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.

Liver transplantation in a patient with Ehlers-Danlos syndrome

“…1,4 EDS type III is characterized by joint hypermobility, whereas EDS type IV (or vascular EDS) has a collagen III a1 gene mutation that affects the synthesis of collagen III 1,7 and can present major complications such as aneurysms, pseudoaneurysms, and dissections of major arteries and/or spontaneous vascular or visceral rupture. [2][3][4] Because of the difficulty in managing fragile tissues, surgical procedures in patients with EDS type IV are associated with high rates of complications, including severe postoperative bleeding (37%), re-exploration (20%), and late graft anastomotic complications (40%). 8 Although EDS type IV is associated with the most serious complications and a significantly increased risk of death, vascular or visceral disease can present in different types of EDS patients.…”

“…8 Although EDS type IV is associated with the most serious complications and a significantly increased risk of death, vascular or visceral disease can present in different types of EDS patients. 2 On the basis of the available literature, one could infer that the risk for surgery is high in patients with EDS type IV, intermediate in patients with type I or II, and low in patients with type III.…”

“…Because of the uncertainty about the grade of vascular fragility, a cutdown, open access, and repair should be considered in patients with a history of previous vascular complications, when large-bore devices are used or severe coagulopathy is present. 2 Ideally, the surgeon should be aware of the diagnosis of EDS before any procedure, although the patient is aware of the diagnosis in <30% of cases. 2,8 In surgery, gentle, atraumatic, and delicate handling of tissues is mandatory to prevent vascular tears, intimal dissection, and peri-adventitial hematomas.…”

“…2 Ideally, the surgeon should be aware of the diagnosis of EDS before any procedure, although the patient is aware of the diagnosis in <30% of cases. 2,8 In surgery, gentle, atraumatic, and delicate handling of tissues is mandatory to prevent vascular tears, intimal dissection, and peri-adventitial hematomas. The use of soft, protected arterial clamps or balloon occlusion is preferable to the use of standard vascular clamps.…”

“…Autologous grafts are contraindicated if vascular reconstruction is required. 2 The postoperative course can be complicated by common findings in EDS such as chronic fatigue, muscle weakness, 9 and skin dehiscence. [1][2][3] Few if any medical options exist to lessen the surgical risk.…”