Endovascular Treatment of Duplicated Inferior Vena Cava Compression From Retroperitoneal Fibrosis

Abstract

Retroperitoneal fibrosis (RPF) is a rare condition that can affect the iliocaval veins, leading to treatment of this condition. We report a unique case of RPF causing symptomatic compression of a duplicated inferior vena cava treated successfully with stenting. A 68-year-old African American man presented for evaluation of severe debilitating venous claudication and bilateral lower extremity swelling. He reported chronic and progressive symptoms of venous insufficiency that became lifestyle limiting a few months before presentation. His past medical history was significant for hypertension, anemia, and idiopathic RPF with mild renal insufficiency. His surgical history was significant for retroperitoneal exploration and ureterolysis for left-sided hydronephrosis as well as bilateral hydrocele resection. On physical examination, he had normal pulses and severe edema, right worse than left, but no varicosities. Duplex ultrasound was negative for deep venous thrombosis or reflux but demonstrated blunting of respiratory variations in the bilateral external iliac veins. Computed tomography scan showed near obliteration of the venous structures in the pelvis (Fig 1, A). The patient was fitted for compression stockings but could not get relief. Venography was performed and demonstrated a duplicated inferior vena cava with severe bilateral stenosis of the iliocaval vasculature (Fig 2). Intravascular ultrasound was performed to confirm the sites of stenosis and for sizing. Bilateral balloon angioplasty and iliocaval stenting were performed using two 16-mm Wallstents (Boston Scientific, Marlborough, Mass) on the right and two 14-mm Wallstents on the left. The completion venogram showed excellent flow bilaterally (Fig 3). Intravascular ultrasound confirmed complete resolution of all areas of stenosis. The patient was started on clopidogrel and was discharged the next day. The edema cleared within a week, and he experienced excellent relief of symptoms. After 1 year, he was switched to aspirin, and he continues to have minimal symptoms with no recurrence. On his latest follow up, 33 months after intervention, computed tomography scan demonstrated patent stents in the duplicated cava and no venous stenosis (Fig 1, B). Iliocaval compression from RPF is rare but can be debilitating. Endovascular stenting seems to be an effective and durable treatment.Fig 2Pelvic venogram demonstrating severe stenosis in the right (A; arrow) and left (B; arrow) iliocaval systems.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 3Completion venogram after iliocaval stenting showing brisk flow and resolution of areas of stenosis on the right (A) and left (B).View Large Image Figure ViewerDownload Hi-res image Download (PPT)