Inferior Vena Cava Agenesis and Total Caval Reconstruction

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 University of Michigan Department of Radiology

Disclosures None

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/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

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/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

Physical Examination • Gross examination – 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: 43. 0 cm • CEAP score: C 3

Magnetic Resonance Imaging Axial T 1 -weighted post-contrast Coronal T 1 -weighted post-contrast Absence of hepatic and infrahepatic IVC (arrows)

Magnetic Resonance Imaging Axial T 1 -weighted post-contrast Proximal and distal continuation of IVC (arrows)

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 16 -18 years once he reached full height potential • Patient presented 1 year later with increased right lower extremity swelling and endovascular caval reconstruction was performed

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

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).

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

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 (subcardinal, supra-cardinal, and post-cardinal veins) and vitelline veins, each one contributing to the final 5 segment adult venous system (supra-hepatic, supra-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

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, nonthrombotic, and thrombotic caval and venous outflow diseases

References • Williams DM. Iliocaval reconstruction in chronic deep vein thrombosis. Tech Vasc Interv Radiol. 2014 Jun; 17(2): 109 -13. • 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. 2007 Nov; 46(5): 979 -990. • 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. 2001 Mar; 15(2): 227 -32. • 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. 2005 Dec; 42(6): 1138 -44. • 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. 2015 Oct; 38(5): 1198 -204. • Kandpal H, Sharma R, Gamangatti S, Srivastava DN, Vashisht S. Imaging the inferior vena cava: a road less traveled. Radiographics. 2008 May-Jun; 28(3): 669 -89.

Thank You Jeffrey Forris Beecham Chick, MD, MPH, DABR chickj@med. umich. edu Muhammed Noor, BS mnoor 005@fiu. edu David M. Williams, MD, FSIR davidwms@med. umich. edu