A 16 year old male with a history of hereditary haemorrhagic telangiectasia presented initially to an outpatient clinic with nine months of progressive exertional nausea and dry heaving. Symptoms were most severe after intense exercise. The physical exam was unremarkable and no superficial abdominal collaterals were seen. Cardiology and allergy and immunology consultants attributed the symptoms to motion sickness. Stress echocardiogram and interrogated labs were unrevealing. The patient was given antihistamines with poor relief of symptoms.
Two years later while playing basketball, the patient re-presented to an outside hospital with sudden onset of nausea, back pain, and bilateral calf pain and swelling. Computed tomography (CT) venography demonstrated occlusive DVT bilaterally from the common iliac to the popliteal veins. The infrahepatic IVC was not visualised and replaced with collateral veins. Hypercoagulability workup was negative and the patient did not have a history of DVT. The patient underwent catheter directed thrombolysis and mechanical thrombectomy with improved symptoms and was discharged on anticoagulants. However, he was re-admitted within 24 hours with recurrent bilateral iliofemoral vein thrombosis and worsening lower extremity pain and oedema. He was transferred to the present authors' institution for further management.
Pre-intervention CT venogram confirmed extensive lower extremity DVT, partial absence of the intrahepatic IVC, complete absence of infrahepatic IVC, and dilated thrombosed paraspinal collateral veins draining into enlarged azygos and hemiazygous veins (Fig. 1). The patient was taken to the interventional radiology suite daily for three consecutive days.
On the first day, bilateral lower extremity venography was performed via posterior tibial vein access, demonstrating a clot burden from the bilateral posterior tibial veins to the common iliac veins (CIVs) (Fig. 2A). The IVC was not visualised from the confluence of the CIVs and no significant abdominopelvic collaterals were identified (Fig. 2B). Mechanical thrombectomy with Angiojet (Boston Scientific, Marlborough, MA, USA) was performed bilaterally in the iliac, femoral, popliteal, and posterior tibial veins with marginal success. Cragg-McNamara infusion catheters (Medtronic, Minneapolis, MN, USA) were placed bilaterally, terminating at the confluence of the CIVs, and tPA was infused at 0.5 mg/h in each catheter for 24 hours.
On the second day, repeat venography demonstrated near complete resolution of thrombus throughout both lower extremities and the outflow azygos vein (Fig. 3A). Within 10 minutes of terminating tPA, there was re-thrombosis of the azygos and CIVs (Fig. 3B). Thus, infusion catheters were again placed and tPA infusion was restarted. Given the rapid re-thrombosis, the patient agreed to an attempt at IVC reconstruction to restore venous outflow.
On the third day under general anaesthesia and with the patient in the supine position, venography of the proximal intrahepatic IVC was performed via a right internal jugular vein access, demonstrating complete occlusion of the infrahepatic and distal intrahepatic IVC (Fig. 4A). Venography was performed at the confluence of the CIVs via bilateral common femoral vein access, revealing a small remnant of the IVC (Fig. 4B). Recanalisation of the IVC was performed through this diminutive stub in a caudal to cranial direction using a variety of sharp recanalisation techniques, including a 16 gauge Colapinto needle (Cook Medical, Bloomington, In, USA), back end of a 0.035 Amplatz Super Stiff guidewire (Boston Scientific), and 9 and 12 French vascular sheath dilators as bougies. The atretic IVC was traversed from both the right internal jugular vein and common femoral vein accesses using a 0.035 Roadrunner wire (Cook Medical) and 0.035 Quick-Cross catheter (Spectranetics, Colorado Springs, CO, USA). The right renal vein was catheterised and venography was performed, confirming that the recanalised vessel was the IVC (Fig. 4C). A snare was used to gain body floss access and venoplasty of the atretic IVC was performed from the confluence of the CIVs to the infrahepatic IVC with a 10 mm balloon. Intravascular ultrasound (IVUS) was performed from the right atrium down through the IVC and bilateral common and external iliac veins, revealing extensive synechiae within the IVC and bilateral common and external iliac veins. Venoplasty with 16 mm and 24 mm balloons was performed in the IVC followed by repeat venography and IVUS that showed a reduction in synechiae and venous collaterals with significant recoil throughout the atretic IVC. Two overlapping 24 mm x 70 mm Wallstents (Boston Scientific) were placed in the recanalised IVC, starting in the intrahepatic IVC down to the iliac confluence. These were dilated to 24 mm and repeat venography and IVUS was performed. Two kissing 18 mm x 90 mm Wallstents were placed starting at the caudal aspect of the inferior 24 mm Wallstent and ending in each CIV. The IVC end of the 18 mm Wallstents was dilated to 14 mm and the common iliac end was dilated to 16 mm (Fig. 4D and E). Final venography and IVUS demonstrated a widely patent reconstructed IVC with good wall apposition of the stents (Fig. 4F and G).
The patient was discharged on post-operative day four on enoxaparin 80 mg SC BID (1 mg/kg), and clopidogrel 75 mg PO daily for one month. At the one month clinic visit, CT venography demonstrated a patent reconstructed IVC and bilateral iliac veins (Fig. 5, Supplemental Video 1). The patient was transitioned to lifelong apixaban 5 mg PO BID and aspirin 81 mg PO daily. He reported complete resolution of exertional nausea and denied lower extremity pain or swelling at the one and seven month post-procedural clinic visits and remains asymptomatic at 2.5 years of follow up. The patient will be seen annually going forward.