Novel Use of Time-Resolved Magnetic Resonance Angiography to Evaluate Retrograde Lumbar Vein Flow and Epidural Venous Plexus Enhancement in Chronic Headache Patients With and Without Nutcracker Physiology.

OBJECTIVE: Compression of the left renal vein by the superior mesenteric artery, known as nutcracker phenomenon (NCP), can cause retrograde flow and congestion in communicating venous systems. It has recently been speculated that NCP can result in retrograde flow and congestion of the lumbar veins and epidural venous plexus (EVP), thereby affecting the central nervous system. This study describes the novel use of time-resolved magnetic resonance angiography (trMRA) to evaluate for retrograde left second lumbar vein (L2LV) flow and early EVP enhancement in patients with chronic daily headache (CDH) with and without NCP. METHODS: A retrospective analysis was performed of 31 patients with CDH (27 females and 4 males; median age, 38 years [range, 18-63 years]) who underwent trMRA centered over the L2LV to evaluate the direction of blood flow and presence of early EVP enhancement from May 2020 to March 2022. Descriptive statistics were performed, and anatomic associations were analyzed in patients with and without retrograde L2LV flow and early EVP enhancement. The accuracy of magnetic resonance imaging findings in detecting these flow patterns was also assessed. RESULTS: Patients with NCP who demonstrated narrowing of the left renal vein, a positive beak sign ( P = 0.052), decreased aortomesenteric distance ( P = 0.038), and decreased SMA angle demonstrated increased rates of retrograde L2LV flow and early EVP enhancement. A positive beak sign was 83% specific, and an aortomesenteric distance of ≤6.5 mm was 61% sensitive and 83% specific for identifying retrograde L2LV flow with early regional EVP enhancement in patients with CDH. CONCLUSIONS: Retrograde L2LV flow with early EVP enhancement in CDH patients can be effectively evaluated using trMRA and was seen with greater propensity in those patients with NCP.

Two Peculiarities of the Nutcracker Syndrome: Rare Association with Multiples Thrombosis and Successful Medical Treatment (Pentoxifylline).

The nutcracker syndrome (NCS) is a rare condition characterized by the entrapment of the left renal vein between the superior mesenteric artery and the aorta. Clinically, it presents with hematuria, flank pain, and symptoms of pelvic venous congestion. It is more frequent in females. Conservative treatment is usually proposed children but in adults, NCS has to be treated by surgical or endovascular methods. Drug therapy is not proposed for the treatment of NCS. We report a case of a young patient who presented with recurrent gross hematuria. Our patient was treated with pentoxifylline three times daily and anti-coagulation, with a favorable outcome.

Los puntos oscuros de la insuficiencia venosa pélvica / The dark spots of pelvic venous insufficiency

La insuficiencia venosa pélvica es un tema de mucho debate en congresos de nuestro ámbito profesional. Sin embargo, cuanto más se reconoce y se trata esta patología en nuestro medio, más casos encontramos en los que, aplicando un tratamiento estándar de embolización venosa o stenting, se obtiene un resultado desfavorable. Para evitar este patrón de resultados, debemos estudiar con detenimiento la circulación venosa abdominopélvica estableciendo paralelismos con la circulación venosa infrainguinal y, además, aplicar tecnología IVUS en los casos de duda. Con estas dos medidas, podemos afrontar esta patología en su conjunto con unos resultados muy positivos para nuestros pacientes

Pelvic Venous Insufficiency is a topic of much debate in Congresses of our professional field. However, the more this pathology is recognized and treated in our setting, the more cases we find in which applying a standard venous embolization or stenting, treatment results in an unfavorable result. To avoid this type of results, we must carefully study the abdomino-pelvic venous circulation, establishing parallels with the infrainguinal venous circulation, and also apply IVUS technology in cases of doubt. With these two measures, we can face this pathology as a whole with very positive results for our patients

Localized solitary left renal vein thrombus complicating nutcracker syndrome: a case report and review of the literature.

Nutcracker syndrome (NCS) refers to compression of the left renal vein (LRV) between the abdominal aorta and the superior mesenteric artery (SMA). The clinical presentation of NCS includes hematuria, abdominal and left flank pain, gonadal varices, and varicocele formation. Theoretically, thrombosis can occur in the LRV in patients with NCS. However, an isolated solitary left renal vein thrombus (LRVT) complicating NCS is rare. In addition, the clinical features of an LRVT complicating NCS remain unclear. We describe a 43-year-old woman presenting with an asymptomatic LRVT complicating NCS. She was referred to our hospital for investigation of dysfunctional uterine bleeding, and detailed examination revealed endometrial cancer. Computed tomography angiography (CTA) and Doppler ultrasonography revealed compression of the LRV between the aorta and the SMA, as well as an LRVT. CTA performed 4 months after the administration of an anticoagulant showed complete disappearance of the LRVT. We have also included a review of published reports describing LRVT complicating NCS and discussed the clinical features of such a presentation.

