October is Vascular Ehlers-Danlos Syndrome Action Month
October is Vascular Ehlers-Danlos Syndrome (VEDS) Action Month. Rare Disease 360, in partnership with The Marfan Foundation, spoke with James Hamilton Black III, MD, chief of Vascular Surgery at Johns Hopkins Hospital in Baltimore, about surgical considerations for patients with VEDS.
Question:
What are your recommendations for patients with Vascular Ehlers-Danlos Syndrome contemplating surgery?
James Hamilton Black III, MD:
This has been an area that I’ve really spent a lot of time and thought and energy on over the last 20 years of my career. There’s a couple of things that I recognized very early, one is that there are people with other Ehlers-Danlos subtypes who are being confused with patients who have VEDS, and those patients, of course, were not having interventions. What I realized was when they did have interventions, they are actually doing quite well. For that group of people with EDS who aren’t VEDS, they should certainly proceed with surgical interventions as they need and as seen fit.
What I appreciated also was that the VEDS group was, for the most part, only being offered interventions when essentially they were symptomatic. That is, the aneurysms were getting bigger, they were starting to bleed, and that’s an unfair proposition. Of course, the surgeon is in a tougher spot and a patient is in a tougher spot, and it’s a bit of a bad recipe.
The good news is with endovascular therapies, we can sometimes address vascular rupture in VEDS patients with very simple, straightforward, what we call embolization cases where we can go into the vessels, actually cork them off with vascular devices and stem bleeding. There are some situations where the arteries can’t be treated in that manner, and there are suitable stents to consider for some of those anatomies also. Lastly, open surgery performed on an elective fashion before you get into those life-and-death situations and life-and-death equations, it’s actually very safe and can be performed with proper preparation, very strict blood pressure control, and really a proper assessment from the genetic medicine team about the concern of tissue fragility, and then a surgeon who has experience in the area.
