Massive Inferior Vena Cava Thrombosis Associated With Retroperitoneal Venolymphatic Malformation: Use of Intravascular Ultrasound for Diagnosis and Management

^aDepartment of Medicine, Division of Cardiovascular Diseases, Creighton University School of Medicine, Omaha, Nebraska
^bDepartment of Radiology, Division of Interventional Radiology, Creighton University School of Medicine, Omaha, Nebraska

↵∗Address for correspondence:
Dr. Raviteja R. Guddeti, Division of Cardiovascular Diseases, Creighton University School of Medicine, 7500 Mercy Road, Omaha, Nebraska 68124.

Key Words

Inferior vena cava (IVC) anomalies are uncommonly described. Many are associated with a higher rate of thromboembolic complications. Here we report a case of IVC thrombosis in a 20-year-old woman with a large isolated retroperitoneal venolymphatic malformation (VLM) with IVC envelopment in which a multimodality imaging approach was implemented to confirm the diagnosis and guide treatment strategy.

A previously healthy 20-year-old woman on oral contraceptive pills presented with a 5-day history of right lower quadrant abdominal pain with severe tenderness, nausea, and vomiting. Computed tomography of the abdomen with contrast showed large thrombus occluding the IVC and retroperitoneal cystic changes at the level of the renal veins (Figure 1A). Doppler ultrasound of the lower extremities was positive for extensive acute deep vein thrombosis. Factor V Leiden, prothrombin gene mutation, and anticardiolipin antibodies were negative. Inferior vena cavography after an initial mechanical thrombectomy of the iliac veins and IVC showed a patent-appearing IVC, but her abdominal pain persisted (Figure 1B). Magnetic resonance imaging of the abdomen showed renal and suprarenal IVC thrombus with early and late contrast filling of an 11.8 × 9.5 cm retroperitoneal mass encasing the IVC and aorta at the level of the renal veins consistent with a benign VLM, which was confirmed on computed tomography–guided biopsy (Figures 1C and 1D). Because of her continued symptoms, intravascular ultrasound (IVUS) of the IVC, using a 3.5-F, 20-MHz coronary IVUS catheter (Eagle Eye Platinum, Volcano, San Diego, California) was performed, revealing cystic and septated changes in the IVC wall and focal near-occlusive thrombus (Figure 1E, Online Video 1). There was an 8 mm Hg venous pressure gradient across the occlusion. Catheter-directed thrombolysis was performed, and follow-up IVUS 18 h later (Figure 1F, Online Video 2) showed significantly reduced thrombus burden and improved IVC flow with no residual pressure gradient. The patient was placed on rivaroxaban 20 mg daily for 3 months for deep vein thrombosis. She subsequently underwent sclerotherapy for VLM and remained symptom free at 6 months.

Figure 1

Inferior Vena Cava Thrombus With Associated Retroperitoneal Venolymphatic Malformation

(A) Computed tomography showing thrombus in the inferior vena cava (IVC) (red arrow) and retroperitoneal cystic changes at the level of the renal veins (green arrow). (B) Inferior vena cavography. (C) Magnetic resonance imaging T2 sequence demonstrating hyperintensity of the mass (yellow arrow) surrounding the IVC (red arrow). (D) Delayed enhancement of the mass (green arrow) surrounding the IVC. (E) Pre-thrombolysis and (F) post-thrombolysis IVUS showing septations (white arrow) in the IVC (see Online Videos 1 and 2, respectively).

Online Video 1

Pre-Thrombolysis Intravascular Ultrasound Showing Inferior Vena Cava Septations and High Clot Burden

Online Video 2

Post-Thrombolysis Intravascular Ultrasound Showing Improved Clot Burden in the Inferior Vena Cava

In this unusual case of VLM-associated IVC thrombus, IVUS was used to diagnose VLM caval wall involvement and thrombosis and assist in thrombolysis management.