) is a rare cause of massive hematuria, and patients with renal AVM may present with symptoms like urinary tract infections in the emergency department. CASE REPORT: A 37-year-old woman presented to the emergency department with symptoms of hematuria, urinary hesitancy, and severe suprapubic pain that had been present for a few hours. A urine examination revealed no pyuria, but urine occult blood for 3+ and a red blood cell count of >100 per high-power field. Bedside echocardiography revealed right kidney hydronephrosis and a distended bladder with a blood clot. A 3-way Foley catheter was inserted and drained 800 mL of bloody urine. A contrast-enhanced computed tomography scan was ordered that showed a 1.1-cm hypervascular tumor in the lower pole of right kidney, with active bleeding and rupture into the adjacent collecting system. Active renal tumor bleeding or renal AVM was suspected. The patient was transferred to a tertiary medical center where right renal artery angiography was arranged and disclosed an AVM with aneurysm formation at the right renal lower pole. Transarterial embolization was performed immediately to embolize the 3 feeders of the AVM. WHY SHOULD EMERGENCY PHYSICIANS BE AWARE OF THIS?: Renal AVM is a rare but potentially life-threatening cause of massive hematuria. Delayed or missed diagnosis is possible because renal AVM may present with symptoms like urinary tract infection, especially in young females. Renal artery angiography is the diagnosis of choice, and emergent transarterial embolization is now the standard of treatment.

Anatomical variants of renal veins: A meta-analysis of prevalence.

The main aim of this article is to establish the actual prevalence of renal vein variations (circumaortic renal vein, retroaortic renal vein, double renal vein), and to increase awareness about them. To this purpose, we have performed a meta-analysis of prevalence, using the MetaXL package, We included 105 articles in the final analysis of prevalence, of which 88 contained data about retroaortic renal vein, 84 - about circumaortic renal vein, and 51 - about multiple renal veins. The overall prevalence for retroaortic renal vein was 3% (CI:2.4-3.6%), for circumaortic renal vein - 3.5% (CI:2.8-4.4%), and for multiple renal veins - 16.7% (14.3-19.2%), much higher on the right 16.6 (14.2-19.1%) than on the left side 2.1 (1.3-3.2%). The results were relatively homogenous between studies, with only a minor publication bias overall.