Inferior vena cava malformations and deep venous thrombosis

We carried out a prospective study of 116 patients under 50 years of age who had deep venous thrombosis of the lower extremities to determine whether the presence of congenital anomaly of the inferior vena cava (IVC) was a risk factor for the disease. All patients were investigated by Doppler echography. Some 37 patients who had iliac vein occlusion also underwent phlebography. In 10 patients in whom the IVC was difficult to image, magnetic resonance angiography or computerized axial tomography was carried out. In all patients, studies of antithrombin, protein C and protein S deficiency, factor V Leiden, prothrombin G20210A, antiphospholipid antibodies, and acquired risk factors were also performed. Of the 37 patients who had iliac vein occlusion, six had an IVC anomaly. Two of these patients had antiphospholipid antibodies, while another had prothrombin G20210A. Two patients with an anomaly had recurrent thrombotic occlusion. In conclusion, congenital IVC anomalies were present in 16.2% (95% confidence interval, 6.2-32%) of young patients with iliac thrombosis.     

Acute right lower extremity iliofemoral deep venous thrombosis secondary to an anomalous inferior vena cava: a report of two cases

A congenital anomaly of the inferior vena cava is an increasingly identified risk factor for iliocaval deep venous thrombosis in young patients. We present two cases of acute right lower extremity iliofemoral deep venous thrombosis in 16 and 18-year-old patients that were ultimately diagnosed with an underlying anomalous iliocaval venous system. In one patient, the inferior vena cava was congenitally absent and the right iliac vein was diffusely stenotic with a proximally located high-grade stricture. In the other subject, the infrarenal inferior vena cava and right iliac vein were diffusely hypoplastic. Compensatory venous flow in both subjects was via large perilumbar venous collateral veins and a prominent azygous system. Both patients were successfully treated with a combination of initial catheter-directed thrombolysis followed by balloon angioplasty and venous stent placement.     

Coexistence of left-sided inferior vena cava, deep vein thrombosis of the upper and lower extremities and prothrombotic polymorphisms in a young patient: a case report.

A literature review suggests an interaction between an anomaly of the inferior vena cava and thrombophilia in the pathogenesis of deep vein thrombosis. Genetic thrombotic abnormalities have been found in some of the subjects having venous thromboembolic diseases. We report a case of a young man presenting with venous thrombosis of the upper and lower extremities, left-sided vena cava inferior and with combination of heterozygosity of the mutation of the genes Methylenetetrahydrofolate reductase 677 and Factor V 1691.     

Portal and mesenteric vein and inferior vena cava thrombosis associated with antiphospholipid syndrome

We report a 48-year-old man with thrombosis of the portal and superior mesenteric vein and inferior vena cava associated with primary antiphospholipid syndrome (APS). Primary APS was diagnosed by a positive reaction with anticardiolipin antibody (aCL) and the absence of any evidence suggesting the presence of other disease states known to be associated with aCL. A coeliac angiography showed obstruction of the portal and superior mesenteric vein with prominent collaterals and cavernous transformation. Femoral vein angiography showed total obstruction of the external iliac vein and inferior vena cava, and dilation of the pelvic veins, with contrast medium in the lumbar vein. This case is noteworthy as a report of primary APS accompanied by extensive abdominal and pelvic venous thrombosis.     

Acute thrombosis of pelvic and leg veins in agenesis of the renal segment of the inferior vena cava

