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Long-term indwelling central venous catheters (CVCs) may cause complications, such as central venous disease (CVD). CVD eliminates the possibility of establishing new arteriovenous access. We herein describe a case in which an arteriovenous graft (AVG) is implanted through the peripheral veins and blood flows back to the patent azygos vein, secondary to superior vena cava stenosis. This shows that if the CVD is correctable or the collateral circulation is an alternative vascular pathway that allows blood to return back to the right atrium, the internal fistula flow is well-balanced with the collateral circulation and there are no clinical symptoms of CVD, an arteriovenous access can be established for the operation. This provides a viable, long-term vascular access option for hemodialysis patients with central venous resource depletion., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Competing Interests: Competing interests: None declared.

We report a unique case of unusual drainage of the bifurcated retroaortic left renal vein, with the cranial wider branch draining into a dilated lumbar azygos vein and caudal thinner branch connecting with the inferior vena cava. The right renal vein was duplicated. The anomaly was discovered on multimodal 18 F-labeled fluorodeoxyglucose positron emission tomography/computed tomography performed for oncological purposes. The basis enabling occurrence of such variation was probably persistent developmental extra left-right venous connections, intercardinal, or intersupracardinal, depending on the theory. The embryology of the chest and abdominal veins is a complicated process and there is no unanimity concerning its concepts. The old models are currently being questioned and reevaluated. Knowledge of possible variants of renal and azygos veins course is important from clinical, imaging, and surgical points of view. The retroaortic left renal veins course may sometimes cause pain, hematuria, proteinuria, and pelvic congestion syndromes. Dilated parts of uncommonly located veins, because of assuming a nodular shape on transverse images, may be mistaken for abnormal lymph nodes, other tumors or aneurysms on imaging. During a variety of surgical procedures, including venous sampling, renal transplantation, or any retroperitoneal surgery, knowledge of an aberrant venous course may be important for the success of the procedure and may be crucial even earlier during the qualification process.

Congenital arteriovenous communications arising from subclavian artery are very rare. We report the first case of multiple congenital arteriovenous fistulas between the right subclavian artery and azygos vein presenting with congestive heart failure., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: Deep venous thrombosis causes blood flow deviation. It is hypothesized that with stent placement, developed collateral veins become redundant. This article evaluates the relation between the surface area of the collaterals and stent patency., Methods: The azygos and hemiazygos veins were identified and the largest surface area was measured at thoracic level. Patency rates of stented tracts were evaluated and related to collateral vein lumen size., Results: The vena cava occlusion and the azygos and hemiazygos vein surface area measurements were positive and statistically significant related (OR 1.01, 95% CI 1.003-1.019, p = 0.004) respectively (and OR 1.007, 95% CI 1.001-1.013, p = 0.004). An azygos surface area measurement of 23 (p<0.001) and hemiazygos surface area measurement of 40 (p = 0.008) was shown as cut-off point related to higher occlusion rates., Conclusions: The surface area of major venous collateral pathways seems to be related to stent occlusion in deep venous interventions.

During pediatric kidney transplant, surgical challenges occasionally occur. In particular, vascular anastomosis should be considered for children with small body weight < 12 kg, multiple renal arteries, vascular anomaly, and inferior vena cava occlusion. In pediatric patients, a living-donor renal graft is usually donated from a parent. Therefore, the renal artery and vein are too large to be anastomosed with the recipient's internal iliac artery and external iliac vein. In children who are > 12 kg, the renal artery and vein could be anastomosed with the external iliac artery and the external iliac vein. In children who are < 10 kg, the renal artery and vein should be anastomosed directly with the aorta and inferior vena cava. A pediatric transplant surgeon should consider arterial and venous anastomosis sites before transplant surgery. In small children with partial or total inferior vena cava occlusion, the venous anastomosis site should be evaluated. If the graft is placed on the left side, a venous graft must be used as a bridge between the renal vein and inferior vena cava. In 13 kidney transplants in children with inferior vena cava occlusion, 7 were on the left and 6 were on the right side. A patent segment of the inferior vena cava, the left original renal vein, an ascending lumbar vein, an azygos vein, the first graft renal vein, and a portal vein were used for venous anastomosis in 6, 2, 2, 1, 1 and 1 recipient, respectively. One child had graft loss due to renal vein thrombosis and one died of hemorrhage immediately posttransplant. Three had grafts with relatively long-term function, but these were lost due to chronic allograft nephropathy 100, 122, and 137 months posttransplant. However, the other 8 recipients have so far maintained graft function from 6 to 138 months since transplant.

The duplication of the inferior vena cava (IVC) is a rare congenital anomaly, which also has some variations regarding the complex embryological development of the IVC. In the typical form, infrarenal IVC segments are duplicated and the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion to join the right IVC. In variant forms, the interruption of the intrahepatic segment of the IVC, azygos or hemiazygos continuation, or retroaortic course of the renal vein may be seen. An intrahepatic venous shunt accompanying a double IVC variant is an extremely rare anomaly. We report a case of 40-year-old female patient with double IVC, hemiazygos continuation, intrahepatic IVC interruption, and a transhepatic venous shunt.

Objectives: To measure azygos, portal and aortic flow by two-dimensional cine phase-contrast magnetic resonance imaging (2D-cine PC MRI), and to compare the MRI values to hepatic venous pressure gradient (HVPG) measurements, in patients with cirrhosis., Methods: Sixty-nine patients with cirrhosis were prospectively included. All patients underwent HVPG measurements, upper gastrointestinal endoscopy and 2D-cine PC MRI measurements of azygos, portal and aortic blood flow. Univariate and multivariate regression analyses were used to evaluate the correlation between the blood flow and HVPG. The performance of 2D-cine PC MRI to diagnose severe portal hypertension (HVPG ≥ 16 mmHg) was determined by receiver operating characteristic curve (ROC) analysis, and area under the curves (AUC) were compared., Results: Azygos and aortic flow values were associated with HVPG in univariate linear regression model. Azygos flow (p < 10(-3)), aortic flow (p = 0.001), age (p = 0.001) and presence of varices (p < 10(-3)) were independently associated with HVPG. Azygos flow (AUC = 0.96 (95 % CI [0.91-1.00]) had significantly higher AUC than aortic (AUC = 0.64 (95 % CI [0.51-0.77]) or portal blood flow (AUC = 0.40 (95 % CI [0.25-0.54])., Conclusions: 2D-cine PC MRI is a promising technique to evaluate significant portal hypertension in patients with cirrhosis., Key Points: • Noninvasive HVPG assessment can be performed with MRI azygos flow. • Azygos MRI flow is an easy-to-measure marker to detect significant portal hypertension. • MRI flow is more specific that varice grade to detect portal hypertension.

