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(A) Left lumbar vein thrombosis (arrow) in a patient with IVCA (CT scan); (B) an axial image at the same level in a normal patient.
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Inferior vena cava agenesis (IVCA) is a rare condition, found in almost 5% of patients under 30 years old with unprovoked deep venous thrombosis (DVT). We describe 10 consecutive patients with IVCA-associated DVT and conducted an extensive literature review to investigate the typical spectrum of IVCA-associated DVT. Among our patients (eight men an...
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Citations
... In human and canine patients, most of the cases with this condition are detected incidentally (Harder et al., 2002). Nevertheless, it has also been reported in symptomatic patients associated with thrombosis of the CVC and/or renal veins, and pulmonary thromboembolism (Harder et al., 2002;Lambert et al., 2010;Lockwood et al., 2018;Newman et al., 2020), as well as with other congenital malformations such as polysplenia, situs anomalies or portosystemic shunt (Bass et al., 2000;Fischetti and Kovak, 2008;Li et al., 2021). In the case described herein, the patient had a CT examination as a part of a diagnostic investigation of clinical signs related to the respiratory system, and the abdomen was included in the protocol to rule out clinically relevant comorbidities. ...
Background
Segmental aplasia of the caudal vena cava (CVC) with azygos continuation is a congenital malformation macroscopically described in mammals including humans, dogs, and rodents. It is usually detected as an incidental finding and the final diagnosis is reached by computed tomography (CT), fluoroscopy, or post-mortem dissection.
Case Description
A 3-year-old guinea pig (Cavia porcellus) presented with subacute dyspnea. A computed tomographic examination was performed for the evaluation of subtle pulmonary changes previously suspected on conventional radiography, and a segmental aplasia of the CVC with azygos continuation was identified as an incidental finding.
Conclusion
According to database negative results, this is the first report describing a segmental aplasia of the CVC and azygos continuation in a guinea pig by CT.
... Collateral pathways play a crucial role in maintaining proper drainage from the legs and pelvis through the deep, median, portal, and superficial pathways. [4][5][6] Malformations of the IVC include IVCA, a left IVC, double IVC, azygos continuation of the IVC, a circumaortic left renal vein, a retroaortic left renal vein, a double IVC with a retroaortic right renal vein and hemiazygos continuation, a double IVC with retroaortic left renal vein and azygos continuation, a circumcaval ureter, and isolated accessory hemiazygos vein. 5,7 IVCA has a prevalence of 0.0005% to 1% 6 and occurs due to underdevelopment of the posterior and supracardinal veins. ...
... Reports of patients with IVCA and DVT have reported successful management with anticoagulation and compression hosiery, without DVT recurrence. 6,8 Among 62 reported cases, most patients were treated similarly, with some receiving thrombolysis before anticoagulation and a few requiring bypass surgery. 6 IVCO treatment typically involves stent placement in veins such as the common femoral, external iliac, and common iliac veins and the IVC, with a high success rate. ...
... 6,8 Among 62 reported cases, most patients were treated similarly, with some receiving thrombolysis before anticoagulation and a few requiring bypass surgery. 6 IVCO treatment typically involves stent placement in veins such as the common femoral, external iliac, and common iliac veins and the IVC, with a high success rate. 9 Therefore, when navigating the retroperitoneal lumbar to azygos connections in the context of chronic IVCO, the technical aspects are similar to performing conventional iliac vein recanalization. ...
Chronic venous insufficiency, caused by inferior vena cava occlusion, can lead to thromboembolic complications and tissue loss. We present two cases of azygos vein stenting (AVS) in which vena cava recanalization techniques were exhausted. In the first case, the left iliac vein and vena cava were recanalized and stented; however, the right iliac vein had been previously resected and required AVS. Conventional recanalization attempts from the right and left iliac systems failed in the second patient with congenital inferior vena cava occlusion; therefore, AVS was chosen to establish in-line drainage
... Keywords [4][5][6], а среди людей старше 40 лет -в 82% случаев [7,8]. Вариант аплазии печеночного сегмента НПВ с дренированием в непарную вену встречается в 0,6% случаев в популяции [9,10]. ...