A 19-year-old, otherwise asymptomatic man presented to the hospital of orthopaedic surgery with acute severe pain like lumbago. Symptomatic treatment was performed after extensive orthopaedic diagnostic procedures. On the third day after admission he showed clinical signs of deep vein thrombosis with painful swelling and livid discoloration of both legs. Colour duplex ultrasound revealed complete thrombosis of the leg and pelvic veins bilaterally, but the cranial extent was not clear. Contrast-enhanced helical computer tomography of the abdomen and the pelvis confirmed deep pelvic vein thrombosis and showed extension into the inferior vena cava. Moreover, the study revealed the agenesis of the renal segment of the inferior vena cava with collateral flow through dilated lumbar veins to enlarged azygous and hemiazygous, through vertebral and paravertebral venous plexus. The renals were drained via dilated capsular veins. The agenesis of renal vena cava is a very rare anomaly causing acute thrombosis of the deep leg and pelvic veins. Other risk factors of thromboembolic disease were not found. The patient was treated successfully with systemic thrombolysis. Therefore we used ultra-high streptokinase infusion (9 million units over 6 hours). Colour duplex ultrasound revealed good flow into deep leg and pelvic veins after three cycle of lysis. Magnetic resonance angiography of the abdomen and pelvis was performed to evaluate the successful fibrinolysis with complete recanalisation of the pelvic veins and to demonstrate the venous anatomy. Permanent oral anticoagulation with phenprocoumon is indicated to decrease the high rate of recurrent thrombosis. Compression stockings were prescribed. To prevent thrombosis, additional risk factors like smoking, immobilization and unusual physical activity should be strictly avoided.     

Congenital familial vascular anomalies: a study of patients with an anomalous inferior vena cava, and of their first-degree relatives

With magnetic resonance angiography and computed tomography, congenital anomalies of the inferior vena cava are diagnosed more frequently than they used to be. Accessory renal arteries identified by magnetic resonance angiography in a patient with an anomalous inferior vena cava indicated a combination of arterial and venous abnormalities. The study was initiated to screen consecutive patients with an anomalous inferior vena cava for concomitant abdominal and pelvic arterial abnormalities, and their first-degree relatives for congenital vascular anomalies. Magnetic resonance angiography identified in 2 of 5 patients with an anomalous inferior vena cava concomitant accessory renal arteries and in 5 of 11 first-degree relatives major abdominal vascular anomalies including accessory renal arteries, accessory renal veins, and anomalies of the hepatic artery. None of the relatives showed abnormalities of the inferior vena cava. The familial occurrence of vascular anomalies strongly suggests an underlying pathogenetic component in affected family members. In patients with a congenital anomaly of the inferior vena cava, concomitant arterial abnormalities should be considered. First-degree relatives may be at risk for congenital vascular anomalies.     

Congenital absence of the inferior vena cava as a risk factor for pulmonar thromboembolism

The absence of the inferior vena cava is a rare congenital anomaly. Currently its diagnosis is based on non-invasive imaging techniques (computerised axial tomagraphy and nuclear magnetic resonance). In most cases, it constitutes a casual finding upon practising these image tests unrelated to this congenital anomaly. In the symptomatic patients, the complaints associated are secondary to venous insufficiency and/or deep vein thrombosis. Recently the congenital absence of inferior vena cava has been described as a risk factor of deep vein thrombosis in young patients. We present a case of congenital absence of inferior vena cava that was admitted in our hospital because of pulmonary thromboembolism.     

Transformation of inferior vena caval thrombosis to membranous obstruction in a patient with the lupus anticoagulant

A 24-yr-old woman with hemolytic anemia developed multiple thrombosis of the hepatic vein and inferior vena cava. She was found to have circulating lupus anticoagulant that could have been causally related to the thrombosis and hence the Budd-Chiari syndrome. On her first admission to the hospital vena cava and hepatic vein catheterizations revealed partial thrombotic occlusion of the cava at the level of the diaphragm, which was subsequently transformed into complete membranous obstruction. The right hepatic vein, which was patent on the first admission, was also completely occluded. These observations support the theory that membranous obstruction of the inferior vena cava is a sequela to inferior vena caval thrombosis rather than a congenital anomaly.     

