Resolution of Large Azygos Vein Aneurysm Following Stent-Graft Shunt Placement in a Patient with Ehlers-Danlos Syndrome Type IV

Authors: D'Souza, Estelle; Williams, David; Deeb, G.M.; Cwikiel, Wojciech

Source: CardioVascular and Interventional Radiology, Volume 29, Number 5, October 2006 , pp. 915-919(5)

Publisher: Springer

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Abstract:

Ehlers-Danlos syndrome (EDS) type IV is a rare connective tissue disorder associated with thin-walled, friable arteries and veins predisposing patients to aneurysm formation, dissection, fistula formation, and vessel rupture. Azygos vein aneurysm is an extremely rare condition which has not been reported in association with EDS in the literature. We present a patient with EDS type IV and interrupted inferior vena cava (IVC) with azygos continuation who developed an azygos vein aneurysm. In order to decrease flow through the azygos vein and reduce the risk of aneurysm rupture, a stent-graft shunt was created from the right hepatic vein to the azygos vein via a transhepatic, retroperitoneal route. At 6 month follow-up the shunt was open and the azygos vein aneurysm had resolved.

Keywords: Azygos vein; Azygos vein aneurysm; Ehlers-Danlos syndrome; Shunt; Stent-graft

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s00270-004-4189-9

Affiliations: 1: Email: cwikiel@med.umich.edu

Publication date: 2006-10-01