Purpose: Superior vena cava (SVC) syndrome owing to benign etiology is rare and endovascular techniques have been advocated as the treatment of choice. We report a case of endovascular revascularization of a port catheter-associated complete occlusion of the SVC with reversed flow in the azygos vein., Methods: In this setting using a sheath in combination with its dilatator to pass the occlusion of the SVC after neither a diagnostic catheter nor a PTA balloon would pass the lesion may be a valid option. A dual venous approach was established using the right common femoral vein and an indwelling port catheter in the right cephalic vein to dilate and stent the lesion. Finally, a port may be implanted after the revascularization had been successful., Results: Passage through the port catheter-associated occlusion of the SVC was only possible by use of the sheath in combination with its dilatator. A dual venous access by the femoral approach and the indwelling central catheter is helpful in treating a SVC occlusion., Conclusions: Long-term central venous catheters may cause SVC syndrome, especially with a catheter tip located too far cranially. An endovascular revascularization of a complete occlusion of the SVC represents the therapy of choice.

Objectives: An almost universal incidence of developing pulmonary arteriovenous fistulas after the Kawashima operation has been reported. Exclusion of the hepatic venous flow from the pulmonary circulation causes the development of these malformations. Redirection of hepatic venous flow to the pulmonary circulation mostly leads to the regression of the arteriovenous fistulas., Methods: We analyzed 11 patients with arteriovenous fistulas that developed after the Kawashima operation. The hepatic-to-azygos shunts were performed with an off-pump technique through a lateral thoracotomy in all but one. Operative and postoperative data were retrospectively collected., Results: No intraoperative complications occurred, and no patient died in the hospital. Up to 10-year follow-up showed a significant postoperative improvement of patients' oxygen saturation and New York Heart Association class. Apart from 2 re-thoracotomies for bleeding in 1 patient, no complications occurred and no patient died during follow-up. Two other patients underwent reoperation for an undiagnosed additional hepatic vein. The improvement of patients' oxygen saturation and New York Heart Association class persisted during the follow-up period., Conclusions: The surgical connection can be performed safely with an off-pump technique that avoids the risks related to extracorporeal circulation and circulatory arrest. The results at 10 years follow-up confirmed the efficacy and safety of the surgical technique described., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to focus on patients suffering from cochleo-vestibular disorder with and without Ménière disease (MD) in order to verify whether chronic cerebrospinal drainage abnormalities could play a role in the etiopathogenesis of endolymphatic hydrops., Methods: Fifty-two volunteers were enrolled and subdivided into two groups: 24 definite MD and 28 not-MD. Both magnetic resonance venography imaging with contrast-enhanced imaging of the venous cerebrospinal system (MRV) and venous echo-color Doppler (ECD) were performed., Results: MRV showed abnormalities in 83% of MD and 57% of not-MD subjects (p < 0.001). Asymmetrical cervical venous flow, assessed by MRV, was confirmed by ECD in 62.5% of MD but in only 21.5% of not-MD subjects (p<0.001)., Conclusion: Chronic cerebrospinal venous insufficiency might be the anatomical background, which provides a predisposing factor for the development of endolymphatic hydrops in MD patients., (© The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Introduction: Chronic cerebrospinal venous insufficiency (CCSVI) is a vascular condition characterized by anomalies of the internal jugular veins (IJVs) and/or azygos veins with disturbed flow and formation of collateral venous channels. The presence of CCSVI has been associated with multiple sclerosis (MS). Percutaneous venous angioplasty (PVA) has been proposed to improve extracranial outflow; however, a non-invasive, post-procedural follow-up outcome measure has not been established., Aim of the Study: To evaluate the short-term hemodynamic follow-up of CCSVI after PVA using color Doppler ultrasound (CDU) and to investigate whether hemodynamic variation correlated with clinical variation., Materials and Methods: Forty-five patients affected by MS with confirmed CCSVI underwent IJVs PVA. Venous hemodynamic (VH) parameters indicative of CCSVI and the Venous Hemodynamic Insufficiency Severity Score (VHISS) were evaluated by CDU at baseline and 3 months post-PVA. Concurrently, the MS-related disability status (EDSS) was evaluated., Results: The VH parameters and VHISS 3 months after IJVs PVA significantly decreased: the VH parameters -32.1% and the VHISS -33.8% (p < 0.001). The EDSS score 3 months after IJVs PVA was significantly lower than the baseline (-5.5%, p < 0.001). Using the median value of the VHISS variation as the cut-off, we were able to identify two groups of patients: responders, group A; and non-responders, group B. The EDSS score variation at 3 months was 0.310 in group A and 0.275 in group B (p = 0.746)., Conclusions: CCSVI endovascular treatment can induce an improvement in VH parameters and the VHISS. The neurological disability score (EDSS) also improved after PVA; however, there was no correlation to the VHISS variation after PVA, MS type and duration., (© The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Aim: To investigate perioperative outcomes in patients undergoing modified laparoscopic splenectomy and azygoportal disconnection (MLSD) with intraoperative autologous cell salvage., Methods: We retrospectively evaluated outcomes in 79 patients admitted to the Clinical Medical College of Yangzhou University with cirrhosis, portal hypertensive bleeding and secondary hypersplenism who underwent MLSD without (n = 46) or with intraoperative cell salvage and autologous blood transfusion, including splenic blood and operative hemorrhage (n = 33), between February 2012 and January 2014. Their intraoperative and postoperative variables were compared. These variables mainly included: operation time; estimated intraoperative blood loss; volume of allogeneic blood transfused; visual analog scale for pain on the first postoperative day; time to first oral intake; initial passage of flatus and off-bed activity; perioperative hemoglobin (Hb) concentration; and red blood cell concentration., Results: There were no significant differences between the groups in terms of duration of surgery, estimated intraoperative blood loss and overall perioperative complication rate. In those receiving salvaged autologous blood, Hb concentration increased by an average of 11.2 ± 4.8 g/L (P < 0.05) from preoperative levels by the first postoperative day, but it had fallen by 9.8 ± 6.45 g/L (P < 0.05) in the group in which cell salvage was not used. Preoperative Hb was similar in the two groups (P > 0.05), but Hb on the first postoperative day was significantly higher in the autologous blood transfusion group (118.5 ± 15.8 g/L vs 102.7 ± 15.6 g/L, P < 0.05). The autologous blood transfusion group experienced significantly fewer postoperative days of temperature > 38.0°C (P < 0.05)., Conclusion: Intraoperative cell salvage during MLSD is feasible and safe and may become the gold standard for liver cirrhosis with portal hypertensive bleeding and hypersplenism.