Non-invasive methods for vascular studies in the diagnosis of deep venous thrombosis

The sonographic diagnosis of deep venous thrombosis must be made up of a functional continuous wave Doppler study of the whole deep venous system of the limbs, including leg veins, as well as saphenous veins. Then, high resolution B-mode real time sonography is used for the detection of direct (echogenic thrombus) or indirect (incompressible vein) signs of thrombosis. This noninvasive approach offers a good sensitivity (about 96%) and a high level of specificity (about 98%). Moreover, B-mode sonography can ensure the differential diagnosis (hematoma, extrinsic compression...) in most cases. So, X-Ray venography is required only when an interventional therapy is planned (thrombectomy, fibrinolysis, inferior vena cava interruption...), or when the noninvasive techniques are not able to show the upper limit of the thrombosis (especially for iliac veins or inferior vena cava), or when there is still a doubt about deep venous thrombosis. Therefore, the number of X Ray venographies can be consistently reduced, thus decreasing both cost and risks.     

Bilateral iliac vein thrombosis after seat belt-related trauma revealing hypoplasia of the inferior vena cava--a case report

Hypoplasia of the inferior vena cava can be revealed by a deep venous thrombosis of the lower limbs. Associated precipitating factors or clotting defects leading to thrombosis are frequently observed. A case of bilateral iliac veins thrombosis occurring after a motor vehicle accident with seat belt injury is reported, revealing hypoplasia of the inferior vena cava. This young man was totally asymptomatic up to the crash, and did not have coagulation abnormalities. The patient had a very good outcome after anticoagulant treatment with complete regression of venous thromboses. Hypoplasia of the inferior vena cava was a predisposing anatomic abnormality that led to thrombosis, but seat belt trauma was probably the precipitating factor. This observation should be kept in mind in the evaluation of a deep venous lower limb thrombosis.     

Deep vein thrombosis caused by congenial interruption of the inferior vena cava--a case report

Congenital interruption of the inferior vena cava is an uncommon vascular anomaly. In this setting, the appearance of deep vein thrombosis is very rare because associated azygous or portal continuation develops as a collateral system for venous return. The authors present a case of infrahepatic interruption of the inferior vena cava in a 21-year-old man who presented with symptoms of deep vein thrombosis. Clinical features and prognosis of this entity are discussed.     

Indirect CT venography following CT pulmonary angiography: spectrum of CT findings

SUMMARY: Pulmonary embolism (PE) and deep venous thrombosis (DVT) represent two manifestations of the same syndrome, venous thromboembolism. Contrast-enhanced computed tomography (CT) angiography is a practical, efficient alternative to conventional imaging for PE. Following the pulmonary examination, the inferior vena cava (IVC) and the iliac, femoral, and popliteal veins can be studied with CT without additional intravenous contrast administration. Indirect CT venography (CTV) after CT pulmonary angiography (CTPA) simplifies and shortens venous thromboembolism work-up. Initial studies indicate that CTV is comparable to ultrasound in the evaluation of femoral/popliteal DVT. CTV has the advantage of evaluating the iliac veins and inferior vena cava, vessels poorly seen on sonography and venography. Combining CTV with CTPA increases confidence in withholding treatment when results for both the pulmonary arteries and leg veins are negative and increases the diagnosis of venous thromboembolism by 25% over CTPA alone. This pictorial essay will review the normal venous anatomy, CTV technique, and the findings of acute and chronic DVT. Interpretive pitfalls and alternative diagnoses are also reviewed.     

Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism

Congenital anomalies of the inferior vena cava such as absence or atresia are uncommon vascular defects and result from aberrant development during embryogenesis. We report a case of a young female patient affected by proximal deep venous thrombosis (DVT) complicated by liver and pulmonary embolism; subsequent extensive evaluation revealed the congenital absence of infrarenal inferior vena cava, with emboli probably occurring through collateral veins. Accordingly, in young patients with idiopathic DVT of the lower extremities and pelvic veins, the presence of inferior vena cava abnormalities should always be considered and investigated, together with classic coagulation factors, as a factor predisposing to thromboembolic complications.     

