Background
Inferior vena cava agenesis (IVCA) is a rare anomaly that presents in 5% of cases of unprovoked lower extremity deep venous thrombosis (DVT), mostly bilateral and significantly under the age of 30 years. Conventional treatment with systemic anticoagulation may not be sufficient in limb-threatening stages. Catheter-directed thrombolysis (CDT) could be the modality of choice in this condition.
Aim
The purpose of this case series is to report our experience with CDT to manage patients with congenital absence of inferior vena cava presented with bilateral acute iliofemoral venous thrombosis.
Patients and methods
From February 2015 to March 2017, three patients were referred to Ain Shams University Hospitals and underwent CDT to treat bilateral iliofemoral DVT with IVCA after failure of treatment with systemic anticoagulation. Periprocedural and postprocedural details were recorded. Follow up with clinical examination and duplex ultrasound was performed regularly for 2 years.
Results
The mean procedural time was 52 h. Technical and clinical success were achieved in all the procedures. No further balloon angioplasty or stent placement was required. No major bleeding occurred. None of the patients had symptoms of pulmonary embolism. The mean postoperative hospital stay was 7 days. During the follow up, none of the patients developed recurrent DVT. Duplex ultrasound showed a patent deep system with only one patient who got unilateral femoral vein reflux, while computed tomography venography showed patency of pelvic and abdominal collaterals in all patients.
Conclusion
CDT is a safe and effective modality of treatment for patients with bilateral acute iliofemoral DVT associated with IVCA. It helps to reestablish the patient’s baseline venous drainage for limb salvage, rapid symptomatic relief, and prevention of postthrombotic syndrome.