Objective: To examine the incidence and distribution of extravascular compression of the extracranial venous pathway (the jugular and/or azygous veins) in multiple sclerosis patients with chronic cerebrospinal venous insufficiency evaluated by mulitislice computer tomographic angiography., Methods and Results: Study group consisted of 51 consecutive patients with multiple sclerosis in whom chronic cerebrospinal venous insufficiency was diagnosed by color Doppler sonography (CDS). Mulitislice computer tomographic angiography was performed in all patients, and it revealed significant extravascular compression (>70%) of extracranial venous pathway in 26 patients (51%), while in 25 patients (49%) no significant extravascular compression was seen. Extracranial compression due to transverse processus of cervical vertebrae was seen in 23 patients, carotid bulb compression was seen in two patients, and in one case, compression presented as a thoracic outlet syndrome., Conclusion: Our data indicate that extravascular compression of the extracranial venous pathway is frequent in multiple sclerosis patients with chronic cerebrospinal venous insufficiency, and that it is mainly due to compression caused by transverse processus of cervical vertebrae. Further studies are needed to evaluate potential clinical implications of this phenomenon., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Aim: To investigate perioperative outcomes in patients undergoing modified laparoscopic splenectomy or open splenectomy and azygoportal disconnection for portal hypertension., Methods: This study included 44 patients who underwent modified laparoscopic splenectomy and azygoportal disconnection (MLSD) and 71 who underwent open procedures for portal hypertension. Blood samples were collected before surgery and on days 1, 3, and 7 after surgery. Markers of liver and renal function, C-reactive protein (CRP), interleukin-6 (IL-6), and procalcitonin (PCT) were measured, and perioperative variables were compared between the two groups., Results: The modified laparoscopic group showed significantly better and faster recovery, better liver and renal function, and fewer complications than the open group. CRP, IL-6, and PCT concentrations on postoperative days 1, 3, and 7 were significantly lower in the modified laparoscopic group than in the open group., Conclusion: MLSD was associated with lower inflammatory immune responses, less impairment of liver and renal function, and faster and better recovery.

We report the case of a 2.5-year-old patient with single-ventricle physiology who underwent cardiac magnetic resonance study for cyanosis after Kawashima operation. Magnetic resonance imaging study showed a veno-venous collateral redirecting systemic venous flow, responsible for cyanosis.

Objectives: Condition known as chronic cerebrospinal venous insufficiency (CCSVI) is characterized by insufficient cerebral vein drainage in patients with multiple sclerosis (MS) and internal jugular vein (IJV), vertebral and/or azygos veins stenoses. However, external compression on the IJV was not clearly described as a potential cause of CCSVI. We aim to present a case of CCSVI in a patient with MS caused by bilateral IJV inverted valves combined with IJV external compression by carotid bulb., Methods: A 31-year-old female patient was admitted to our institute for IJV and vertebral veins morphological and haemodynamical assessment after being treated for MS for the last 14 years. Colour Doppler ultrasonography showed right IJV prestenotic dilation and inverted valves in both IJV. Computerized tomography angiography showed bilateral IJV compression by carotid bulb. Haemodynamical Doppler parameters showed that external IJV compression significantly contributed to CCSVI occurrence., Results: Bilateral IJV confluence percutaneous angioplasty (PTA) was done, and the patient was discharged for further neurological examination. Partial carbon dioxide pressure was significantly lower in the distal part of both IJV following PTA and oxygen saturation increased., Conclusion: In the case presented, PTA of the IJV confluence resulted in haemodynamic improvement despite the presence of IJV external compression.

We present the case of a 45-year-old man with univentricular heart, small outlet chamber, and L-transposition of the great vessels. As an infant, the patient underwent multiple palliative surgical interventions. He presented with worsening dyspnea and fatigue and was found to have systemic oxygen saturation of 85% on 2 L of oxygen by nasal cannula, whereas he had chronically remained between 90 and 95% throughout most of adulthood. There was no evidence of significant valvular regurgitation or stenosis, nor was there an overt intracardiac shunt by echocardiography. Cardiac CT and cardiac MRI revealed a large serpiginous systemic to pulmonary venovenous collateral located behind the left atrium. The collateral drained into the lower right pulmonary vein as it entered the left atrium. The tributary veins to the "giant" collateral were determined by these images modalities. The patient underwent a percutaneous embolization of this giant venovenous collateral via a remnant supracardinal vein originating from the infrarenal inferior vena cava using two Amplatzer Vascular Plug II. Immediately after the procedure the patient's oxygen saturation increased to 90% on room air at rest. At 2 months follow-up the patient had a marked clinical improvement with oxygen saturation as high as 95% on room air while walking. Our case illustrates a successful embolization of a giant collateral via an embryological venous remnant connecting the IVC to the azygos system., (Copyright © 2012 Wiley Periodicals, Inc.)

Introduction: Misplacement of extracorporeal membrane oxygenation (ECMO) venous cannula in the azygos vein has previously been described only in newborns., Case: For the first time, we report an aberrant ECMO cannula placement in the azygos vein in a child outside neonatal period. History of chronic lung disease and ligation of persistent ductus arteriosus leading to elevated right ventricular pressure was the potential risk factor., Conclusions: This rare complication should be considered whenever the patient has inadequate venous return on ECMO and it can be easily ruled out with a lateral chest radiograph.