Detection of a combination of aplasia of the inferior vena cava and retroaortic left renal vein is extremely rare. A description of a clinical case with similar anomalies in the development of the inferior vena cava and the left renal vein is given. In our case, the combination of an anomaly of the inferior vena cava and the left renal vein in a 59-year-old male patient was an incidental finding during examination and treatment for acute cerebrovascular accident. Asymptomatic aplasia of the prerenal and hepatic sections of the inferior vena cava with drainage into the azygous vein was revealed. The hepatic veins empty into the right atrium. The identified variant of aplasia of the prerenal and hepatic sections of the inferior vena cava with drainage into the unpaired vein is the result of atrophy of the right subcardinal vein. A rare unclassified type of retroaortic left renal vein was found, the lower branch of which flows into the inferior vena cava, and the upper branch into the unpaired vein. The retroaortic left renal vein is formed while maintaining the anastomosis between the right and left supracardinal veins. The possibility of accidental radiographic findings of anomalies in the development of the inferior vena cava and its tributaries in humans before clinical manifestations has been established. Given the possible manifestation of an anomaly of the inferior vena cava in people over 40 years of age with a clinic of peripheral venous thrombosis, and the manifestation of the retroaortic left renal vein - the development of congestive venous hypertension in the kidney, the patient was given the necessary recommendations.
... This is because emboli get trapped in the azygos-hemiazygos system before reaching the pulmonary circulation. 12 It is important to note that IVCA is likely underdiagnosed since it cannot be detected through Doppler ultrasound. In cases where suspicion arises, it is crucial to perform computed tomography or magnetic resonance imaging to confirm the diagnosis. ...
... Historically, treatment with VKAs was proposed. 12,13 The use of DOACs in this context lacks sufficient data, and there has even been a case report describing the lack of efficacy of rivaroxaban. 13 Large-scale validation studies on DOACs, [16][17][18] and studies evaluating the continuation of halfdose therapeutic anticoagulation for patients without major thrombophilia have not included any IVCA patients. ...
... Patients should also be advised to avoid additional risk factors, such as prolonged immobilization and oral contraceptives. 12 Given the limitations of VKA monitoring, the use of DOAC presents an appealing alternative for extended anticoagulation in young individuals. There is no evidence to suggest that this congenital defect would lead to DOAC resistance. ...
Deep vein thrombosis (DVT) is common in the general population, with an annual incidence of 1 to 2 per 1000 people. Inferior vena cava agenesis (IVCA) increased the risk of developing DVT and is found in approximately 5% of young adults (20-40-year-olds) diagnosed with unprovoked proximal DVT. IVCA can be caused by a defective embryological process, or be a result of intrauterine or perinatal thrombosis. Its estimated incidence in the general population ranges from 0.0005% to 1%, usually involving a partial absence of one of the four segments of the inferior vena cava (IVC). The management during the extended phase of patients with DVT associated with IVCA is not yet harmonized, as it is poorly described in the literature. Patients with IVCA are considered to be at high risk of DVT occurrence, prompting physicians to continue extended anticoagulation, often using vitamin K antagonists. In this retrospective study, we present a cohort of 11 patients diagnosed with IVCA following a DVT, who subsequently received extended treatment with a direct oral anticoagulants. These findings offer reassuring insights into the extended utilization of direct oral anticoagulants, demonstrating both antithrombotic efficacy and a favorable safety profile.
... Inferior vena cava anomalies may be accompanied by cardiac pathologies (10,11), venous thrombus (12) and pulmonary embolism, polysplenia (13) and renal vein anomalies (14,15,16,17,18,19). In accordance with this information, two of our previously mentioned cases presented a clinical picture of renal vein anomalies. ...