In-Hospital Risks and Management of Deep Venous Thrombosis According to Location of the Thrombus

BackgroundWhether deep venous thrombosis involving the pelvic veins or inferior vena cava is associated with higher in-hospital mortality or higher prevalence of in-hospital pulmonary embolism than proximal or distal lower extremity deep venous thrombosis is not known.MethodsThis was a retrospective cohort study based on administrative data from the Nationwide Inpatient Sample, 2016, 2017. Patients hospitalized with a primary diagnosis of deep venous thrombosis at known locations were identified by International Classification of Diseases-10-Clinical Modification codes.ResultsIn-hospital all-cause mortality with deep venous thrombosis involving the inferior vena cava in patients treated only with anticoagulants was 2.2% versus 0.8% with pelvic vein deep venous thrombosis (p<0.0001), 0.7% with proximal deep venous thrombosis (p<0.0001) and 0.2% with distal deep venous thrombosis (p<0.0001). Mortality with anticoagulants was similar with pelvic vein deep venous thrombosis compared with proximal lower extremity deep venous thrombosis, 0.8% versus 0.7% (p=0.39). Lower mortality was shown with pelvic vein deep venous thrombosis treated with thrombolytics than with anticoagulants, 0% versus 0.8% (p<0.0001). In-hospital pulmonary embolism occurred in 11% to 23%, irrespective of the site of deep venous thrombosis.ConclusionPatients with deep venous thrombosis involving the inferior vena cava had higher in-hospital mortality than patients with deep venous thrombosis at other locations. Pelvic vein deep venous thrombosis did not result in higher mortality or more in-hospital pulmonary embolism than proximal lower extremity deep venous thrombosis. The incidence of in-hospital pulmonary embolism was considerable with deep venous thrombosis at all sites.     

Successful blood sampling through azygos continuation with interrupted inferior vena cava. A case report and review of the literature

Interrupted inferior vena cava (IVC) with azygos continuation is a rare congenital anomaly, and is frequently associated with other cardiovascular malformations and situs anomalies, such as left isomerism. These patients usually develop deep vein thrombosis (DVT), and asymptomatic patients above 60 years of age are very rare. Here we report a case of interrupted IVC which we diagnosed in a 72-year-old woman. She was admitted to our hospital suffering from heart failure and supraventricular tachycardia. Echocardiography detected secundum atrial septal defect (ASD). An abnormal paravertebral pleural line on the chest X-rays indicated the existence of venous anomaly. Anatomical images obtained by Multidetector Computed Tomography (MDCT) helped us to successfully perform right heart catheterization procedures through azygos continuation including blood sampling from pulmonary veins. Even in elderly patients, a careful examination of chest X-rays can indicate undiagnosed venous anomalies; thus, it is critically important before planning surgical or interventional procedures.     

Arterial and venous thrombosis in rare congenital bleeding disorders: a critical review

A thorough review of the literature and of personal files has allowed the gathering of 81 patients with rare congenital bleeding disorders and thrombotic phenomena. Sixteen of these patients had congenital afibrinogenemia, eight involved factor V deficiency, 20 factor VII defects, 33 factor XI deficiencies and only one, a factor XIII defect. Altogether 42 patients showed arterial thrombosis (myocardial infarction [MI] in 28 cases; ischemic stroke in 4; arterial occlusion in 8; 2 patients with disseminated intravascular coagulation (DIC)). Ages varied between 13 and 74. Twenty-two patients were males and 16 females. In four cases, sex was not reported. There were three fatalities: two after a MI and one because of heart failure. With regard to venous thrombosis: 9 patients had pulmonary embolism, 15 patients had deep vein thrombosis, 9 patients had both pulmonary embolism and deep vein thrombosis; 1 patient had superficial vein thrombosis, whereas, 5 cases had an unusual site venous thrombosis (two portal systems, two cerebral sinuses, one inferior vena cava) for a total of 39 cases. Age varied between 3 and 86. In this case, 20 patients were males and 17 were females. In two cases, sex was not reported. There were three fatalities: two because of pulmonary embolism and one because of inferior vena cava thrombosis. The fact that thrombosis has never been described in patients with factor II or factor X seems to underscore the central antithrombotic role that these two factors have in the coagulation system.     