[Nutcracker syndrome]. / Síndrome del cascanueces.

Nutcracker syndrome is a vascular anomaly consisting in the compression of the left renal vein between the superior mesenteric artery and the aorta. Clinical features in nutcracker syndrome include pelvic pain, flank pain, haematuria, gonadal varices or simply asymptomatic. We are presenting two cases, one of them with macroscopic haematuria and flank pain and the other was studied for hypertension but with previous antecedents of left renal vein embolization in the setting of varicocele. We discuss the clinical presentation as well as diagnostic and therapeutic aspects related to this syndrome.

El síndrome del cascanueces es una anomalía vascular en la que se comprime la vena renal izquierda a su paso entre la unión de la aorta y la arteria mesentérica superior, debido a un ángulo muy cerrado entre ambas arterias. Clínicamente puede presentarse como hematuria macro o microscópica, dolor episódico en flanco izquierdo, dolor pélvico, várices gonadales o simplemente cursar de forma asintomática. Presentamos dos casos clínicos, uno de ellos con dolor abdominal tipo cólico nefrítico y hematuria macroscópica y otro estudiado por hipertensión pero con antecedentes de embolización de la vena renal izquierda por varicocele izquierdo. Discutimos los aspectos clínicos, diagnósticos y terapéuticos de este síndrome.

Síndrome del cascanueces / Nutcracker syndrome

El síndrome del cascanueces es una anomalía vascular en la que se comprime la vena renal izquierda a su paso entre la unión de la aorta y la arteria mesentérica superior, debido a un ángulo muy cerrado entre ambas arterias. Clínicamente puede presentarse como hematuria macro o microscópica, dolor episódico en flanco izquierdo, dolor pélvico, várices gonadales o simplemente cursar de forma asintomática. Presentamos dos casos clínicos, uno de ellos con dolor abdominal tipo cólico nefrítico y hematuria macroscópica y otro estudiado por hipertensión pero con antecedentes de embolización de la vena renal izquierda por varicocele izquierdo. Discutimos los aspectos clínicos, diagnósticos y terapéuticos de este síndrome.

Nutcracker syndrome is a vascular anomaly consisting in the compression of the left renal vein between the superior mesenteric artery and the aorta. Clinical features in nutcracker syndrome include pelvic pain, flank pain, haematuria, gonadal varices or simply asymptomatic. We are presenting two cases, one of them with macroscopic haematuria and flank pain and the other was studied for hypertension but with previous antecedents of left renal vein embolization in the setting of varicocele. We discuss the clinical presentation as well as diagnostic and therapeutic aspects related to this syndrome.

Evaluation of posterior nutcracker phenomenon using multisection spiral CT.

AIM: To prospectively evaluate the epidemiological, anatomical classification, clinical importance, and radiological features of posterior nutcracker phenomenon (PNP) in patients examined with multisection spiral computed tomography (MSCT). MATERIAL AND METHODS: Patients who underwent abdominal enhanced MSCT from January 2012 to July 2016 were evaluated retrospectively. Combined with the blood, normal biochemistry, and urine tests, patients were assessed retrospectively for renal vein diameter measurements, the compression ratio of blood vessels, gender differences, anatomical classification, percentage of proteinuria and haematuria, and clinical symptoms. RESULTS: The study included 6,225 consecutive patients, PNP was observed in 2.06% involving the CLRV in 1.22% and retro-aortic left renal vein in 0.84%. The proportion of posterior nutcracker syndrome (PNS) was lower in PNP (17.2%). There was no difference by sex and age in the prevalence of PNP (p>0.05). In patients with urological symptoms, the number of type II abnormalities was more frequent than type Ia and type Ib abnormalities. Meanwhile, the number of type II abnormalities was frequent than the other two types. There was a significant difference between PNS and asymptomatic PNP (aPNP) in all measured values (p<0.001), the ratio was >4, especially in type II abnormalities (ratio >5.0). CONCLUSIONS: PNP is a type of left renal vein entrapment. It is exceptionally rare and might be underdiagnosed. MSCT and measured values can be useful in diagnosis and recognition.

Incidental Anatomic Finding of Celiacomesenteric Trunk Associated with 'Nutcracker Phenomenon,' or Compression of the Left Renal Vein.

