Objectives: This is one of the few case reports of congenital inferior vena cava atresia (IVCA) with extensive bilateral lower extremity ulcers.
Background: IVCA is a congenital anomaly characterized by poor venous return in the lower extremities with increased risk of deep vein thrombi (DVTs) and, rarely, non-healing ulcers.
Results: A 45-year-old woman with history significant for DVTs presented with extensive bilateral lower extremity ulcers, and a CT scan revealed congenital IVCA. Oedema management, DVT prevention, routine wound care and skin grafts resolved the ulcers.
Conclusion: Congenital venous abnormalities should be included in the differential diagnosis of multiple unprovoked DVTs and non-healing ulcers.
Inferior vena cava (IVC) agenesis is a rare congenital abnormality affecting the infrarenal segment, the suprarenal or the whole of the IVC. It has an estimated prevalence of up to 1% in the general population that can rise to 8.7% when abnormalities of the left renal vein are considered. Most IVC malformations are asymptomatic but may be associated with nonspecific symptoms or present as deep vein thrombosis (DVT). Up to 5% of young individuals under 30 years of age with unprovoked DVT are found to have this condition. Regarding the treatment of IVC agenesis-associated DVT, there are no standard guidelines. Treatment is directed towards preventing thrombosis or its recurrence. Low molecular weight heparin and oral anticoagulation medication, in particular vitamin K antagonists (VKAs) are the mainstay of therapy. Given the high risk of DVT recurrence in these patients, oral anticoagulation therapy is suggested to be pursued indefinitely. As far as we know, this is the first case reporting the use of a direct factor Xa inhibitor in IVC agenesis-associated DVT. Given VKA monitoring limitations, the use of a direct Xa inhibitor could be an alternative in young individuals with anatomical defects without thrombophilia, but further studies will be needed to confirm its efficacy and safety.
Among thrombophilic risk factors for deep venous thrombosis (DVT), agenesis of the inferior vena cava (AIVC) is very rare, but it must be considered in specific settings. Here, we present the case of an 18-year-old woman who was admitted to the Emergency Department with swelling and pain of her left leg. Clinical examination and ultrasonography detected extensive proximal DVT of the left leg. After attempted mechanical thrombectomy failed, an abdominal CT scan was obtained, which demonstrated bilateral thrombosis of the iliac-femoral axis in the context of congenital AIVC.