SS26. Contemporary Management of Vascular Complications Associated with Ehlers-Danlos Syndrome

Abstract

There has been debate regarding the safety of performing elective procedures in patients with vascular manifestations associated with Ehlers-Danlos Syndrome (EDS). The purpose of this study was to review the surgical management and clinical outcomes of EDS patients at a tertiary medical center with multimodality expertise in connective tissue disorders. All patients with EDS undergoing elective treatment at a single institution academic medical center from 1994 to 2008 were retrospectively reviewed. Clinical data was evaluated including demographics, hospital course, types of vascular procedures, length of stay (LOS), and in-hospital mortality outcomes. A total of 183 patients with EDS were identified (mean age 32±17yrs, 102 females) including 25 diagnosed with type IV EDS. These individuals collectively underwent 32 endovascular & 18 open procedures for vascular disease during the time period, including abdominal & peripheral aneurysm repairs (n=30), and reno-visceral artery or vein embolization (n=20). Endovascular procedures were associated with a median LOS (IQR) of 2 (1-3) days with no procedure related mortality or in-hospital deaths, while open vascular procedures had median LOS (IQR) of 7 (3-9) days with 1 (6%) in-hospital death. The elective surgical management of vascular complications in EDS patients using open and endovascular procedures has been associated with good outcomes. Our results suggest that vascular interventions in these EDS patients can be safely performed and should not be withheld until rupture or acute symptoms arise.