Abstract:Agenesis of the inferior vena cava is an uncommon congenital vascular malformation. Although theoretically it may favor venous stasis, it is not normally associated with venous thrombosis. The authors found only three reports of the coexistence of both entities, and only one of these rules out the existence of associated hypercoagulability. They report the case of a patient with a history of repeated venous thrombosis. Magnetic resonance angiography and venography demonstrated complete agenesis of the inferior… Show more
“…Ten patients with DVT, eight men and two women, whose mean age was 25 ± 4.5 [range: [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] years, were diagnosed as having IVCA ( Table 1). Five of them reported having a first-degree relative with a history of venous thrombosis.…”
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.
“…Ten patients with DVT, eight men and two women, whose mean age was 25 ± 4.5 [range: [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] years, were diagnosed as having IVCA ( Table 1). Five of them reported having a first-degree relative with a history of venous thrombosis.…”
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.
“…In addition, the radiological assessment post insertion of central venous lines can be quite confusing in such patients as illustrated in Figure 4. An aberrant venous return can lead to venous insufficiency of the lower limbs with potential for thromboembolic disease [4]. Finally, the recognition of aberrant venous anatomy can have important implications during surgery or endovascular procedures, as illustrated by a previously reported fatal outcome after ligation of the aberrant vessel in a patient with a hemiazygous continuation of the left IVC [5].Fig.…”
We report a case of hemiazygous-accessory hemiazygous continuation of a double IVC with absent azygous vein, incidentally discovered in an adult patient.
“…Abdominal vascular pathology should be strongly considered in young women or men who complain of sudden swelling or weakness of both legs, regardless of whether it is associated with low abdominal or recent back pain (9). Other IVC malformation should be considered in young patients with idiopathic deep venous thrombosis (10)(11)(12)(13).…”
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