Spontaneous splenoazygos shunt in a patient with portal hypertension: an exceedingly rare occurrence

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Unusual Drainage of the Bifurcated Left Renal Vein Into a Dilated Lumbar Azygos Vein and Inferior Vena Cava.

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 18F-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.

Fistula from Congenital Subclavian Artery to Azygos Vein with Congestive Heart Failure in an Adult.

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.

Venous stent patency may be affected by collateral vein lumen size.

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.

Surgical Challenge in Pediatric Kidney Transplant Vascular Anastomosis.

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.

Double Inferior Vena Cava (IVC) With Intrahepatic Interruption, Hemiazygos Vein Continuation, and Intrahepatic Venous Shunt.

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.

Portal hypertension in patients with cirrhosis: indirect assessment of hepatic venous pressure gradient by measuring azygos flow with 2D-cine phase-contrast magnetic resonance imaging.

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.

Endovascular recanalization of a port catheter-associated superior vena cava syndrome.

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.

Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.

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.

Venous Hemodynamic Insufficiency Severity Score variation after endovascular treatment of chronic cerebrospinal venous insufficiency.

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.

Chronic cerebrospinal venous insufficiency in Ménière disease.

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.

Perioperative advantages of modified laparoscopic vs open splenectomy and azygoportal disconnection.

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.

Incidence and distribution of extravascular compression of extracranial venous pathway in patients with chronic cerebrospinal venous insufficiency and multiple sclerosis.

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.

Compressive syndrome of internal jugular veins in multiple sclerosis: does it matter?

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.

Leaving antegrade pulmonary blood flow results in reversal of flow in the azygous vein and decompression of the cavopulmonary circulation: mechanism of cyanosis defined with cardiac magnetic resonance imaging.

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.