Interruption of inferior vena cava (IVC) with azygos continuation is a rare venous anomaly, and arrhythmogenic IVC is also rarely reported. Arrhythmogenicity of the hepatic segment of IVC in interruption of IVC has never been reported. We describe the case of a 37-year-old female with interrupted left IVC with azygos continuation to right superior vena cava and atrial tachycardia originating from the hepatic segment of IVC.

Plastic bronchitis is a rare life-threatening complication of Fontan operation. When medical treatment is ineffective in the setting of high systemic venous pressures, Fontan fenestration may be considered to decompress venous pressures and improve cardiac output by creation of the right-to-left shunting. However, transcatheter approach can be difficult in patients with complex venous anatomy. We report a 4-year-old girl born with hypoplastic left ventricle and heterotaxy syndrome, who developed plastic bronchitis following extracardiac Fontan procedure. Her venous anatomy was complex with dextrocardia and interrupted inferior vena cava with azygos continuation. Stent fenestration was successfully performed via transhepatic approach, which was selected based on the anatomical relationship (between extracardiac conduit, left atrium, and hepatic veins) delineated by pre-catheterization cardiac MRI. Simultaneous transesophageal echocardiography guided the intervention. Her plastic bronchitis improved significantly in 3 months but slowly progressed after the stent fenestration. At her 8-month follow-up, stent fenestration remains open and she is currently under heart transplantation evaluation due to persistent plastic bronchitis. Treatment of plastic bronchitis can be undertaken with Fontan fenestration, with pre-procedural MRI playing an essential role in patients with complex venous anatomy., (Copyright © 2012 Wiley Periodicals, Inc.)

Purpose: We aimed to describe and compare azygos vein flow patterns of patients with obstructed and unobstructed systemic venous baffle after atrial switch repair for d-transposition of the great arteries (TGA). We hypothesized that phase-contrast magnetic resonance imaging would enable characterization of retrograde collateral flow across the azygos vein in cases of systemic venous baffle obstruction., Materials and Methods: This is a retrospective, cross-sectional study. Twelve patients with atrial switch repair for TGA were examined. Azygos flow index was measured with phase-contrast magnetic resonance imaging; comparison was made between patients with documented systemic venous baffle obstruction (n=3) and a control group of patients without baffle obstruction (n=9)., Results: Patients with systemic venous baffle obstruction had a distinctive azygos flow pattern that was retrograde and an increased amount of azygos flow compared with patients without obstruction [median (range), -436 (-455/-399) vs. 103 (51/125) mL/min/m2; P=0.01]., Conclusions: Patients with systemic venous baffle obstruction have a characteristic collateral flow across the azygos vein. Azygos vein flow measurement may be used for the assessment of baffle patency in patients after atrial switch repair for TGA. However, diagnostic accuracy needs to be tested in a larger population.

An open study was conducted with the aim of reporting long-term clinical outcome of endovascular treatment for chronic cerebrospinal venous insufficiency (CCSVI) in patients with multiple sclerosis (MS). Twenty-nine patients with clinically definite relapsing-remitting MS underwent percutaneous transluminal angioplasty for CCSVI, outside a clinical relapse. All the patients were regularly observed over at least two years before the first endovascular treatment and for at least two years after it (mean post-procedure follow up 30.6±6.1 months). The following clinical outcome measures were used: annual relapse rate and Expanded Disability Status Scale (EDSS) score. All the patients were observed intensively (mean 6 hours) on the day of the endovascular treatment to monitor for possible complications (bleeding, shock, heart attack, death). We compared the annual relapse rate before and after treatment (in the two years before and the two years after the first endovascular treatment) and the EDSS score recorded two years before versus two years after the treatment. Overall, 44 endovascular procedures were performed in the 29 patients, without complications. Thirteen of the 29 patients (45%) underwent more than one treatment session because of venous re-stenosis: 11 and two patients underwent two and three endovascular treatments respectively. The annual relapse rate of MS was significantly lower post-procedure (0.45±0.62 vs 0.76±0.99; p=0.021), although it increased in four patients. The EDSS score two years after treatment was significantly lower compared to the EDSS score recorded at the examination two years before treatment (1.98±0.92 vs 2.27±0.93; p=0.037), although it was higher in four patients. Endovascular treatment of concurrent CCSVI seems to be safe and repeatable and may reduce annual relapse rates and cumulative disability in patients with relapsing-remitting MS. Randomized controlled studies are needed to further assess the clinical effects of endovascular treatment of CCSVI in MS.

Congenital anomalies of the inferior vena cava (IVC) are rare, but recognized, causing deep venous thrombosis. We present a case of a 50-year-old patient with trauma who suffered an intracranial hemorrhage secondary to a fall while on anticoagulation for deep vein thromboses. Venous return from the lower extremities was determined to be through dilated lumbar venous collaterals into the azygous and hemiazygous systems. A second interesting anatomic finding was a hypoplastic left kidney.

Chronic cerebrospinal venous insufficiency (CCSVI) is a syndrome characterized by stenoses or obstructions of the internal jugular and/or azygos veins with disturbed flow and formation of collateral venous channels. Studies using ultrasound in patients with multiple sclerosis (MS) have demonstrated a high prevalence of CCSVI (mean 70%; range 0-100%; N.=1496), whereas, in normal controls and patients without MS the prevalence was much lower (mean 10%; range 0-36%; N.=635). Ultrasound uses a combination of physiological measurements as well as anatomical imaging and has been used for the detection of CCSVI by different centers with variable results. A high prevalence ranging from 62% to 100% of obstructive lesions has been found by some teams in patients with MS compared with a lower prevalence of 0-25% in controls. However, absence of such lesions or a lower prevalence (16-52%) has been reported by others. This variability could be the result of differences in technique, training, experience or criteria used. The current lack of a methodology shared among experts is a confounding element in epidemiologic studies, and does not permit further Bayesan or other kind of analysis. In order to ensure a high reproducibility of Duplex scanning with comparable accuracy between centers, a detailed protocol with standard methodology and criteria is proposed. This is also necessary for training. It has been shown that inter-rater variability increases post-training (from k=0.47 to k=0.80), while within-rater reproducibility in trained operators was k=0.75. Finally, the consensus document proposes a reporting standard of Duplex measurements, and future research to answer areas of uncertainty.