BACKGROUND Celiacomesenteric trunk (CMT) is a very rare anatomic finding in which the celiac artery and the superior mesenteric artery (SMA) originate from the abdominal aorta through a common trunk. Clinical associations with CMT include arterial aneurysm, thrombosis, and celiac artery compression. However, an association between CMT and abdominal venous congestion caused by left renal vein compression, or 'nutcracker phenomenon,' has not been previously reported. CASE REPORT A 91-year-old woman, who died from a cerebrovascular accident (CVA), underwent a cadaveric examination at our medical school. On examination of the abdomen, there was an incidental finding of CMT. The arterial and venous diameters were measured, and vascular histopathology was undertaken. The vascular anatomy was consistent with CMT type 1-b. Nutcracker phenomenon (NCP) (left renal vein compression) was seen anatomically as dilatation and engorgement of the left renal vein, relative to the right renal vein (10.77±0.13 mm vs. 4.49±0.56 mm, respectively), and dilatation and engorgement of the left ovarian vein, relative to the right ovarian vein (4.37±0.15 mm vs. 1.06±0.09 mm, respectively) with left ovarian varicocele. The aortoceliac angle (ACA) and the aortomesenteric angle (AMA) approached zero degrees. CONCLUSIONS We have described a rare anatomic finding of CMT that created an acute AMA and NCP. Awareness of this rare association between CMT and NCP by clinicians, vascular surgeons, and radiologists may be of value in the future evaluation and surgical management of patients who present clinically with 'nutcracker syndrome.'

Caso 15. Angina intestinal de causa no ateroesclerótica en mujer joven / Case 15. Non-atherosclerotic intestinal angina in a young woman

No disponible

What Each Clinical Anatomist Has to Know about Left Renal Vein Entrapment Syndrome (Nutcracker Syndrome): A Review of the Most Important Findings.

Nutcracker syndrome (NCS) is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. The development of NCS is associated with the formation of the left renal vein (LRV) from the aortic collar during the sixth to eighth week of gestation and abnormal angulation of the superior mesenteric artery from the aorta. Collateralization of venous circulation is the most significant effect of NCS. It includes mainly the left gonadal vein and the communicating lumbar vein. Undiagnosed NCS may affect retroperitoneal surgery and other radiological and vascular procedures. The clinical symptoms of NCS may generally be described as renal presentation when symptoms like haematuria, left flank pain, and proteinuria occur, but urologic presentation is also possible. Radiological methods of confirming NCS include Doppler ultrasonography as a primary test, retrograde venography, which can measure the renocaval pressure gradient, computed tomography angiography, which is faster and less traumatic, intravascular ultrasound, and magnetic resonance angiography. Treatment can be conservative or surgical, depending on the severity of symptoms and degree of LRV occlusion. Nutcracker syndrome is worth considering especially in differential diagnosis of haematuria of unknown origin.

[Nutcracker Syndrome with Left Inferior Vena Cava : A Case Report].

A 37-year-old male with a history of intermittent gross hematuria visited our hospital with asymptomatic macrohematuria. Cystoscopy findings revealed bloody urine from the left ureteric orifice and the cytodiagnosiswasclas sII. Contrast-enhanced computed tomography imaging revealed the inferior vena cava (IVC) running upward along the left side of the aorta, then crossing the aorta between the aorta and superior mesenteric artery (SMA). The crossing portion of the IVC was severely compressed by the SMA, causing the left-sided IVC (LIVC), and two left renal veins (LRV) were entered at the dilated portion of LIVC. On suspicion of nutcracker syndrome (NCS) with LIVC, color doppler ultrasonography and venography examinationswere performed. Although the resultsdid not meet criteria reported for NCS, venography revealed reflux from the IVC to the upper branch of the left renal vein. LIVC is a rare vessel anomaly, with a rate of incidence ranging from 0.2-0.5%, while NCS with LIVC isextremely rare, with only 7 case reports in the English literature. Herein. we report the first known case in Japan and review previous reports.

The roles of the liver and pancreas in renal nutcracker syndrome.

