Objective: Agenesis of the inferior vena cava (IVC) is a rare vascular malformation. Deep vein thrombosis (DVT) and bilateral pelvic thrombosis develop quite frequently, making surgical therapy necessary.
Patients and methods: Between 1982 and 2006, 15 patients (nine male, six female, mean age 28 standard deviance 9 years) with agenesis of the IVC (IVCA) were treated surgically because of acute or subacute DVT. These patients underwent bilateral transfemoral ante- and retrograde thrombectomy of the iliofemoral and sometimes popliteal veins and replacement of the IVC with an external ring supported PTFE-graft. Bi- or unilateral arteriovenous fistulae were created in the femoral region. The fistulae were closed, on average, 8 months after trans-arterial venography was performed. These patients were examined clinically and by duplex ultrasound imaging during follow-up to assess graft patency and to allow CEAP classification. Patients were assessed for the development of post-thrombotic syndrome (PTS).
Results: No patient died during any part of their treatment or within 60 days. Primary patency of the venous reconstruction was 53%, secondary and long time follow-up patency was 83%. The mean duration of follow-up was 41 SD 12 months. Minor complications were observed in five cases (33%). PTS showed no progression during a follow-up of 41 SD 12 months in all patients. There was no change in the CEAP clinical stage during follow-up nor did any leg ulcer develop.
Conclusion: A surgical approach to restore venous patency is effective and appears to prevent the deterioration of CVI over time.
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