A 33 year old man presented to Accident and Emergency with haematuria and intermittent left loin pain radiating to the groin. On examination he had evidence of lower limb chronic venous insufficiency. Plain abdominal radiograph was unremarkable however computed tomography (CT) urogram and subsequent CT of thorax/abdomen/pelvis with contrast demonstrated azygous replacement of the inferior vena cava with an enlarged azygous vein and multiple enlarged veins in the abdomen and pelvis. A 5.7 cm by 4.2 cm low density lesion with surrounding inflammatory change was also visualised in the left para-aortic region. Ultrasound and magnetic resonance imaging confirmed this as a thrombosed collateral of the aberrant venous web drainage. Lifelong anti-coagulation was commenced with symptomatic relief.

Background: Congenital absence of the inferior vena cava (AIVC) is a rare vascular anomaly that may be associated with deep vein thrombosis (DVT). It is underreported and may be present in up to 5% of young patients with DVT. We report a unique case of simultaneous thrombosis of both superficial and deep veins in a patient with AIVC., Methods and Results: A 20-year-old man presented with a 2-week history of a swollen, painful, left lower limb. On examination, the left leg and thigh were found to be swollen and varicosities were present along the lower abdominal wall. Ultrasound showed extensive superficial and deep venous thrombosis of the entire left lower limb. Computed tomography venogram revealed an infrahepatic AIVC with lower limb drainage through enlarged intrathoracic continuations of the azygous and hemiazygous veins. The patient was put on oral anticoagulant therapy and was well at 6-month follow-up., Conclusion: The hypothesis for DVT in patients with AIVC is that venous drainage of the lower limbs is inadequate, leading to venous stasis and thrombosis. All young patients presenting with idiopathic DVT should be investigated for inferior vena cava anomalies with computed tomography if ultrasound does not visualize the inferior vena cava., (Copyright © 2011 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved.)

Objective: Pulmonary arteriovenous malformations are an important but uncommon complication of cavopulmonary connection, particularly in patients with heterotaxy. Absence of hepatic venous effluent in pulmonary arterial blood seems to be a predisposing factor. Pulmonary arteriovenous malformations are most common after superior cavopulmonary anastomosis, but may develop, progress, or persist in 1 lung after Fontan completion if hepatic venous blood streams completely or primarily to the contralateral lung., Methods: Among 53 patients with heterotaxy and inferior vena cava interruption who underwent a modified Fontan procedure from 1985 to 2005, 8 had unilateral streaming of hepatic venous flow and clinically significant pulmonary arteriovenous malformations after hepatic venous inclusion and underwent reconfiguration of the cavopulmonary pathway. In all 8 patients, the hepatic vein-pulmonary artery pathway was contralateral to and offset from the pulmonary artery anastomosis of the single or dominant superior vena cava. Pathway reconfiguration included pulmonary arterial stenting (n = 2), revision of the superior vena cava-pulmonary artery connection (n = 1), construction of a branched hepatic vein-pulmonary artery conduit (n = 2), and surgical or transcatheter construction of a direct hepatic vein-azygous vein pathway (n = 5)., Results: Hepatic vein-azygous vein connection led to improvement in 4 of 5 patients; other approaches typically did not lead to improvement., Conclusions: Resolution of hypoxemia after cavopulmonary pathway reconfiguration in patients with unilateral pulmonary arteriovenous malformations and hepatic venous flow-streaming after Fontan completion supports the importance of hepatic venous effluent in the pathogenesis of pulmonary arteriovenous malformations and the practice of cavopulmonary pathway revision in such patients. Completion or reconfiguration of the Fontan circulation with direct hepatic vein-azygous vein connection may provide the most reliable mixing and bilateral distribution of hepatic venous blood in this population of patients., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Partial anomalous pulmonary venous drainage (PAPVD) to the azygous vein and benign posterior mediastinal hemangioma in adults are both rare entities in isolation. The coexistence of these two lesions in the same patient has not been reported. We describe a unique case of PAPVD to the azygous vein in an adult woman, where the anomalous left inferior pulmonary vein transited first through a large hemangioma, and then eventuated in the azygous vein., (Copyright © 2011. Published by Elsevier Inc.)

Objective: Pulmonary arteriovenous malformations caused by abnormal hepatic flow distribution can develop in patients with a single ventricle with an interrupted inferior vena cava. However, preoperatively determining the hepatic baffle design that optimizes hepatic flow distribution is far from trivial. The current study combines virtual surgery and numeric simulations to identify potential surgical strategies for patients with an interrupted inferior vena cava., Methods: Five patients with an interrupted inferior vena cava and severe pulmonary arteriovenous malformations were enrolled. Their in vivo anatomies were reconstructed from magnetic resonance imaging (n = 4) and computed tomography (n = 1), and alternate virtual surgery options (intracardiac/extracardiac, Y-grafts, hepato-to-azygous shunts, and azygous-to-hepatic shunts) were generated for each. Hepatic flow distribution was assessed for all options using a fully validated computational flow solver., Results: For patients with a single superior vena cava (n = 3), intracardiac/extracardiac connections proved dangerous, because even a small left or right offset led to a highly preferential hepatic flow distribution to the associated lung. The best results were obtained with either a Y-graft spanning the Kawashima to split the flow or hepato-to-azygous shunts to promote mixing. For patients with bilateral superior vena cavae (n = 2), results depended on the balance between the left and right superior inflows. When those were equal, connecting the hepatic baffle between the superior vena cavae performed well, but other options should be pursued otherwise., Conclusions: This study demonstrates how virtual surgery environments can benefit the clinical community, especially for patients with a single ventricle with an interrupted inferior vena cava. Furthermore, the sensitivity of the optimal baffle design to the superior inflows underscores the need to characterize both preoperative anatomy and flows to identify the best option., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Chronic cerebrospinal venous insufficiency (CCSVI) is a putative new theory that has been suggested by some to have a direct causative relation with the symptomatology associated with multiple sclerosis (MS) [1]. The core foundation of this theory is that there is abnormal venous drainage from the brain due to outflow obstruction in the draining jugular vein and/or azygos veins. This abnormal venous drainage, which is characterised by special ultrasound criteria, called the "venous hemodynamic insufficiency severity score" (VHISS), is said to cause intracerebral flow disturbance or outflow problems that lead to periventricular deposits [2]. In the CCSVI theory, these deposits have a great similarity to the iron deposits seen around the veins in the legs in patients with chronic deep vein thrombosis. Zamboni, who first described this new theory, has promoted balloon dilatation to treat the outflow problems, thereby curing CCSVI and by the same token alleviating MS complaints. However, this theory does not fit into the existing bulk of scientific data concerning the pathophysiology of MS. In contrast, there is increasing worldwide acceptance of CCSVI and the associated balloon dilatation treatment, even though there is no supporting scientific evidence. Furthermore, most of the information we have comes from one source only. The treatment is called "liberation treatment," and the results of the treatment can be watched on YouTube. There are well-documented testimonies by MS patients who have gained improvement in their personal quality of life (QOL) after treatment. However, there are no data available from patients who underwent unsuccessful treatments with which to obtain a more balanced view. The current forum for the reporting of success in treating CCSVI and thus MS seems to be the Internet. At the CIRCE office and the MS Centre in Amsterdam, we receive approximately 10 to 20 inquiries a month about this treatment. In addition, many interventional radiologists, who are directly approached by MS patients, contact the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) for advice. Worldwide, several centres are actively promoting and performing balloon dilatation, with or without stenting, for CCSVI. Thus far, no trial data are available, and there is currently no randomized controlled trial (RCT) in progress Therefore, the basis for this new treatment rests on anecdotal evidence and successful testimonies by patients on the Internet. CIRSE believes that this is not a sound basis on which to offer a new treatment, which could have possible procedure-related complications, to an often desperate patient population.

