Case Report
Introduction: Venous thromboembolism (VTE) and pulmonary embolism (PE) in young adults are frequently associated with hereditary thrombophilia, autoimmune disease, and malignancy. Advances in imaging have allowed for identification of inferior vena cava (IVC) anomalies as another etiology. Observational data indicates that congenital inferior vena cava (IVC) anomalies are quite rare but are associated with as much as a 50-100-fold increase in risk for deep venous thrombosis (DVT). Even more worrisome is the propensity for subsequent PE.
Case: A 30-year-old male with tobacco use disorder presented with two weeks of worsening pleuritic chest pain and dyspnea on exertion, as well as an episode of hemoptysis with clot expectoration. An electrocardiogram showed sinus tachycardia, a rightward axis, and an S1Q3T3 pattern. Computed tomography (CT) imaging of the pulmonary arteries revealed bilateral segmental and subsegmental pulmonary emboli, and he was started on a heparin infusion. Bilateral lower extremity ultrasounds revealed no thrombus, however, given his young age without provoking factors, a CT of his abdomen and pelvis was obtained. Findings demonstrated what appeared to be complete occlusion of the IVC, with extension into the iliac vessels and reconstitution of hepatic IVC, suprahepatic IVC, and hemiazygos veins via collaterals appreciated. A venogram revealed a stenosis of the intrahepatic IVC with no visualized thrombus. After an extensive hypercoagulability workup was unremarkable, he was discharged on apixaban with plans to perform an outpatient intravenous ultrasound (IVUS). He was lost to follow-up and, due to lack of insurance, had difficulty affording his anticoagulant. He presented again several months later with two days of acute leg pain and swelling to his right lower extremity. Ultrasonography revealed an occlusive thrombus in the common femoral, greater saphenous, superficial femoral, and profunda veins requiring thrombectomy. Testing for echinococcus was negative.
Discussion
Congenital IVC stenosis is a rare cause of VTE in young adults and is usually discovered when evaluating a proximal DVT in the absence of thrombophilia, autoimmune disease, or malignancy. Even more rarely, it may manifest itself via PE. Recognition is critical, as delayed diagnosis increases the risk of recurrence and post-thrombotic complications. Even with formation of collaterals, the resultant stasis from inadequate blood flow past the lesion can predispose to proximal DVT. This case highlights the importance of considering central venous anomalies in unexplained VTE and PE in young adults without other risk factors and underscores the role of dedicated imaging for diagnosis. Anticoagulation remains first-line therapy for thrombosis and select patients may be chosen to undergo endovascular stenting, as balloon angioplasty is often insufficient.
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