Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature

Abstract

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 and two women; mean age, 25 ± 4.5 years), DVT followed intense and unusual (major) physical activity for eight of them. DVT was bilateral in six patients and unilateral in four. Ultrasonography was unable to detect IVCA, which was visualized by computed-tomography scans for seven patients, and magnetic resonance imaging and angiography for 10. Hereditary thrombophilia screening, to detect factor V Leiden or prothrombin gene heterozygosity (G20210A mutation), was positive for only two patients. Wearing elastic stockings and taking an indefinite or long-term vitamin K antagonist were prescribed for all 10 patients and nine complied with the latter. To date, 62 patients with IVCA-associated DVT have been reported in the English literature. Analysis of them and our patients yielded a typical spectrum of IVCA-associated DVT characteristics: IVCA occurs in young adults, particularly males, and is revealed by proximal DVT following major physical exertion. All were treated with a prolonged vitamin K antagonist and advised to wear elastic stockings. No precise duration of anticoagulation has been established.

Deep Venous Thrombosis Associated With Inferior Vena Cava Abnormalities And Hypoplastic Kidney In Siblings

“…Some authors recom mend at least 6 months of anticoagulant treatment while in most cases life-long anticoagulation therapy is proposed even when thrombo philia investigation is negative in order to reduce the recurrence risk [1,3,7,8]. There are some reports of recurrence of thrombosis after discontinuation of the treatment [12].…”

“…There is a high risk of DVT recurrence because of inadequate venous return and consequent venous stasis. In case of lumbar DVT back pain is a common finding [3].…”

“…It represents a well-known cause of ileo-femoral deep vein thrombosis (DVT) [2,3]. The incidence of IVC abnormalities in general population is 0.2 -1%; this incidence being higher in young adults with DVT, namely 5% [2,4].…”

Agenesia da veia cava inferior

“…Its most common cause is dysgenesis during embryogenesis (due to the simultaneous occurrence of a defect in the venous system of the three embryonic segments), but it can also occur due to in utero or perinatal thrombosis (without embryological abnormalities). 6 Since the patient shows no symptoms, the diagnosis of agenesis is often accidentally attained during abdominal surgery or radiological procedures; 5 in these cases, collateral circulation develops from the lumbar, azygos, and hemiazygos systems, which compensate for the malformed lower vena cava function. 7 However, in the event of insufficient collateralization, the slow blood flow in the lower limbs and pelvis leads to venous stasis and to an increased propensity for thrombosis.…”

Congenital infra renal agenesis of inferior vena cava - an uncommon entity in clinical practice

“…9 Anticoagulants and elastic stockings help in the symptomatic management of IVC agenesis. Patients should be advised to avoid other risks of thrombosis like immobilization and oral contraceptive [10] . Few have required surgical intervention for relief of symptoms.…”