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Original Report |
1 Department of Diagnostic Imaging, Sheba Medical Center, Tel Hashomer 52621,
Israel.
2 Sackler School of Medicine, Tel-Aviv University, Tel Aviv 69978, Israel.
3 Present address: Department of Diagnostic Imaging, Assaf Harofeh Medical
Center, Zerifin 70300, Israel.
4 Department of Hematology, Institute of Thrombosis and Hemostasis and the
National Hemophilia Center, Sheba Medical Center, Tel Hashomer 52621,
Israel.
5 Department of Diagnostic Imaging, Sapir Medical Center, Kfar Saba 44281,
Israel.
6 Department of Internal Medicine D, Sheba Medical Center, Tel Hashomer 52621,
Israel.
7 Department of Vascular Surgery, Assaf Harofeh Medical Center, Zerifin 70300,
Israel.
Received May 13, 2002;
accepted after revision August 20, 2002.
Address correspondence to G. Gayer.
Abstract
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CONCLUSION. An anomaly of the inferior vena cava should be considered in young patients who present with deep vein thrombosis of the femoral and iliac veins. Coagulation abnormalities, frequently found in these patients, may be a contributory factor.
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We report nine patients with an inferior vena cava anomaly who presented with widespread deep vein thrombosis of the pelvic and femoral veins. To the best of our knowledge, this is the first series of patients whose CT findings showed the uncommon association of congenital anomalies of the inferior vena cava with deep vein thrombosis.
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The clinical presentation, onset of disease (spontaneous or secondary), previous thrombotic episodes, and family history of thrombosis were collected from medical records. Available sonograms, chest radiographs, and venocavograms were also reviewed.
CT of the chest and abdomen was performed using Twin (n = 4) (Elscint, Haifa, Israel), 2400 (n = 3) (Elscint), or MX8000 (n = 2) (Marconi, Haifa, Israel) scanners. Contiguous 6.5- to 11-mm slices were obtained. Scans were obtained both before and after IV administration of contrast medium, usually with a bolus injection of 80 mL of Telebrix ([meglumine ioxitalamate, 300 mg I/mL]; Guerbet, Roissy, France).
Extensive coagulation studies, including activated protein C resistance, antithrombin, protein C and free protein S antigen assays, lupus anticoagulant, antiphospholipid antibodies, homocysteine serum concentration, determination of factor V Leiden (G1691A) mutation, prothrombin gene (G20210A) mutation, and methylenetetrahydrofolate reductase gene (C677T) mutation, were performed in all patients using standard methods.
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Chest radiography was performed in six patients before CT and showed a widening of the right mediastinum at the level of the azygos vein in three patients. Sonography confirmed deep vein thrombosis in all eight patients, but inferior vena cava anomalies were not detected. Collateral veins were suggested to be enlarged lymph nodes in one patient. CT findings of the spine in two patients were normal. MR imaging of the spine performed in one patient showed small round retroperitoneal masses interpreted as enlarged lymph nodes. A venocavogram obtained in one patient showed a thrombus extending from the right common iliac vein to the inferior vena cava to the level of the right renal vein. Cranially, the suprarenal segment of the inferior vena cava was not visualized in this patient, but collateral veins, mainly a prominent azygos vein, were opacified with contrast material. Lung perfusion showed findings suggestive of pulmonary emboli in one patient, and findings of pulmonary CT angiography were normal in the second patient who had clinical signs suggestive of pulmonary emboli.
CT was performed for further investigation in six patients, three of whom were suspected of having a malignant process. Indications in the other three patients included flank pain and hematuria, suspected intraabdominal abscess, and severe lower back pain, respectively. CT revealed three different anomalies of the inferior vena cava, including absence of the suprarenal segment in five patients (Fig. 1A), absence of the infrarenal segment in three (Fig. 2A), and a double inferior vena cava with a retroaortic left renal vein and absence of the suprarenal segment in one (Figs. 3A and 3B). Widespread thrombosis involving the common, external, and internal iliac veins and the femoral veins was seen in all patients (Figs. 1C, 2B, and 3C). Thrombosis was bilateral in six patients (Figs. 1C and 2B) and unilateral in three (Fig. 3C). In addition, a thrombus extended to the left inferior vena cava and left renal vein in the patient with a double inferior vena cava (Figs. 3A and 3B) and to the infrarenal inferior vena cava in two patients who had no suprarenal segment (Fig. 1B). The azygos and hemiazygos system was prominent in all patients (Figs. 1D and 4). The following collateral veins were observed: ascending lumbar veins (Figs. 1C and 2A), internal paravertebral venous plexus (Fig. 1C), and abdominal wall veins (Fig. 3C). Additional anomalies were present in only one patient, who had a tiny right kidney and a large left kidney due to compensatory hypertrophy and a cavernous transformation of the portal vein (Fig. 4).
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The coagulation studies revealed a thrombophilic defect predisposing to thrombosis in seven of the nine patients. Prothrombin gene mutation was found in three patients, high homocysteine serum concentration in two, and factor V Leiden mutation in two. Two patients had two different defects. One patient with factor V Leiden mutation also had antiphospholipid antibodies, and one patient with high homocysteine serum concentration also had a methylenetetrahydrofolate reductase gene mutation.
