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Contemporary management of vascular complications associated with Ehlers-Danlos syndrome
Benjamin S Brooke1, George Arnaoutakis, Nazli B McDonnell
1Division of Vascular and Endovascular Surgery, Johns Hopkins Hospital, Baltimore, MD 21287, USA.
Insights
Elective vascular procedures in patients with Ehlers-Danlos syndrome (EDS) are safe and associated with good outcomes. Interventions should not be delayed until symptoms arise, offering better results for EDS vascular disease management.
Area of Science:
- Vascular Surgery
- Genetics
- Connective Tissue Disorders
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
- Vascular manifestations in EDS can lead to significant morbidity and mortality.
- The safety of elective surgical intervention for vascular disease in EDS patients remains debated.
Purpose of the Study:
- To review the surgical management and clinical outcomes of patients with Ehlers-Danlos syndrome undergoing vascular procedures.
- To evaluate the safety and efficacy of both endovascular and open vascular interventions in EDS patients.
- To provide evidence-based recommendations for the timing of vascular interventions in EDS.
Main Methods:
- Retrospective review of all patients with EDS undergoing endovascular and open vascular procedures at a single academic medical center (1994-2009).
- Data collected included patient demographics, length of stay (LOS), and mortality outcomes (in-hospital and long-term follow-up).
- Analysis of procedural types including embolization, angioplasty, arterial bypass, and aortic aneurysm repair.
Main Results:
- 40 EDS patients underwent 45 endovascular and 18 open procedures, predominantly electively.
- Endovascular procedures had a median LOS of 2 days with no mortality; open procedures had a median LOS of 6 days with one in-hospital death in a vascular EDS patient.
- Five-year survival free of complications was 85% for endovascular and 54% for open procedures.
Conclusions:
- Elective surgical management of vascular disorders in EDS patients yields good outcomes.
- Vascular interventions in EDS patients can be safely performed electively.
- Interventions should not be withheld until rupture or acute symptoms arise to improve patient outcomes.
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.
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