1. Inferior Vena Cava Agenesis and Total Caval Reconstruction
Jeffrey Forris Beecham Chick, MD, MPH, DABR; Muhammad Noor, BS; Minhaj S. Khaja, MD, MBA; David M. Williams, MD, FSIR
Suggested Presenter: Muhammad Noor, BS

2. Disclosures
None

3. Brief Description
A 15-year-old male presented with right lower extremity swelling
Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated absence of the suprahepatic/hepatic/infrahepatic inferior vena cava (IVC) with many compensating collateral veins
Total endovascular caval reconstruction was performed, was technically and clinically successful, and right lower extremity swelling resolved
6 and 12-month venography showed patent caval stents
Endovascular caval reconstruction is a viable treatment option for IVC agenesis

4. History
A 15-year-old male presented to an outside institution with right lower extremity swelling
Patient was found to have complete absence of the suprahepatic/hepatic/infrahepatic IVC with many compensating collateral veins on both CT and MRI
Patient wore full right lower extremity compression stockings (20-30 mm) daily, for 3 years, without improvement in symptoms

5. Physical Examination Gross examination Measurements: CEAP score: C3
Significant right lower extremity swelling (thigh, calf, and foot)
Measurements:
10 cm proximal to proximal patellar edge: 54.0 cm
10 cm distal to tibial tuberosity: cm
CEAP score: C3

6. Magnetic Resonance Imaging
Axial T1-weighted post-contrast
Coronal T1-weighted post-contrast
Absence of hepatic and infrahepatic IVC (arrows)

7. Magnetic Resonance Imaging
Axial T1-weighted post-contrast
Axial T1-weighted post-contrast
Proximal and distal continuation of IVC (arrows)

8. IR Consultation
IR was consulted for IVC recanalization and caval reconstruction in an effort to improve right lower extremity swelling
Given the young age of the patient, and the potential for future growth, recommended treatment after the age of years once he reached full height potential
Patient presented 1 year later with increased right lower extremity swelling and endovascular caval reconstruction was performed

9. Digital subtraction image
Initial Venography
Fluoroscopic image
Digital subtraction image
Termination of the IVC (arrows) with multiple lumbar and peritoneal collateral vessels

10. Digital subtraction image
Caval Reconstruction
Digital subtraction image
Fluoroscopic image
Angioplasty of the IVC was performed using 14 mm, 16 mm, and 18 mm balloons (arrows).
3 overlapping 20 mm Wallstent endoprostheses were subsequently placed (arrows).

11. Completion Venography
Digital subtraction images
Brisk flow of contrast throughout the reconstructed IVC

12. IVC Agenesis
Abnormalities of the IVC represent a complex and poorly understood clinical entity
Development of the IVC involves the formation and subsequent regression of 3 paired cardinal veins (sub-cardinal, supra-cardinal, and post-cardinal veins) and vitelline veins, each one contributing to the final 5 segment adult venous system (supra-hepatic, hepatic, supra-renal, renal, and infra-renal segments)
IVC is subject to anatomical variations and malformations including total IVC agenesis, IVC atresia with secondary thrombosis, IVC atresia without thrombosis, left-sided IVC, double IVC, IVC interruption with azygous and hemiazygous continuation, and congenital extrahepatic portocaval shunts

13. Total Caval Reconstruction
Besides long-term anticoagulation and compression stockings for the treatment of deep venous thrombosis, no consensus has been reached in regards to the best treatment of IVC agenesis or IVC atresia
Literature suggests open thrombectomy and open caval reconstruction, however, as two possible treatment modalities
Endovascular Iliocaval stenting and reconstruction offers a viable treatment option for congenital, non-thrombotic, and thrombotic caval and venous outflow diseases

14. References
Williams DM. Iliocaval reconstruction in chronic deep vein thrombosis. Tech Vasc Interv Radiol Jun;17(2):
Neglén P, Hollis KC, Olivier J, Raju S. Stenting of the venous outflow in chronic venous disease: long-term stent-related outcome, clinical, and hemodynamic result. J Vasc Surg Nov;46(5):
Juhan C, Hartung O, Alimi Y, Barthélemy P, Valerio N, Portier F. Treatment of nonmalignant obstructive iliocaval lesions by stent placement: mid-term results. Ann Vasc Surg Mar;15(2):
Hartung O, Otero A, Boufi M, De Caridi G, Barthelemy P, Juhan C, Alimi YS. Mid-term results of endovascular treatment for symptomatic chronic nonmalignant iliocaval venous occlusive disease. J Vasc Surg. 2005Dec;42(6):
Raju S. Treatment of iliac-caval outflow obstruction. Semin Vasc Surg. 2015 Mar;28(1):47-53.
de Graaf R, de Wolf M, Sailer AM, van Laanen J, Wittens C, Jalaie H. Iliocaval Confluence Stenting for Chronic Venous Obstructions. Cardiovasc Intervent Radiol Oct;38(5):
Kandpal H, Sharma R, Gamangatti S, Srivastava DN, Vashisht S. Imaging the inferior vena cava: a road less traveled. Radiographics May-Jun;28(3):

15. Thank You Jeffrey Forris Beecham Chick, MD, MPH, DABR
Muhammed Noor, BS
David M. Williams, MD, FSIR