经皮腘静脉穿刺诊治下肢深静脉血栓39例临床分析

目的探讨经皮腘静脉血流顺向性介入治疗下肢深静脉血栓的可行性。方法对39例下肢深静脉血栓患者经健侧股静脉置入下腔静脉滤器后,穿刺患侧腘静脉,以腘静脉为入路,行血管内溶栓、血栓消融器消融和(或)球囊成形及腔内支架置人术等治疗。结果经皮腘静脉穿刺均获成功,无严重并发症发生。结论以患侧腘静脉为入路,血流顺行性介入治疗下肢深静脉血栓操作简便,是一种安全有效的方法。     

Combined percutaneous aspiration thrombectomy and rheolytic thrombectomy in massive subacute vena cava thrombosis with IVC filter occlusion.

PURPOSE: To report the combined use of percutaneous aspiration thrombectomy and rheolytic thrombectomy in the setting of extensive inferior vena cava (IVC) thrombosis and filter occlusion. CASE REPORT: A 28-year-old paraplegic man with a vena cava filter in situ for previous deep vein thrombosis (DVT) was referred to our center for evaluation of dyspnea and right leg edema and swelling. Computed tomography excluded a pulmonary embolism and revealed severe, massive DVT of both iliac veins and the IVC, including the vena cava filter. Percutaneous aspiration thrombectomy was attempted because intravenous heparin therapy was ineffective, and moderate anemia contraindicated regional thrombolysis. Several passes of a guiding catheter proximally and distally to the filter, with suction provided by a 50-mL syringe, achieved minimal IVC recanalization. Subsequently, a 6-F AngioJet catheter was passed via the guiding catheter through the filter, the IVC, and both iliac veins, obtaining a satisfactory result. The patient was discharged after 7 days and did very well at 6-month follow-up, with no recurrent DVT. CONCLUSION: This case demonstrates the usefulness of combined percutaneous aspiration and rheolytic thrombectomy in treating extensive IVC thrombosis and occluded IVC filters, especially when thrombolytic therapy cannot be used.     

Transjugular intrahepatic portosystemic shunt in combination with oral anticoagulant for Budd-Chiari syndrome.

The antiphospholipid antibody syndrome is characterized by arterial and venous thrombosis including hepatic veins. Although transjugular intrahepatic portosystemic shunt or liver transplantation have been considered for Budd-Chiari syndrome, treatment options for patients with complete obstruction of three hepatic veins including the junction with the inferior vena cava are limited. We describe a 27-year-old female, who suffered thrombotic obliteration of hepatic veins including the portion confluent with the inferior vena cava (Budd-Chiari syndrome) associated with marked ascites and liver dysfunction. Transjugular intrahepatic portosystemic shunt using a Wall-stent (10 mm in diameter) between inferior vena cava and intrahepatic portal vein was performed. Intrastent coagulation and recurrence of thrombosis were prevented by combination therapy with warfarin potassium and ticlopidine hydrochloride. These treatments induced loss of ascites and improvement of liver function, and she has been able to resume daily life. The portosystemic shunt described above in addition to combination therapy with warfarin potassium and ticlopidine hydrochloride appeared to be one of the options for treating Budd-Chiari syndrome associated with antiphospholipid antibody syndrome.     

Incidence of vena cava thrombosis in the United States

From 1979 through 2005, vena cava thrombosis (either superior or inferior) was diagnosed in 99,000 hospitalized patients. Most, 78%, had isolated vena cava thrombosis. From 2000 to 2005, 5,000 patients were diagnosed yearly with vena cava thrombosis (1.5% of patients hospitalized with deep venous thrombosis). The population-based incidence of diagnosis of vena cava thrombosis from 2001 to 2005 was 1.7 in 100,000. The incidence increased with age. It was rare in Asian Americans. Pulmonary embolism occurred in 12% of patients with isolated vena cava thrombosis. Cancer was frequently associated with vena cava thrombosis (37.5%). Among all patients hospitalized with cancer, however, it was an uncommon complication (0.07%). In conclusion, isolated vena cava thrombosis is an uncommon cause of pulmonary embolism but may be considered if the veins of the extremities show no deep venous thrombosis.