Secondary risk factors for deep vein thrombosis were present in two patients. One patient, a soldier, reported strenuous physical effort before the appearance of deep vein thrombosis, and the other patient had been receiving oral contraception. There was no family history of deep vein thrombosis in any patient.
Seven of our nine patients are currently undergoing treatment with oral anticoagulant therapy of 6 months' (the most recently diagnosed patient) to 6 years' duration with no evidence of recurrence. The other two patients received oral anticoagulant therapy for only 6 months after diagnosis.
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Lately, anomalies of the inferior vena cava have been recognized as a
possible risk factor for deep vein thrombosis, particularly in young adults
[2,3,4,5,6],
with only anecdotal CT descriptions
[6,7,8,9,10,11].
Ruggeri et al. [2] found an
anomalous inferior vena cava in four of 75 young patients with a first episode
of deep vein thrombosis. Those authors estimated the prevalence of an
anomalous inferior vena cava in that group of patients to be around 5.3%, but
they assumed that their figures were necessarily conservative because some
cases might have been missed by an incomplete radiology study or inadequate
awareness of the possible causative relationship of an inferior vena cava
anomaly with deep vein thrombosis. Obemosterer et al.
[4] prospectively evaluated 31
patients with ileofemoral deep vein thrombosis on venography and MR
angiography and found five with anomalies of the inferior vena cava. Other
authors also found a high prevalence (
9.5%) of an anomalous inferior vena
cava in young patients with deep vein thrombosis
[3,
6] instead of an expected rate
of about 0.3% [11].
Regarding the pathophysiology of deep vein thrombosis in individuals with an anomaly of the inferior vena cava, blood return may be inadequate in spite of prominent collaterals [4]. This inadequate blood return may increase the blood pressure in the veins of the lower extremities, with ensuing venous stasis and subsequent deep vein thrombosis, which is bilateral in more than 50% of patients [6]. This prevalence of bilateralism is in contrast to a reported incidence of less than 10% in patients with deep vein thrombosis with a normal inferior vena cava.
Sonography is usually the first imaging modality in the evaluation of young patients with deep vein thrombosis, but anomalies of the inferior vena cava may be missed on sonography. These anomalies have, however, characteristic features on CT, as shown in our patients. When a segment of the inferior vena cava is absent, the iliac veins can be seen to drain directly into dilated ascending lumbar veins. A double inferior vena cava appears on CT as two tubular structures parallel to the aorta, with the left inferior vena cava continuing from the left common iliac vein and the left renal vein draining into the left inferior vena cava. Identification of a venous anomaly can usually be made by tracing the abnormal vessel to its destination.
Contrast-enhanced CT can show inferior vena cava anomalies and rule out a pelvic mass obstructing the venous blood flow. Contrast-enhanced CT is the modality of choice in patients with deep vein thrombosis, particularly when it is bilateral or when it occurs in more proximal veins.
Extensive collateral flow was observed in all our patients, with the azygos and hemiazygos systems being particularly prominent. Collaterals included the ascending lumbar veins, internal paravertebral venous plexus, and anterior abdominal wall veins. The markedly dilated paravertebral veins may have caused the lower back pain that was present in eight of the nine patients.
The inferior vena cava anomalies and the associated collateral veins may be missed or misinterpreted on imaging studies. A dilated azygos vein may present as widening of the mediastinum on chest radiography, suggesting a mediastinal mass. This finding, together with deep vein thrombosis, led the clinician to suspect a malignant process in three of our patients. Duplication of the inferior vena cava or the left inferior vena cava, particularly when one of the segments is thrombosed, can be confused with aortolumbar lymphadenopathy. All these diagnostic pitfalls can be avoided by performing helical CT with optimal IV contrast administration.
All patients in our study were screened for thrombophilia markers, and seven were found to have markers positive for thrombophilia. In reviewing the literature, we found reports of 20 patients with anomalies of the inferior vena cava and deep vein thrombosis who underwent evaluation for thrombophilia [2,3,4,5,6, 9, 10, 12]. Screening for thrombophilia markers was, however, incomplete in many of the patients reported; therefore, these findings may underestimate the true incidence of the markers. Of the combined group of 29 patients (our nine patients and the 20 reported), 13 were found to have positive results for thrombophilia markers: six were found to carry factor V Leiden gene mutation, three had the prothrombin gene mutation, one had a low protein S level, two had a high level of serum homocysteine concentration, one had the methylenetetrahydrofolate reductase gene mutation, and two had positive results for antiphospholipid antibodies (two of our patients had two markers each positive for thrombophilia). Although Ruggeri et al. [2] suggested that an anomalous inferior vena cava may be a sufficient cause for development of deep vein thrombosis, data from our literature review suggest an interaction between an anomaly of the inferior vena cava and thrombophilia in the pathogenesis of deep vein thrombosis. Further investigation with a larger series of patients is needed to confirm this hypothesis, which might affect clinical considerations regarding duration of oral anti-coagulant treatment.
In conclusion, deep vein thrombosis of the inferior vena cava, iliac veins, and femoral veins may be associated with congenital anomalies of the inferior vena cava, specifically in young patients with bilateral deep vein thrombosis. A thorough investigation for thrombophilia markers may also be useful to complete the evaluation of these patients.
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