Chronic cerebrospinal venous insufficiency (CCSVI) is a syndrome characterized by stenosies of the internal jugular and/or azygous veins (IJVs-AZ) with opening of collaterals and insufficient drainage proved by reduced cerebral blood flow and increased mean transit time in cerebral MRI perfusional study. The present review is aimed to give a comprehensive overview of the actual status of the art of the diagnosis and treatment of this condition. As far as the origin of venous narrowing is concerned, phlebographic studies of the IJVs and AZ systems demonstrated that venous stenoses were likely to be truncular venous malformations; mostly, they are intraluminal defects such as malformed valve, septa webs. CCSVI condition has been found to be strongly associated with multiple sclerosis (MS), a disabling neurodegenerative and demyelinating disease considered autoimmune in nature. In several epidemiological observations performed at different latitudes on patients with different genetic backgrounds, the prevalence of CCSVI in MS ranges from 56% to 100%. To the contrary, by using venous MR and/or different Doppler protocols, CCSVI was not detected with the same prevalence. Two pilot studies demonstrated the safety and feasibility in Day Surgery of the endovascular treatment of CCSVI by means of balloon angioplasty (PTA). It determines a significant reduction of postoperative venous pressure. Restenosis rate was found out elevated in the IJVs, but negligible in the AZ. However, PTA seems to positively influence clinical and QoL parameters of the associated MS and warrants further randomized control trials.

The truncular venous malformation (VM) represents an embryologically defective vein where developmental arrest has occurred during the vascular trunk formation period in the 'later stage' of the embryonic development. A relatively simple truncular VM lesion such as a venous web at the hepatic venous outlet causes portal hypertension giving a profound damage/impact to the liver. A similar condition involving the head and neck venous system may cause chronic cerebro-spinal venous insufficiency (CCSVI) and may be involved in the development or exacerbation of multiple sclerosis.

The pyramidal pathway is frequently affected early on in multiple sclerosis (MS) and impaired motor performance is a major cause of disability. Pyramidal tract function can be assessed using transcranial magnetic stimulation (TMS). TMS supports the diagnosis of MS, detecting corticospinal tract involvement and monitoring its course with or without treatment. It has been never investigated whether any relationship exists between the TMS outcome measure and minimally invasive treatment of multiple severe extracranial stenosis, affecting the principal ce rebrospinal venous segments in MS patients. We report the clinical and transcranial magnetic stimulation follow-up of a patient during a relapse in relapsing-remitting MS. She underwent percutaneous balloon angioplasty of the associated chronic cerebrospinal venous insufficiency (CCSVI), due to membranous obstruction of the proximal azygous vein, with severe stenosis of the left internal jugular vein. Treatment of the associated CCSVI made a parallel improvement in both clinical and neurophysiological parameters, allowing us to avoid high dose steroid therapy. The relationship between the clinical and neurophysiological course on the one hand, and haemodynamic correction of the associated CCSVI on the other, calls for further exploration on a wider number of patients. The impact of CCSVI on the different neuro-physiological parameters has not been fully estimated, but the intriguing case here reported suggests that it may be greater than previously assumed. The demonstration of a modification of the cerebrovenous function with both clinical manifestation and via TMS suggests that the hampered cerebral venous return may contribute to the clinical course of MS.

Aim: Chronic fatigue (CF) severely affects patients with multiple sclerosis (MS), but its pathogenesis remains elusive and the effectiveness of available treatments is modest. We aimed to evaluate the effect on CF of the balloon dilatation of stenosing lesions affecting the main extracranial veins configuring the chronic cerebrospinal venous insufficiency (CCSVI), a condition strongly associated with MS., Methods: Thirty-one MS consecutive patients (16 males, age 46.2+/-9.4 years) with associated CCSVI and CF underwent the endovascular procedure. Fatigue was assessed using the Fatigue Severity Scale (FSS) and Modified Fatigue Impact Scale (MFIS) at baseline (T0) and one (T1), six (T6) and twelve (T12) months after the procedure. In ambulatory patients (N.=28), mobility was evaluated using the 6-min walking test at T0 and T1., Results: and MFIS scores significantly improved from preoperative values, and the positive trend was maintained at one year (FSS: T0=5.1+/-1.0 to T12=3.5+/-1.8, P<0.001; MFIS-total score: T0=34.9+/-14.8 to T12=22.5+/-13.7, P<0.001; MFIS-Physical subscale: T0=21.2+/-8.0 to T12=13.5+/-9.7 P<0.001; MFIS-Cognitive subscale: T0=9.2+/-9.5 to T12=6.0+/-6.3, P=0.03; MFIS-Psychosocial subscale: T0=4.5+/-2.1 to T12=2.5+/-2.1, P<0.001). Six-min walking distance (6MWD) at T1 improved significantly (332+/-190m to 378+/-200m, P=0.0002). In addition, an inverted correlation between 6MWD and MFIS-physical subscale variations was found in the subgroup of patients (N.=8) with no lower limb motor impairment (r=-0.74, P=0.035)., Conclusion: The reestablishment of cerebral venous return dramatically reduced CF perception in a group of MS patients with associated CCSVI, suggesting that CF is likely the symptom of CCSVI.

