Abstract
Double inferior vena cava (DIVC) with deep venous thrombosis (DVT) is rare, and there is only one reported case of DIVC with DVT treated by catheter-directed thrombolysis. We report a case of a 32-year-old man with an extensive venous clot involving the infrarenal segment of a double IVC who received filter implantation and catheter-directed thrombolysis.
Double inferior vena cava (DIVC) occurs in 0.2% to 3% of the population. 1 We present one case of DIVC with deep venous thrombosis (DVT) that was treated by filter implantation and catheter-directed thrombolysis (CDT).
Case Presentation
A 32-year-old male patient without significant past medical history was sent to our hospital with pain and swelling of the right lower extremity. Physical examination revealed a stiff thigh and tenderness of right femoral triangle. There was no evidence of right calf tenderness or edema, and Homan's sign was negative bilaterally. The circumference of the right thigh (taken 15 cm above the knee) was 6 cm larger than the left thigh, while the circumferences of both calves were the same. A venous duplex Doppler examination revealed the presence of an occluding thrombus in the right superficial femoral vein, as well as the common femoral vein and iliac vein. Dual-source CT scan established embolism of both pulmonary arteries. The D-dimer concentration was 1.17 mg/L. Filter implantation and CDT were advised.
The initial venogram via the left femoral vein showed that the left iliac vein did not join the right one at the level of the L4 vertebra to form the IVC but rather ascended along the left side of the vertebral column. A second venogram (Figure 1) was performed, which revealed the left IVC connected with the right IVC at the level of the L1 vertebra. At the same level, the left renal vein drained into the left IVC. The third venogram (Figure 2), via the right jugular vein, established that there were two IVCs that formed a common IVC. The diameters of the left and the right IVC trunk were 7 and 25 mm, respectively, while the common IVC was 8 cm long. The venogram also showed a thrombus in the right IVC just below the right gonadal vein. After a retrievable vena cava filter (Gunther Tulip, Cook) was implanted in the right IVC, a thrombolytic catheter (Unifuse, AngioDynamics) was placed along the right common femoral vein, the right iliac vein and the right IVC. A bolus of urokinase (300,000 U) was injected through the catheter, and the patient was sent back to the ward for further thrombolytic treatment with urokinase infusion at 12,500 U/h. Follow-up venography (Figure 3) after 72 h revealed recanalization of the right iliac vein with a moderate residual clot and a thrombosis defect (Figure 4) in the filter. Though all the clinical manifestations had disappeared by then, an additional 48 h of thrombolytic treatment was given. The final venogram (Figure 3) showed that all the vessels from the right femoral vein to the right IVC were patent and the residual thrombus was less than 50% of its original size. The patient refused filter retrieval because of worries about the risk of the thrombus fall-off during the operation.
Left IVC (LRV, left renal vein). Common IVC (HV, hepatic vein; RRV, right renal vein; GV, gooadal vein; LIVC, left inferior vena cava; T, thrombus). Venogram via the thrombolytic cather (L, day0; M, day 3; R, day 5). Thrombus defect in the filter (white arrow).



The patient was given oral anticoagulation treatment and compression therapy. Further laboratory results found no evidence of a hypercoagulable state. The patient had no DVT recurrence or clinical presentation of postthrombotic syndrome after 12 months’ follow-up. A CTV (Figure 5) confirmed the position of the double IVC and a 3D-reconstruction of CTV show the patency of the right IVC and the communication vein between the two IVCs (Figure 6).
CTV taken in the fellow-up(12 m). 3D-reconstruction of CTV in the fellow-up (15 m).

Discussion
DIVC occurs in 0.2% to 3% of the population. 1 IVC anomalies, such as DIVC, are more common in men than in women. 2 The embryonic creation of the IVC is a complex process, and failure of normal regression of any of three paired venous structures (vitelline veins, umbilical veins and posterior cardinal veins) can lead to DIVC. 3
Although DIVC may potentially influence retroperitoneal surgery and venous intervention, most cases are clinically silent. Some authors think such anomalies may cause a venous stasis, 4 which could ultimately predispose to venous thrombosis, especially in young adults.4,5 The case we presented had significant signs of thigh stiffness and tenderness, while the calf was normal. We think those symptoms may be the result of previous venous stasis that increases the tolerance to the acute DVT. The DVT developed on the right side, where the IVC diameter was smaller than the left, similar to cases presented by other authors.5–7 However, a direct relationship between DVT formation and the diameter of the IVC cannot be assumed because of different combinations of the diameters of the IVC and the communication veins.
There are only a few case reports of DIVC with DVT in the English-language literature. According to these papers, the cases often present with recurrent DVT or pulmonary embolism (PE) despite filter implantation, which may be the result of the communication veins between the DIVC. Chen et al. summarized 109 cases of DIVC and found 74 (67.9%) had communication veins. 3 With the development of evidence-based medicine, Doppler ultrasound has become the preferred diagnostic tool for venous disease. Because of the position of the IVC and the interference from intestinal gas, DIVC are often ignored during US scanning, so the venogram remains the gold standard.
Vena cava filters are the main means of PE prevention in patients with DIVC and DVT. Some authors proposed that if the DVT is in the small IVC, filter implantation can be reserved because the risk of symptomatic PE is very small. 6 Because the catheter operation inside the thrombus can induce thrombus fall-off, 8 we routinely use filters during CDT. For patients <65 y, we use retrievable filters. In the present case, the common IVC was short (8 cm). To prevent any positive influence on the venous flow of the left IVC, as well as the renal vein and hepatic vein, we placed the filter in the right IVC immediately below the right renal vein. That decision placed one leg of the filter in the thrombus, but the patient experienced no further complications, such as thrombosis extension or PE. We are required by law to use Gunther Tulip retrievable filters. We planned to retrieve the filter after CDT, but the patient refused this to avoid the risk of thrombus fall-off during the operation.
The aims of DVT treatment are to 1) prevent the extension of the thrombosis; 2) prevent PE; and 3) prevent postthrombotic syndrome. There are different treatments for DIVC with DVT: 1) anticoagulation; 2) filter implantation in the both IVCs 9 or in the common IVC; 10 3) embolization of the communication between the DIVC; 11 and 4) stent implantation. 5 Few data have been reported about the long-term follow-up of DIVC with DVT, and only one case report 10 mentions leg edema in a patient with DIVC and DVT treated by filter implantation and anticoagulation. CDT is effective to decrease the incidence of postthrombotic syndrome. 12 We searched all the papers written in English and found only one case of thrombolytic treatment for DIVC and DVT, 6 which did not mention if the patient had signs of postthrombotic syndrome.
Conclusion
DIVC with DVT is rare. We treated one case with filter implantation and CDT. One-year follow-up revealed no signs of postthrombotic syndrome.
Footnotes
Funding
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
