Contemporary management of vascular complications associated with Ehlers-Danlos syndrome

Abstract

Objectives: 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 undergoing vascular procedures at a tertiary medical center with multimodality expertise in connective tissue disorders.

Methods: All patients with EDS undergoing endovascular and open vascular procedures at a single-institution academic medical center from 1994 to 2009 were retrospectively reviewed. Clinical data were evaluated including patient demographics, length of stay (LOS), and mortality outcomes during hospital course and long-term follow-up.

Results: A total of 40 patients with EDS were identified, including individuals diagnosed with classic (n = 15), hypermobility (n = 16), and vascular (n = 9) types of EDS. These patients collectively underwent 45 endovascular and 18 open procedures for vascular disease during the time period, including embolization (n = 37), angioplasty (n = 8), arterial bypass (n = 5), and aortic aneurysm repair (n = 13). All cases were performed electively, except for one (2%) urgent endovascular and one (5%) emergent open procedure. Endovascular procedures were associated with a median LOS (interquartile range [IQR]) of 2 (1 to 3) days with no procedure-related mortality or in-hospital deaths among all EDS types, whereas open vascular procedures had median LOS (IQR) of 6 (5 to 8) days with one (6%) in-hospital death occurring in a vascular EDS patient. Survival free of any complication at 5 years was 85% and 54% following endovascular and open procedures, respectively.

Conclusions: The elective surgical management of vascular disorders 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.

Fig 1

Kaplan-Meier estimates of cumulative survival free of any vascular complication among 40 patients with Ehlers-Danlos syndrome (EDS) who underwent 45 endovascular (dashed line) and 17 open vascular procedures (solid line). SEM > 10% after 4 years for open procedures and after 3 years for endovascular procedures.

Fig 2

Reconstruction of abdominal CT angiography scan showing a ruptured right anterior hepatic arterial aneurysm and associated hemoperitoneum in a patient with vascular Ehlers-Danlos syndrome (EDS) who presented with 2.4 years following successful elective coil embolization of a splenic artery aneurysm.

Fig 3

Technique for management of the access vessel in fragile vasculature of Ehlers-Danlos syndrome (EDS) patient. (A) A “U” stitch monofilament suture is placed with a pledget buttress, and needle entry is made within the area. (B) Sheath access is obtained and manipulated or exchanged minimally to avoid femoral tears. (C) The pledgetted “U” stitch suture is tied down as sheath is removed. (D) Circumferential felt reinforcement is approximated to reduce systolic pulse wave stretch on sutures and to prevent late pseudoaneurysm formation.