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Case Reports
. 2018 Apr 22:39:12-15.
doi: 10.1016/j.ejvssr.2018.03.005. eCollection 2018.

Inferior Vena Cava Agenesis: An Unusual Cause of Deep Vein Thrombosis and Pulmonary Embolism in Young Adult Patients

J Ramos Aranda  1 , C Ramírez Cerda  2 , S Cohen Mussali  2 , J Valdés Flores  2
Affiliations
Case Reports

Inferior Vena Cava Agenesis: An Unusual Cause of Deep Vein Thrombosis and Pulmonary Embolism in Young Adult Patients

J Ramos Aranda et al. EJVES Short Rep. .

Abstract

Introduction: Inferior vena cava agenesis (IVCA) is one of the many anomalies of this vessel. It is one of the most uncommon anomalies, with an estimated prevalence of 0.0005-1% in the general population. Around 5% of the patients younger than 30 years with a diagnosis of deep vein thrombosis (DVT) have a total or segmental IVCA.

Report: Here two unique cases of young and previously healthy male patients are reported: one with bilateral lower extremity DVT, the second with lower extremity DVT and pulmonary embolism. Both patients were found to have segmental agenesis of the inferior vena cava on computed tomography angiography (CTA). Treatment consisted of ultrasound enhanced thrombolysis (EKOS + alteplase) and venous angioplasty. Both patients were discharged with long-term (up to 24 months) oral anticoagulation and compression stockings. Follow up at 3 and 12 months revealed no new thrombotic episode.

Discussion: IVCA can be asymptomatic but the majority of the symptomatic patients present with DVT. IVCA confers a risk factor for DVT. IVCA should be considered and ruled out as a rare but important risk factor and cause of DVT in previously young healthy patients. Once diagnosed, aggressive treatment must be started because of the high risk of post-thrombotic syndrome.

Keywords: Deep vein thrombosis; Inferior vena cava agenesis; Ultrasound enhanced thrombolysis.

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Figures

Figure 1
Figure 1
Acute prominent engorged abdominal collateral veins.
Figure 2
Figure 2
Computed tomography angiography, coronal reconstruction, shows a confluent inferior vena cava stump (asterisk), dilated gonadal vein (right black arrow), dilated lumbar vein (left black arrow) and iliac veins (white arrowheads), and thrombosed right gonadal vein (long white arrow).
Figure 3
Figure 3
Computed tomography angiography, coronal reconstruction shows discontinuity of the inferior vena cava at suprarenal level (white arrow), duplicated inferior vena cava draining in to the left renal vein (black arrow).
Figure 4
Figure 4
Computed tomography angiography, coronal reconstruction shows the drainage of the left renal vein (white arrowhead) into the azygous system.
Figure 5
Figure 5
Renal venography shows both renal veins draining directly into the thrombosed renal inferior vena cava, the right renal vein (black arrow) drains through a collateral into the right ventricle and the left renal vein drains into the azygous system (white arrow), also through a collateral.
Figure 6
Figure 6
Right renal vein venography showing direct drainage into the right ventricle through a collateral (white arrow).
See this image and copyright information in PMC

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