INTRODUCTION: To assess the frequency and significance of presence of the liver and pancreas at the left renal vein (LRV) level in patients with suspected renal nutcracker syndrome (NCS). MATERIALS AND METHODS: We included 101 patients with hematuria who underwent urography three-dimensional CT between April 2009 and November 2013. These patients were divided into NCS (n=25) and non-NCS (n=76) patients according to the following CT criteria: (1) the presence of beak sign and (2) hilar-aortomesenteric left renal vein diameter ratio >4. Patients were grouped according to the presence of the liver and pancreas at the LRV: group LP (both liver and pancreas), group L (only liver), group P (only pancreas), and group O (neither liver nor pancreas). The difference in the frequencies of groups was analyzed between NCS and non-NCS patients. Multivariate analysis was used to determine the independent factors between NCS and non-NCS patients. RESULTS: The frequencies of group LP, group L, group P, and group O in NCS vs. non-NCS were 88% vs. 5.3% (p<0.001), 4.0% vs. 2.6% (p=0.75), 4.0% vs. 11.8% (p=0.45), 4.0% vs. 80.3% (p<0.001), respectively. Multivariate analysis demonstrated that group was a predictor for differential diagnosis between NCS and non-NCS (p=0.022), and group LP was an independent factor for the presence of NCS (odds ratio, 43.8; 95% confidence interval, 3.8-500.3; p<0.002; reference, group O). CONCLUSION: The presence of the liver and pancreas at the level of the LRV was frequently found in NCS and was the independent factor for NCS.

Left crossed fused renal ectopia L-shaped kidney type, with double nutcracker syndrome (anterior and posterior).

Crossed fused renal ectopia (CFRE) is the second most common fusion anomalies (FAs) of the kidneys after horseshoe kidney. Crossed fused renal ectopia (CFRE) results from one kidney crossing over to the opposite side and subsequent fusion of the parenchyma of the two kidneys. We report, by multidetector-row computed tomography (MDCT) angiography, an extremely rare case of a left CFRE (L-shaped kidney type), consisting of multiple renal arteries (one main renal artery for the upper renal parenchyma, and three renal arteries (one main and two additional) for the lower renal parenchyma) and two left renal veins, which produced a double nutcracker syndrome (both anterior and posterior). The L-shaped left kidney has a maximum length of 18.5 cm, a maximum width of 10.2 cm, and a maximum thickness of 5.3 cm. The upper pole of the kidney is located at the level of the lower third of T12 vertebral body (4.6 cm left to the mediosagittal plan); the lower pole is located along the lower half of the L5 vertebral body (1.5 cm left to the mediosagittal plan). The following case will focus on the relevant anatomy, embryology, and the clinical significance of this entity.

A therapeutic option in nutcracker syndrome and ovarian vein insufficiency.

OBJECTIVES: The nutcracker syndrome (NS) may lead to insufficient perirenal collaterals as well as incompetence of the left ovarian vein with consecutive ovarian vein insufficiency. METHODS: A female patient with NS and severe genital varicosis was treated with dilation of the renal vein and coiling of a left perirenal collateral vein feeding an insufficient left ovarian vein (LOV) with pelvic vein varicosity. RESULTS: In re-evaluation 18 month later with left renal vein (LRV) and LOV phlebography, a widely patent LRV was found. The embolized LRV to LOV collateral was occluded. However, left hypogastric phlebography showed incompetent branches of the left hypogastric vein feeding the genital varicose veins. These were successfully embolized with coils and the genital varicosity decreased on follow-up. CONCLUSIONS: In our patient a combined therapeutic approach with balloon dilation of the NS and embolization of the genital varicose veins by left hypogastric vein coil was performed.

Incidental finding of the nutcracker phenomenon detected by 18F-FDG PET/CT.

A 46-year-old man was found to have an increased carcinoembryonic antigen level (25.7 µg/L) during physical examination, and an (18)F-FDG PET/CT scan was subsequently performed. The PET image indicated abnormal radioactivity levels in the left kidney, and the coregistered CT revealed compression of the left renal vein between the aorta and superior mesenteric artery. An ultrasonography was then performed, which revealed that the ratio between the renal hilum and the aortomesenteric left renal vein was 3.2. Therefore, the nutcracker phenomenon was diagnosed. When abnormal radioactivity is found in the left kidney on PET/CT scanning, the nutcracker syndrome should be considered.

Treatment of nutcracker syndrome with spermatic vein ligation and iliac vein anastomosis: case report of three cases.

Three patients with nutcracker syndrome (NCS) were enrolled in the study. Their clinical symptoms mainly included proteinuria and serious varicocele. Color Doppler ultrasound and computed tomography showed that the patients had constricted abdominal aortas, reduced superior mesenteric artery angles, and thinned left renal veins. Patients then underwent left spermatic vein ligation and iliac vein anastomosis. They were checked three months after their operation, and results showed that their sperms were improved and their proteinuria disappeared. The color Doppler ultrasound showed new cycle pathways. NCS clinical manifestations are complicated, and need combination diagnosis of symptoms and auxiliary examination to determine surgical indications. Ligation of spermatic vein and vein anastomosis can be used to treat NCS.