title: Resolution of large azygos vein aneurysm following stent-graft shunt placement in a patient with Ehlers-Danlos syndrome type IV.
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.
authors.affiliation: Department of Radiology, University of Michigan Hospitals, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-0030, USA.
authors.forename: Estelle S
authors.initials: ES
authors.lastname: D'Souza
authors.forename: David M
authors.initials: DM
authors.lastname: Williams
authors.forename: G M
authors.initials: GM
authors.lastname: Deeb
authors.forename: Wojciech
authors.initials: W
authors.lastname: Cwikiel
mesh_terms: D000328:Adult; D000783:Aneurysm / Q000175:diagnosis / Q000628:therapy*; D001401:Azygos Vein / Q000000981:diagnostic imaging / Q000473:pathology; D019917:Blood Vessel Prosthesis Implantation; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006503:Hepatic Veins / Q000000981:diagnostic imaging; D006801:Humans; D015641:Radiography, Interventional; D012082:Renal Veins / Q000000981:diagnostic imaging; D015607:Stents; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging / Q000473:pathology