Objective: Inferior vena cava (IVC) anomalies are very rare vascular embryological variations, the incidence rate in the general population is 0.5%. IVC anomalies are usually asymptomatic and detected incidentally. The inferior vena cava is formed between the 6th and 8th weeks of intrauterine embryological development. IVC occurs due to the fusion of the supracardinal, postcardinal, and subcardinal veins during the embryological period. This union results from complex anastomosis of the embryological stage veins. During this time, various IVC anomalies may develop. IVC anomalies increase the risk of deep vein thrombosis in the lower extremity. Because of the increased risk of deep vein thrombosis in these patients, anti-embolism prophylaxis can be performed before the operation. Therefore, the risk of pulmonary embolism increases as well. The use of computed tomography has become more common nowadays. The detection rate of IVC anomalies has increased in examinations performed for other purposes. Case: IVC anomalies are important in terms of surgical interventions and the use of a vena cava filter. Knowing the existence of IVC anomalies can be crucial in preventing complications that may arise during surgical and radiological procedures. The objective of this study is to present three different cases with incidentally detected IVC anomalies.
... 2 Although the risk of DVT is increased in these patients, the rate of pulmonary embolism (PE) is low (9%). 11 To reach the pulmonary circulation, thrombus would have to cross a tortuous route through collateral veins, being retained therein in most cases. 11 Other symptoms that may result are: venous claudication, 12 hematuria caused by bladder varicosities, 13 lumbar pain due to venous collaterals in the vertebral and paravertebral plexus, causing compression of the thecal sac and at the output of the nerve roots, 14 cauda equina symptoms such as bladder or bowel dysfunction and lower limb weakness, 15 visceral compressions by the gonadal venous system, 16 venous ulcers due to deep venous insufficiency, 17 and venous aneurysm. ...
... 11 To reach the pulmonary circulation, thrombus would have to cross a tortuous route through collateral veins, being retained therein in most cases. 11 Other symptoms that may result are: venous claudication, 12 hematuria caused by bladder varicosities, 13 lumbar pain due to venous collaterals in the vertebral and paravertebral plexus, causing compression of the thecal sac and at the output of the nerve roots, 14 cauda equina symptoms such as bladder or bowel dysfunction and lower limb weakness, 15 visceral compressions by the gonadal venous system, 16 venous ulcers due to deep venous insufficiency, 17 and venous aneurysm. 16 Diagnosis is made after Angio-CT, magnetic angioresonance, or phlebography, of which Angio-CT offers the best cost-effectiveness ratio and easier access. ...
Inferior vena cava agenesis is a rare condition and is often misdiagnosed. This anomaly is asymptomatic in the majority of cases and is usually diagnosed during imaging tests carried out for other purposes. The most frequent manifestation is deep vein thrombosis (DVT) in lower limbs and anticoagulation therapy is the most frequent treatment option. Other techniques such as thrombolysis and venous bypass are also described. We report two cases diagnosed at our institution during the last year, both of which presented with an episode of DVT. We opted for indefinite anticoagulation therapy and both patients remain asymptomatic, after 1 year of surveillance in the first case and 6 months in the second, with no new episodes of DVT. Although it is not a life-threatening anomaly, it is important to make an appropriate diagnosis and provide treatment to improve the symptoms and quality of life of these patients.
... Tufano et al. [13] report in their study from 2020 that patients with IVCA and DVT were more likely to be young, male, have unprovoked proximal or bilateral DVT, and were less likely to have a pulmonary embolism (PE), probably due to the difficulty of passing the embolus through the network of collaterals, as first reported in the review of the literature by Lambert et al. [14] . In the same paper, Lambert stated that only about 10% of cases had PE. ...
The inferior vena cava agenesis (IVCA) is a rare and often asymptomatic malformation due to the abundant development of the collateral circulation. However, it is frequently found in young people and carries a significant risk of deep venous thrombosis (DVT). It is estimated that about 5% of patients under 30 years of age presenting with DVT have this condition. We report a case of a previously healthy 23-year-old patient presenting with signs of acute abdomen and hydronephrosis due to the thrombophlebitis of an unusual iliocaval venous collateral, which developed secondary to IVCA. After treatment, the iliocaval collateral and hydronephrosis completely regressed on a 1-year follow-up. To our knowledge, this is the first such case reported in the literature.
