Endovascular IVC Reconstruction in an 18 Year Old Patient with Subtotal IVC Atresia

Abstract

Introduction: Inferior vena cava (IVC) atresia is an uncommon venous anomaly that is an under recognised cause of unprovoked acute deep venous thrombosis (DVT) in young adults. The purpose of this case report is to highlight endovascular IVC reconstruction as a feasible treatment option, particularly in challenging cases when other therapeutic modalities have failed.

Report: This is the report of an 18 year old patient with near complete IVC atresia and a longstanding history of exertional nausea of unknown aetiology, who presented with extensive acute DVT. He was treated successfully by endovascular IVC reconstruction after failing initial anticoagulation and thrombolysis. Symptom resolution and venous patency were maintained at 2.5 year follow up.

Discussion: IVC atresia is an important aetiology to consider in a young patient presenting with unprovoked DVT. Endovascular stenting can restore venous patency and is feasible even when there is near complete IVC atresia. This case was uniquely challenging in the length of atretic IVC that was reconstructed and also highlights an atypical clinical presentation of IVC atresia.

Keywords: Deep venous thrombosis; Endovascular intervention; IVC agenesis; IVC atresia; IVC reconstruction; Post thrombotic syndrome.

Figure 1

Pre-procedural axial CT venogram images proceeding from a caudal to cranial direction. (A) The bilateral common iliac veins (arrows) are dilated by acute thrombus. (B) At the expected location of the confluence of the common iliac veins, there are enlarged, thrombosed paraspinal venous collaterals (arrows). (C) These collaterals eventually drain into the azygos system (arrows). (D) Superiorly, the azygos vein is patent, but distended (small arrows). The hemiazygous vein is also engorged (large arrow). (E) The IVC is only identified at the superior aspect of the liver, at the confluence of the hepatic veins (arrow).

Figure 2

Digital subtraction angiography images from Day 1 thrombolysis and thrombectomy. (A) Initial venography from the left posterior tibial vein demonstrates clot through the femoral veins (arrows). (B) Extensive clot burden is also seen in the bilateral common iliac veins (white arrows). The IVC is absent at its expected location (black arrow).

Figure 3

Digital subtraction angiography images from Day 2 lysis check. (A) Following 24 hours of catheter directed thrombolysis, there is near complete resolution of thrombus, and a large outflow azygos vein (arrows) is identified. (B) However, within 10 minutes of terminating the tissue plasminogen activator infusion, the azygos vein is no longer seen on repeat venography. Re-thrombosis also occurred in the right common iliac vein (white arrow). The IVC remained absent at its expected location (black arrow).

Figure 4

IVC reconstruction on Day 3. (A, B) Fluoroscopic images demonstrate abrupt termination of the proximal IVC at the level of the hepatic veins (black arrow). A small remnant of the IVC (white arrow) is now identified at the confluence of the common iliac veins. The dashed lines outline the expected course of the atretic IVC and the length to be reconstructed. (C) Using several recanalisation techniques and after traversing the atretic IVC (black arrow), venography of the right renal vein (white arrow) confirmed that the recanalised vessel was the IVC. (D, E) After extensive venoplasty, intravascular ultrasound evaluation, and deployment of Wallstents in the IVC and common iliac veins, the final venogram demonstrates a patent, fully reconstructed IVC. (F, G) Intravascular ultrasound demonstrates a recanalised IVC with a patent IVC stent (open red arrow) and well apposed kissing iliac stents (solid red arrows) at the inferior aspect.

Figure 5

Post-procedural CT venogram images at one month. Representative axial images (A–D) and coronal image (E) at the iliac veins (A), distal IVC (B), renal IVC (C), and intrahepatic IVC (D) demonstrate patency of the kissing 18 mm Wallstents and 24 mm Wallstent, which are free of thrombus.