Aim: We previously reported unexpectedly robust associations between vascular haemodynamic (VH) anomalies in the principal extracranial cerebral veins, causing chronic cerebrospinal venous insufficiency (CCSVI), and multiple sclerosis (MS). Aim of this study was to investigate the relationship between the VH changes and MRI measures of MS disease severity in a cross sectional survey., Methods: The number of anomalous VH criteria were measured using an echo-color Doppler, whereas CSF flow, atrophy and lesion measures were obtained from quantitative magnetic resonance imaging (MRI) analysis in sixteen consecutive relapsing-remitting MS patients, (mean age: 36.1+/-SD 7.3 years, disease duration: 7.5+/-1.9 years and median EDSS: 2.5) and in 8 healthy controls (HC) with similar age and sex distributions., Results: All 16 MS patients investigated and none of the HCs met the VH criteria for CCSVI (P<0.0001). MS patients showed significantly lower net CSF flow compared to the HC (P=0.038) that was associated with number of anomalous VH criteria present (r=0.79, P<0.001). Moreover, increases in the number of anomalous VH criteria present were negatively associated with lower whole brain volume (Spearman R=-0.5, P=0.05)., Conclusion: VH changes occur more frequently in MS patients than controls. Altered VH is associated with abnormal CSF flow dynamics and decreased brain volume.

Aim: Chronic cerebrospinal venous insufficiency (CCSVI) is associated with multiple sclerosis (MS). CCSVI is detected by transcranial and extracranial color-Doppler high-resolution examination (TCCS-ECD) and venography that permit to identify five types of venous malformations and four major (A-D) hemodynamic patterns of anomalous extracranial-extravertebral venous outflow. We investigated possible correlation between such hemodynamic patterns and both the symptoms at onset and clinical course in patients with MS and CCSVI., Methods: TCCS-ECD, selective venography and clinical records of 65 patients affected by definite MS and CCSVI were reviewed., Results: The four hemodynamic patterns of CCSVI were unevenly (P<0.0001) distributed with respect to the types of clinical presentation and course. In particular the Type A or B patterns were common in patients with onset of optic neuritis, but rare in patients presenting with spinal cord symptoms who typically showed a type D pattern. As well, the type A or type B hemodynamic were more common in patients with relapsing remitting course than in patients with secondary progressive course and rare in patients with primary progressive course. The C hemodynamic pattern was not observed in patients with primary progressive course who showed a remarkable prevalence of the type D pattern., Conclusion: The distribution of venous malformations and the resulting hemodynamic pattern show correlation with symptoms at onset and clinical course in patients with MS and CCSVI.

A chronic state of impaired venous drainage from the central nervous system, termed chronic cerebrospinal venous insufficiency (CCSVI), is claimed to be a pathologic phenomenon exclusively seen in multiple sclerosis (MS). This has invigorated the causal debate of MS and generated immense interest in the patient and scientific communities. A potential shift in the treatment paradigm of MS involving endovascular balloon angioplasty or venous stent placement has been proposed as well as conducted in small patient series. In some cases, it may have resulted in serious injury. In this Point of View, we discuss the recent investigations that led to the description of CCSVI as well as the conceptual and technical shortcomings that challenge the potential relationship of this phenomenon to MS. The need for conducting carefully designed and rigorously controlled studies to investigate CCVSI has been recognized by the scientific bodies engaged in MS research. Several scientific endeavors examining the presence of CCSVI in MS are being undertaken. At present, invasive and potentially dangerous endovascular procedures as therapy for patients with MS should be discouraged until such studies have been completed, analyzed, and debated in the scientific arena.

Pedicle screw fixation is a challenging procedure in thoracic spine, as inadvertently misplaced screws have high risk of complications. The accuracy of pedicle screws is typically defined as the screws axis being fully contained within the cortices of the pedicle. One hundred and eighty-five thoracic pedicle screws in 19 patients that were drawn from a total of 1.797 screws in 148 scoliosis patients being suspicious of medial and lateral malpositioning were investigated, retrospectively. Screw containment and the rate of misplacement were determined by postoperative axial CT sections. Medial screw malposition was measured between medial pedicle wall and medial margin of the pedicle screw. The distance between lateral margin of the pedicle screw and lateral vertebral corpus was measured in lateral malpositions. A screw that violated medially greater than 2 mm, while lateral violation greater than 6 mm was rated as an "unacceptable screw". The malpositions were medial in 20 (10.8%) and lateral in 34 (18.3%) screws. Medially, nine screws were rated as acceptable. Of the 29 acceptable lateral misplacement, 13 showed significant risk; five to aorta, six to pleura, one to azygos vein and one to trachea. The acceptability of medial pedicle breach may change in each level with different canal width and a different amount of cord shift. In lateral acceptable malpositions, the aorta is always at a risk by concave-sided screws. This CT-based study demonstrated that T4-T9 concave segments have a smaller safe zone with respect to both cord-aorta injury in medial and lateral malpositions. In these segments, screws should be accurate and screw malposition is to be unacceptable.

Persistence of the primitive hepatic venous plexus (PPHVP) is an uncommon anomaly that can present associated with complex congenital heart disease and inferior vena cava (IVC) development anomalies and complicate endovascular and surgical procedures. We report a case of PPHVP in a patient with complex congenital heart disease with unexplained persistent arterial desaturation after Kawashima operation and describe magnetic resonance angiography (Angio-MRI) findings leading to the diagnosis. We recommend that IVC-Angio-MRI be performed in preoperative evaluation of patients with complex congenital heart disease and left isomerism when redirection of systemic venous blood to the pulmonary arterial circulation is considered.