... A case report in 2018 reported a patient with a duplicated CIV who developed edema and pain in the LLE following a hysterectomy and was found to have thrombi in the inferior vena cava and iliac vein [17]. It has been found that up to 5-6.7% of adults with spontaneous DVT have an anomaly of the inferior vena cava [18]. Ruggeri M et al. proposed in 2001 that congenital anomalies of the pelvic vasculature may be a risk factor for DVT due to formed collateral veins being unable to withstand an increase in blood flow during physical exertion, thus leading to venous stasis and clotting [19]. ...
Arising from the external and internal iliac veins, the common iliac veins (CIVs) carry blood from the lower extremities and pelvic region into the inferior vena cava at the level of the fifth lumbar vertebra. It is sometimes common to observe slight anomalies in vascular anatomy in patients; however, anomalies of the CIVs are rare. We present a case of a patient with significant edema of the left lower extremity due to extrinsic compression (May-Thurner syndrome [MTS]) involving a duplicated left CIV found during vascular angiography. Anomalies in pelvic vasculature are well documented in the medical literature; however, documented cases of a duplicated CIV remain few and far between. These anomalies in pelvic vascular anatomy are essential to be aware of to avoid surgical complications and understand their implications in associated pathologies.
... As presented in the case, the patient had an isolated left sided IVC with azygos continuation. Left sided IVC has been associated with increased prevalence of venous thromboembolism in lower extremities and venous insufficiency [9]. It is considered that the collaterals that physiologically compensate for venous return likely increase the risk of DVTs. ...
The merits of utilizing point of care ultrasound (POCUS) in acutely ill patients is leading to a widespread embrace. Assessment of IVC via POCUS as part of a comprehensive multi-organ approach can help guide volume tolerance. Anatomical/developmental variations of IVC can vary widely in prevalence. As the use of POCUS expands as a diagnostic modality, it is prudent for frontline POCUS users to be cognizant of the IVC anomalies. We present a case of left sided IVC with azygous continuation discovered with POCUS that was performed to assess the volume status of the patient. This case illustrates that the awareness of different anomalies of the IVC is necessary for POCUS users to prevent misinterpretation of aberrant vessels and avoid diagnostic pitfalls.
... Due to turbulent flow, they remain a persistent risk factor for the development of VTE in the lower limbs [22]. Co-existent pulmonary embolism (PE) is rare, since thrombi are trapped within collateral circulations (such as azygos vein) before reaching the pulmonary circulation [21,23]. Young age and unprovoked DVT, especially if proximal and bilateral, should raise suspicion of IVC agenesis [21]. ...
... In contrast to MTS which predominantly affects women, IVC agenesis and hypoplasia is more common in males [22,24]. Interestingly, despite the fact that individuals have had the variant lifelong, they often do not present until the third or fourth decade, there is probably an additional "trigger" for the thrombosis, such as starting the COCP in a female; one report suggests DVT may follow intensive and unusual physical activity as the collaterals can increase blood flow which then generates venous stasis within the IVC segment [23,25]. The gold standard modality for assessment is either CT or MRI. ...
Patients with venous anomalies are at increased risk of developing venous thromboembolism (VTE) and subsequent complications, but they are often under-recognised. While unprovoked VTE may trigger testing for inherited thrombophilias and malignancy screening, anatomic variants are considered less often. Venous anomalies increase the risk due to venous flow disturbance, resulting in hypertension, reduced flow velocity and turbulence. Recognition is important as endovascular or surgical intervention may be appropriate, these patients have a high rate of VTE recurrence if anticoagulation is ceased, and the anomalies can predispose to extensive VTE and severe post-thrombotic syndrome (PTS). In this case series, we present representative cases and radiological images of May-Thurner syndrome (MTS), inferior vena cava (IVC) variants and venous aneurysms, and review the available literature regarding optimal diagnosis and management in each condition.