Background: In cirrhosis, repeated flares of portal pressure and collateral blood flow provoked by postprandial hyperaemia may contribute to variceal dilation and rupture., Aim: To examine the effect of the extent of the collateral circulation on the postprandial increase in portal pressure observed in cirrhosis., Patients and Methods: The hepatic venous pressure gradient (HVPG), hepatic blood flow and azygos blood flow were measured in 64 patients with cirrhosis before and after a standard liquid meal., Results: Peak increases in HVPG (median+14.9%), hepatic blood flow (median+25.4%), and azygos blood flow (median+32.2%) occurred at 30 min after the meal. Compared with patients with marked postprandial increase in HVPG (above the median, n = 32), those showing mild (<15%, n = 32) increase in HVPG had a higher baseline azygos flow (p<0.01) and underwent a greater postprandial increase in azygos flow (p<0.02). Hepatic blood flow increased similarly in both groups. Postprandial increases in HVPG were inversely correlated (p<0.001) with both baseline azygos flow (r = -0.69) and its postprandial increase (r = -0.72). Food intake increased nitric oxide products in the azygos (p<0.01), but not in the hepatic vein. Large varices (p<0.01) and previous variceal bleeding (p<0.001) were more frequent in patients with mild increase in HVPG., Conclusions: Postprandial hyperaemia simultaneously increases HVPG and collateral flow. The extent of the collateral circulation determines the HVPG response to food intake. Patients with extensive collateralisation show less pronounced postprandial increases in HVPG, but associated with marked flares in collateral flow. Collateral vessels preserve their ability to dilate in response to increased blood flow.

Aim: To detect the hemodynamic alterations in collateral circulation before and after combined endoscopic variceal ligation (EVL) and splenectomy with pericardial devascularization by ultrasonography, and to evaluate their effect using hemodynamic parameters., Methods: Forty-three patients with esophageal varices received combined EVL and splenectomy with pericardial devascularization for variceal eradication. The esophageal vein structures and azygos blood flow (AZBF) were detected by endoscopic ultrasonography and color Doppler ultrasound. The recurrence and rebleeding of esophageal varices were followed up., Results: Patients with moderate or severe varices in the esophageal wall and those with severe peri-esophageal collateral vein varices had improvements after treatment, while the percentage of patients with severe para-esophageal collateral vein varices decreased from 54.49% to 2.33%, and the percentage of patients with detectable perforating veins decreased from 79.07% to 4.65% (P < 0.01). Color Doppler flowmetry showed a significant decrease both in AZBF (43.00%, P < 0.05) and in diameter of the azygos vein (28.85%, P < 0.05), while the blood flow rate was unchanged. The recurrence rate of esophageal varices was 2.5% (1/40, mild), while no re-bleeding cases were recorded., Conclusion: EVL in combination with splenectomy with pericardial devascularization can block the collateral veins both inside and outside of the esophageal wall, and is more advantagious over splenectomy in combination with pericardial devascularization or EVL in preventing recurrence and re-bleeding of varices.

Background: The study compared the preoperative portal vein congestion index estimated by Doppler ultrasound and the postoperative portal vein thrombosis of patients submitted to esophagogastric devascularization and splenectomy (EDS)., Methods: 65 patients with portal hypertension due to schistosomiasis and previous gastrointestinal bleeding submitted to EDS were divided into two groups: GROUP A (28 patients without postoperative portal vein thrombosis) and GROUP B (37 patients with postoperative portal vein thrombosis). The following parameters of preoperative Doppler ultrasound of the portal vein were analyzed: diameter, area, mean blood flow velocity and blood flow, whereupon the congestion index was calculated., Results: The diameter, area and blood flow of the portal vein were greater in group B (mean of 1.52 cm; 1.77 cm(2) and 2533.12 ml / min) than in group A (mean of 1.33 cm; 1.44 cm(2) and 1609.03 ml / min) with p = 0.03; 0.03 and 0.04 respectively. Difference of the congestion index was not statistically significant between the two groups (p = 0.07)., Conclusions: The portal vein congestion index at the preoperative of EDS estimated by Doppler ultrasound was not predictive of portal vein thrombosis in the postoperative of patients with portal hypertension due to schistosomiasis.

Study Design: In the last 2 years, we have examined 9640 patients experiencing back pain or sciatica, using MRI. There were 13 (0.13%) patients who had radicular symptoms that clinically mimicked lumbar disc herniation or spinal stenosis. All of these patients had inferior vena caval obstruction or occlusion that caused engorgement in the epidural and paravertebral venous system, causing nerve root compression., Objective: To illustrate the imaging characteristics of 13 patients with epidural engorged veins due to inferior vena cava obstruction or occlusion causing sciatica and low back pain., Summary of the Background Data: Abnormalities or pathological changes of epidural venous network may give rise to symptoms similar to or mimicking lumbar disc herniation or spinal stenosis. Multiple lumbar epidural varices can cause nerve root and thecal sac compression. Lumbar epidural varices have been infrequently described in the literature. To date, the cause of anterior epidural venous enlargement has been poorly understood, and both congenital and acquired causes have been proposed. This report describes enlarged epidural veins in patients with inferior vena caval thrombosis or obstruction presenting with radicular syndromes., Methods: The authors have seen 13 patients with radicular symptoms. All of the patients had inferior vena caval obstruction or occlusion that caused engorgement in the epidural veins, causing nerve root compression. The ages of these patients ranged from 20 to 53 (mean, 30) years. All of the patients were examined with color Doppler ultrasonography and magnetic resonance scanner., Results: Ten of 13 patients had inferior vena cava thrombosis located just under the renal vein orifices. In two patients, there was compression to inferior vena cava due to parity. In the remaining patient, a huge intra-abdominal mass was observed, and this mass was causing inferior vena cava obstruction and invasion. Enlargements of epidural venous plexus were demonstrated in all of these cases. All of the patients presented with the acute onset of low back pain followed shortly thereafter by acute radicular symptoms. The compression to inferior vena cava could not be treated in one patient because of intra-abdominal malignancy. The remaining 12 patients with inferior vena cava obstruction or occlusion experienced complete resolution of symptoms after treatment or delivery., Conclusion: The authors believe that epidural venous engorgement should be considered when the symptoms of patients with deep venous and inferior vena cava thrombosis are accompanied by radicular and/or back pain, because pathologic processes compressing a nerve root can cause pain.